Rhumatology masterclass 2010

8/17/2019 Rhumatology masterclass 2010

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1. A 32-year-old man presents with a 4-monthhistory of back pain. The pain is worse in themorning and after sitting watching TV. Plainradiograph of the spinepel!is shows e!idence

of sacroiliitis. "ach of the following clinicalfeat#res might be identi$ed on clinicale%amination of this patient "&'"PT(

A.

)eratodermablenorhegica

*.

+he#matoid nod#le

'.

,nycholysis

.

!eitis

". rethritis.

'orrect Answer/ *

'omment

The long (> 1 hr) early morning and rest stifness arehighly suggestive o an underlying inammatorycondition. The pelvic radiograph conrms thepresence o sacroiliitis. Bilateral sacroiliitis are typicalor ankylosing spondylitis. The reuency oasymmetric sacroiliitis may !e higher in other

spondyloarthropathies" e.g." reactive arthritis"#eiter$s syndrome" spondylitis associated %ithpsoriasis" or inammatory !o%el disease.

+eiter0s syndrome is characterised !y a triad oarthritis" urethritis" and con&unctivitis. #eiter's


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syndrome develops in the setting o postdysentericor postvenereal illness. The characteristic rashesof keratoderma blennorrhagic#m and circinatebalanitis may be present.

soriatic spondyloarthropathy is characterised !ypsoriatic plaues. The skin involvement may !esu!tle and should !e searched or careully. The cleto the !uttock" scalp hairline" and penis are site oteninvolved !ut may !e missed easily i thorougheamination o these areas %as not activelyconducted. Psoriatic nail changes include

onycholysis" yello% nails and nail pitting.

*nammatory !o%el disease consists o ulcerativecolitis and +rohn's disease.

, diagnosis o ankylosing spondylitis may !e made%hen specic eatures o #eiter's syndrome"psoriasis" or inammatory !o%el disease are a!sent.

acroiliitis is also encountered in tu!erculosis"sarcoidosis and !rucellosis.

#heumatoid arthritis is not associated %ith lum!er orsacroiliac &oint disease. -o%ever cervical spondylitisand atlantoaial su!luation is not an uncommoneature o #,.

2. A 3-year-old man presents with a 24-ho#rhistory of a painf#l swollen left knee. e hashad minor pain and stiness in both knees formany years and now feels generally #nwell. is


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temperat#re is 3.45'. 6hat is the most likelydiagnosis(

A

.

7o#t

*.

Pse#dogo#t

'.

eptic arthritis

.

+eacti!e arthritis

". +he#matoidarthritis.

'orrect Answer/ *

'omment

*t %ould !e important to eclude septic arthritis !ymicroscopy and culture o uid aspirated rom theknee" !ut this is not the commonest cause o anacute monoarthritis.

The diferential diagnosis is inuenced !y age.+rystal arthritis is the most common cause in elderlypeople" %hereas reactive arthritis tends to occur inseually active young adults.

'alci#m pyrophosphate deposition disease8 oten kno%n as pseudogout" can oten present %ithstriking ever and systemic illness. Treatment is %ithnonsteroidal antiinammatory drugs (/0,*s) orintraarticular steroid in&ection" once sepsis has !eenecluded.


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3. 91: A ;3-year-old man is admitted withcr#shing central chest pain. e has pre!io#sly

been reasonably well b#t has had tro#blesomeasthma for many years and has recently beenfo#nd to ha!e hypertension. An "'7 shows anac#te anterior myocardial infarction8 which istreated promptly with thrombolysis.

Two days later he is noted to ha!e markedli!edo retic#laris o!er his legs. ? #moll. is blood co#nt isnormal apart from an eosinophilia at 3%[email protected] '+P is ele!ated at 4?mgl. ipstick testingof his #rine is positi!e for blood and protein.

6hich two of the following diagnoses seemmost likely(

A.

7oodpast#re0s syndrome

*.

#bac#te bacterialendocarditis

'.

7iant cell arteritis

.

'holesterol embolisyndrome

". Takayas#0s disease


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B. 'h#rg tra#ss syndrome

7.

*ehcet0s disease

.

Aortic dissection


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4. A >-year-old man presents with a 2-dayhistory of se!ere headache localising to his

right temple. e also feels tired and lethargic.e denies any eye problems and pain onchewing. The biopsy of his right temporalartery is shown 9see image:. 6hich of thefollowing statements $t best(

A.

This is a normal temporal artery biopsy.

*.

This man sho#ld be treated with ;? mgprednisolone.

'.

There is no need to consider boneprotection.

.

=?E of patients with this condition de!eloppermanent !is#al loss.

". An "+ of less than 4?mmhr e%cl#des 7iant

'ell Arteritis 97'A:.'orrect Answer/ *

'omment


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This biopsy sho%s thickening and lymphocyticinltration highly suggestive o 4+,. -e shouldindeed !e treated %ith high dose steroid initially. *t isessential to consider !one protection as he is likely to

!e on steroids or up to 5 years. (-e may also needgastric protection.) 16578 o patients developpermanent visual loss. The 90# is only elevated in:78 o cases" so i the history is good" the diagnosisshould still !e considered.

=. A 3=-year-old woman attends clinic

complaining of widespread pain and fatig#e. Ahistory8 e%amination and blood tests areperformed. 6hich two of the following wo#ldbe consistent with a diagnosis of $bromyalgia(

A.

wollen Foints

*

.

+aised creatine kinase 9'):

'.

+aised thyroid-stim#lating hormone9T:

.

+aised ele!ated sedimentation rate9"+:

".


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.


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7.

Aspergillosis

.

Nocardia


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A.

Iacroscopically clear H#id

*

.

Jeedle shaped crystals on microspcopy

'.

ighly !isco#s H#id

.

igh n#mbers of ne#trophils onmicroscopy

". *lood stained H#id

B. Thin8 watery H#id

7.

igh n#mbers of macrophages onmicroscopy

.

+homboid-shaped crystals onmicroscopy


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>. A ;-year-old woman is referred to aspecialist rhe#matology clinic. he has poorlycontrolled rhe#matoid arthritis8 and for thelast $fteen years has been treated with !ario#s

disease-modifying dr#gs incl#dings#lphasalaGine and methotre%ate. *locking thebiological f#nction of which one of thefollowing molec#les will pro!ide signi$cantanti-inHammatory eect in rhe#matoidarthritis(

A.


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therapies include a) !locking o proinammatorycytokines (*?1" *?5" *?@" *?:" T/a" 4+0" 4A+0) or"!) augmentation o antiinammatory cytokines (*?"*?17" *?11" *?1C" T4!" solu!le T/ receptors" solu!le

*?5 receptors).

Two of the maFor cytokines tho#ght to inciteinHammation in +A patients are TJB and


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weight loss8 e%amination re!eals this rash 9seeimage:. 6hile in the clinic he starts to co#gh#p blood and becomes ac#tely breathless. eis admitted and initial in!estigations re!eal a

ser#m creatinine of 1? micromoll8 a positi!emyelopero%idase 9IP,: "C


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'orrect Answer/ <

'omment

This patient has presented %ith the p#lmonary-renal syndrome8 %hich is most commonly caused!y the small vessel vasculitides (Eegener$sgranulomatosis and microscopic polyangiitis (A))although 4oodpasture$s disease" lupus andcryoglo!ulinaemic vasculitis should also !e includedin the diferential diagnosis. ,/+, positivity in thispatient group has a positive predictive value >F78or diagnosing small vessel vasculitis. ,lthoughspecicity to AD is more commonly associated %ithmicroscopic polyangiitis" 568 o patients %ithEegner$s %ill also have a positive AD. *t is thereorenot possi!le to diferentiate these t%o diseases"!ased on ,/+, specicity" nor is it important asimmunosuppressive treatment is indicatedregardless. , diagnosis o lupus is highly unlikely %itha negative ,/, and >F78 o patients %ith

cryoglo!ulinaemic vasculitis %ill have a lo% + as aresult o classical path%ay activation.

1?. A ?-year-old woman presents withinHammatory Foint pain8 fatig#e and a drymo#th. he is fo#nd to ha!e markedhypergammaglob#linaemia and a raised

erythrocyte sedimentation rate with a normal'-reacti!e protein.

6hat is the most likely diagnosis(

A +he#matoid arthritis


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.

*.

Pyrophosphatearthropathy

'.

Primary FLgren0ssyndrome

.

ystemic l#p#serythematos#s

". Bibromyalgia

'orrect Answer/ '

'omment

0icca symptoms" a raised erythrocyte sedimentationrate !ut normal +reactive protein (+#) andhypergammaglo!ulinaemia are classic eatures oprimary FLgren0s syndrome. This syndrome tendsto !egin in the th and sith decades compared %ithlupus" %hich typically !egins !et%een the secondand ourth decades. This patient could haverheumatoid arthritis !ut this is unlikely since the +#is normal. Blood tests are usually normal in patients%ith primary !romyalgia.

11. An >?-year-old woman is admitted ha!ingtripped o!er a mat. Physical e%amination is

apparently #nremarkable. he is #nable toweight bear and the ,rthopaedic team ha!edischarged her with a normal pel!icradiograph. he has been transferred to amedical ward as she is still not able to walk.6hat in!estigation9s: wo#ld yo# consider that


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wo#ld most likely pro!ide a de$niti!ediagnosis(

A

.

+epeat standard pel!ic radiograph and

check a chemical pro$le.*.

*one scan or Iagnetic +esonance


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restriction of the right hip. 6hich two of thefollowing diagnoses seem most likely(

A

.

7o#t

*.

Blare of systemic l#p#s erythematos#s9C":

'.

econdary osteoarthritis

.

Perthe0s disease

". eptic arthritis

B. econdary $bromyalgia

7.


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onset" !loods may !e normal). This patient is at increased risk o osteoporosis !ut ahip racture %ould !e a very rare event at this age"and %ould usually !e associated %ith sudden onset

o pain rather than insidious onset.Dsteoarthritis due to synovitis is almost never seenin 0?9 %hich produces a nonerosive arthritis.

13. A 1-year-old girl de!elops m#scleweakness8 a heliotrope rash and 7ottron0spap#les. The creatine kinase 9'): is raised. Them#scle biopsy con$rms an inHammatory

myositis. A diagnosis of dermatomyositis ismade. 6hich of the following symptoms areinconsistent with this diagnosis(

A.

iNc#lty gripping

*.

iNc#lty climbing stairs

'.

iNc#lty t#rning o!er inbed

.

iNc#lty hanging o#twashing

". iNc#lty writing

B. iNc#lty swallowing

7.

iNc#lty lifting head opillow

.

iNc#lty breathing


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'omment

*n a patient presenting %ith a monoarthritis" the mostimportant diagnosis to consider2eclude is a septic

arthritis. This is !est diagnosed !y microscopy andc#lt#re of the syno!ial H#id. #adiography %ill !ehelpul in determining i the patient has preeistingosteoarthritis or chondrocalcinosis. , raised %hitecell count might suggest inection. , raised +reactive protein %ould indicate inammation orinection. This patient has mild heart ailure and somay !e taking !endroumethia=ide or loop diuretics"

%hich may cause hyperuricaemia and gout andhence a monoarthritis.


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1=. A ?-year-old diabetic had problems withdecreased mobility of his back. The problemhad progressi!ely worsened o!er the lastse!en years. is l#mbar spine was remarkable

for loss of the normal lordosis and decreasedrange of motion in all planes. e had no painon perc#ssion o!er the spine or sacroiliac Foints. e was otherwise in good health8 andthe erythrocyte sedimentation rate 9"+: wasnormal. A radiograph of the l#mbar spine isshown. The most probable diagnosis is/

A.

Ankylosing spondylitis 9A:

*.

Alcapton#ria

'.

,steoporosis

.

pondylolisthesis

". i#se idiopathic skeletal hyperostosis9


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signicant degenerative disk disease" and in thea!sence o inammatory sacroiliac or acet changes.There is increased pre!alence of


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A.

Ankylosing spondylitis

*

.

Pse#dogo#t

'.

ystemic l#p#s erythematos#s9C":

.

Primary generaliGedosteoarthritis

". +eacti!e arthritis

B. 7o#t

7.

+he#matoid arthritis

.

Psoriatic arthropathy


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rheumatica).

#emem!er that acute synovitis in early rheumatoidarthritis is associated %ith very little ed &oint

deormity and no etraarticular eatures. Thiscontrasts %ith the deorming arthritis that ischaracteristic o chronic disease.

1. A 3?-year-old woman presents with a 3-month history of rec#rrent episodes of#rticaria. There are no cl#es in the history tos#ggest a possible trigger. #ring each episode

she is tired b#t otherwise well and clinicale%amination is #nremarkable. 6hat is the mostlikely diagnosis(

A.

'1 inhibitor de$ciency

*.

rticarial !asc#litis

'.

Bood allergy

.

ystemic mastocytosis

". 'hronic idiopathic#rticaria.

'orrect Answer/ "

'omment

The cardinal presenting eature o +1 inhi!itordeciency is angioedema.


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rticarial !asc#litis is characteri=ed !y painul%heals oten lasting more than 5 hours andaccompanied !y systemic symptoms.

*n the a!sence o any clues in the history" thispatient's urticaria is unlikely to !e due to ood allergy.Iastocytosis is very rareG the urticaria associated%ith mastocytosis is invaria!ly accompanied !yarier's sign and 4* involvement.

1>. A 34-year-old woman presents to yo#r

clinic complaining of cold hands8 partic#larly inthe winter months. ,n e%amination she hascold d#sky hands8 splinter aemorrhages and apetechial rash.


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". epatitis ' antibodies

B. "%tractable n#clear antibodies9"JA:

7.

"ndomysial antibodies

.

Thyroid antibodies


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1. A 13-year-old boy was hospitalised beca#seof a p#rp#ric rash in!ol!ing the legs 9seeimage: associated with abdominal pain andfe!er 93>5':. The patient also complained ofarthralgia in!ol!ing the knees and ankles.rinalysis showed protein#ria with microscopichaemat#ria. A biopsy of the p#rp#ric lesionre!ealed le#cocytoclastic !asc#litis in the small!essels. 6hich of the following statements istr#e abo#t this boy0s illness(

A.

The #rine abnormality and fe!er is mostprobably related to a recent #rinary tract

infection*.


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.

*ilateral small kidneys are a constantfeat#re on #ltraso#nd of the kidneys

". ntreated8 #p to =?E will de!elop chronic

renal fail#re.

'orrect Answer/ '

'omment

enoch-chonlein P#rp#ra 9P: is recogni=ed asa systemic small vessel vasculitis involving mainlythe !lood vessels o the skin" 4* tract" the kidneys

and the &oints.

-0 afects mainly children !et%een the ages othree and 17 years. Aales are afected more otenthan emales (1.6


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2?. A 23-year-old woman is bro#ght byamb#lance to the "mergency epartmentha!ing collapsed in a resta#rant while eating ac#rry. er friends ha!e gi!en a history of

pre!io#s allergic reactions to n#ts. ,nadmission she is H#shed8 breathless andwheeGy8 has a p#lse rate of 14? bpm and a *Pof >44? mmg.

6hat is the most appropriate $rst line oftreatment(

A.

igh-How o%ygen8 intra!eno#shydrocortisone and chlorpheniramine

*.

igh-How o%ygen and intra!eno#sepinephrine =?? Rg

'.

24E o%ygen and intram#sc#lar epinephrine=?? Rg

.

1 litre intra!eno#s saline and intra!eno#schlorpheniramine

". igh-How o%ygen and intram#sc#larepinephrine =?? Rg

'orrect Answer/ "

'omment


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corticosteroids 9witho#t epinephrine: areinadeM#ate immediate treatments foranaphyla%is" !ut may !e administered in milderallergic reactions and also to prevent late

deterioration in the event o anaphylais.

21. A 2=-year-old African man with known


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kno%n to !e associated %ith ,/+, including !acterialendocarditis and a range o tropical inectionsincluding ,moe!iasis and Aalaria. *t underlines theneed to o!tain histological evidence o small vessel

vasculitis !eore proceeding to immunosuppressivetherapy.

23. A 2?-year-old woman presented withs#dden onset of swelling of the lips andtong#e. he also had abdominal pain and!omiting. er mother con$rmed that her

da#ghter had similar attacks o!er the yearsand e!en as a child. A brother and older sisterha!e the same disorder. 6hich of the followingstatements abo#t this disease is acc#rate(

A.


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a deciency o +1 esterase inhi!itor. This results inintermittent episodes o spontaneous complementactivation. +linically the patient sufers oedema othe skin and mucosal suraces. atalities may occur i

the air%ay is compromised. '4 le!els are typicallylow d#ring an attackO they may be normal inbetween attacks.

AcM#ired angioedema 9AA": is angioedemaassociated %ith allergic reactions" %hich is otenassociated %ith urticaria. Appro%imately 4E ofcases of angioedema are dr#g-ind#ced. Aost are

patients taking angiotensinconverting en=yme (,+9)inhi!itors. *nsect stings" and oods are otherpredisposing actors

24. 92: A 2-year-old man presents withpalpable p#rp#ra and large Foint arthritis. e issystemically well. ipstick testing shows bloodand protein in the #rine. *lood testsre!eal that his ser#m creatinine is ele!ated at

2?? micromoll8 and li!er blood tests show anACT of 12? i#l 9normal 4?:.


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'.

er#m imm#noglob#lins andelectrophoresis

.

Antistreptolysin ,

". "chocardiogram

B. 'ryoglob#lins

7.

*lood c#lt#res

.

ltraso#nd of the kidneys and renal

tract


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2=. A =;-year-old man presents with a 2-monthhistory of progressi!e mid-thoracic spinal pain

that dist#rbs his sleep. A '&+ is normal. iserythrocyte sedimentation rate is ;? mmho#r9normal 1? mm:.

6hich of the following in!estigations is mostlikely to clarify the ca#se of his pain(

A

.


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Plain radiographs and 'T scans %ill sho%evidence o advanced disease !ut may !e normal inearly malignancy and inection. I+< scanning isthe most sensiti!e techniM#e" and %ill also give

inormation a!out associated sot tissues.

2;. A 1-year-old woman collapses in aresta#rant8 shortly after beginning her meal.6hich of the following statements is tr#e(

A.

Anaphyla%is is not the ca#se if neitherstridor nor wheeGe is a#dible.

*.


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oedema" %hee=e and hypotension. Kriticaria andushing may also occur. /ot all eatures are presentin every case. ,cute urticaria in this situation %ould!e suggestive o anaphylais !ut chronic urticaria is

usually nonallergic in nature.

Pallor and hypotension are feat#res of a simplefaint. atients have a slo% %eak pulse. *n situation Bthis is the most likely diagnosis unless additionaleatures o anaphylais are present.

crombroto%in poisoning occurs %hen shes such

as mackerel or tuna are spoiled" %hen they mayproduce large amounts o histamine. Knlikeanaphylais" %here symptoms are usually airlyimmediate" signs o scrom!rotoin poisoning occur atleast an hour ater ingestion o the suspect ood.ymptoms wo#ld be e%pected to respond toepinephrine.

2. 92: A 34-year-old man presents with se!erelow back pain8 which has forced him to stopwork as a b#s dri!er. e has had back pain onand o for many years8 on occasion with right-sided sciatica. The pain #sed to be helped byrest8 b#t is now present more or less all thetime and is stopping him from sleepingproperly. The most likely diagnosis is/

A.

mechanical backpain

*.

ankylosingspondylitis


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'.

myeloma

.

osteoporosis

". osteoarthritis.

'orrect Answer/ A

'omment

The history is typical o a patient %ith mechanicalback pain8 !est treated by encouragingmo!ili=ation" simple analgaesia" a gradedreha!ilitative eercise programme and treatment odepression (i present).

+ed HagK symptoms8 reuiring urgentinvestigation to eclude sinister pathology" include<

age >66 or L1: years progressive pain night pain systemic symptoms progressive neurological decit past history o malignancy or

immunosuppression recent trauma.

2>. A 2=-year-old man presents with a 3-weekhistory of haemoptysis and testic#lar pain. Theonly signi$cant $ndings on e%amination aretestic#lar tenderness and left-sidedepiscleritis. The res#lts of initial in!estigationsare as follows/ ser#m '+P 124 gl 9nr >:8 #rine


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sediment/ red cell casts8 ser#m creatinine >micromolC8 ser#m '3 124 mgdl 9nr ;=-1>=:8ser#m '4 32 mgdl 9nr 1=-=?:8 antin#clearantibody negati!e8 chest radiograph/ bilateral

nod#lar shadowing. 6hich of the followingdiagnoses is most likely to acco#nt for hisclinical presentation(

A.

ystemic l#p#s erythematos#s 9C":

*.

mall cell l#ng carcinoma

'.

Ii%ed cryoglob#linaemia

.

mall !essel !asc#litis of the 6egener0smicroscopic polyangiitis spectr#m

". 7iant cell arteritis.

'orrect Answer/

'omment

This patient has an inHammatory m#lti-systemdisorder o relatively short duration afecting hislungs" kidneys" eyes and testes. The constellation onodular lung shado%s" glomerulonephritis (asevidenced !y red cell casts in the urine)" episcleritis"testicular tenderness and an elevated +# is highly

suggestive o an ,/+,M necrotising small vesselvasculitis. 0ystemic immunecomple diseases suchas 0?9 and mied cryoglo!ulinaemia are ecluded !ythe negative ,/, and normal complement prole"respectively.


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This patient needs assessment o his ,/+, statusand tissue !iopsyeither kidneys or lungs" preera!lykidneys.

2. A 4?-year-old woman s#ddenly de!elopsse!ere +ayna#ds that is tro#blesome in thes#mmer as well as the winter. he starts tode!elop swelling of the $ngers and feels tired.he also de!elops reH#% oesophagitis and hasdiNc#lty swallowing. he has noticed that shehas become more breathless. ,n e%amination8she has skin thickening aecting her hands8

face and tr#nk. 6hich of the following is mostlikely to be positi!e(

A.

s JA antibody

*.

+o antibody

'

.

Anticentromere

antibody

.

Do-1 antibody

". cl-? antibody.

'orrect Answer/ "

'omment

This patient is developing di#se systemicsclerosis. ,nticentromere pattern is associated %ithlimited systemic sclerosis. #o anti!ody is common inpatients %ith primary 0&ogren's syndrome and


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systemic lupus erythematosus (0?9). No1 may !epositive in patients %ith polymyositis.

3?. A ;>-year old man presented with s#dden

se!ere pain and swelling in the left knee.yno!ial H#id analysis shows ab#ndant calci#mpyrophosphate dihydrate 9'PP: crystals.6hich of the following tests is J,T appropriatefor f#rther assessment of this patient illness(

A.

'reatinine kinase

*.

er#m calci#m

'.

Thyroid f#nctiontest

.

er#m ferritinle!el

". b A


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disorders are associated %ith + crystaldeposition" %hich include<

dia!etes mellitus" hemochromatosis" Eilson's disease" hypothyroidism" hyperparathyroidism" hypomagnesemia" hypophosphatasia.

31. A 3?-year-old man has a swollen wrist8 low

back pain with early morning stiness lasting 2ho#rs and mo#th #lcers. e does not ha!e arash. 6hat is the most likely diagnosis(

A.

+he#matoid arthritis 9+A:

*.

Psoriatic arthritis

'.


.

7o#t

". systemic l#p#s erythematos#s9C":

'orrect Answer/ '

'omment

The history is suggestive o a seronegativespondyloarthropathy.


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+A is classically a peripheral8 symmetricalpolyarthritis.

7o#t can aect the wrist b#t is #n#s#al in a

yo#ng person.atients %ith 0?9 can eperience mouth ulcers !ut0?9 is uncommon in young males.

32. A =2-year-old acco#ntant8 with a ;-yearhistory of +ayna#dKs phenomenon presents toher 7.P. with a 4-month history of worseningdysphagia.


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.

.


". Fogren0s disease.

'orrect Answer/ '

'omment

+ayna#dKs phenomenon is common" occurring in168 o the general population and in the ma&orityo cases is not associated %ith connective tissue

disease. -o%ever" the detection o an anticentromere nuclear staining pattern has a highspecicity or limited systemic sclerosis"especially in the contet o dysphagia %hich is aeature o the disease.

33. An 1>-year-old st#dent with known asthmaand pean#t allergy collapses following a meal

in the hospital canteen. tren#o#s attempts atres#scitation are tragically #ns#ccessf#l.


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'.


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.

". Prolonged treatment withcorticosteroids

B. +he#matoid arthritis

7.

'rohn0s disease

.

Asthma


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3=. This patient was trying to stand #p. ,n

e%amination he has periorbital oedema with afaint p#rple h#e. The ser#m creatinine kinase9'): le!els were 1?-times the #pper limit ofnormal. 6hich of the following manifestationsis least likely to be associated with thisdisorder(

A

.

bl#e and white color change in the $ngers

on e%pos#re to cold

*.

diNc#lty in swallowing liM#ids

' do#ble !ision on looking to the sides


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.

.

radial p#lse of 4? beats per min#te

". persistent co#gh8 haemoptysis and weightloss of 12 kg in the last three months

'orrect Answer/ '

'omment

This patient is not a!le to stand up rom a chairunaided. This indicates pro%imal m#scle

weakness. The skin rash and the raised levels omuscle en=yme point to%ards an inammatorymyopathy" namely dermatomyosytis 9I:.A is easily recogni=ed and diagnosed !y acharacteristic rash<

a heliotrope rash (!luepurple discoloration) onthe upper eyelids" %ith oedema

a at red rash on the ace and upper trunk.

"rythema of the kn#ckles accompanied !y araised8 !iolaceo#s scaly er#ption 97ottron0ssign: is also characteristic" and may precede orollo% muscle %eakness. +ayna#d0s phenomenon %ith characteristic color change (pallor" cyanosis andru!or) on eposure to cold is encountered in patient%ith A" more oten %hen there is overlap %ith otherconnective tissue disease such as scleroderma ormied connective tissue disease.*nammation o the skeletal muscles o theoropharyn and upper oesophagus lead todysphagia especially or liuids in the initial stageso the disease %hich progresses to diJculty in


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s%allo%ing solids as the disease progresses.9lectrocardiographic evidence o cond#ctiondefects and arrhythmias occur reuently inchildhood and adult A" although overt clinical

symptoms are uncommon. There is an increased incidence o underlyingmalignancy in adult A" ranging rom 68 to 168.'arcinoma of the bronch#s8 o!aries and breastare the most common.,c#lar m#scles remain normal" even inadvanced" untreated cases" and i these muscles areafected" the diagnosis o inammatory myopathy

should !e in dou!t.

3;. A 34-year-old woman presents to yo#rclinic complaining of cold hands8 partic#larly inthe winter months. ,n e%amination8 she hascold d#sky hands and a petechial rash.


49/187

.

ForgrenKs syndrome is likely if +o and Cae%tractable n#clear antigens are present.

". epatitis ' is #nlikely in this case.

'orrect Answer/

'omment

The symptoms and signs and low '4 ares#ggesti!e of cryoglob#linaemia.

0ludging o proteins at reduced temperatures (oreample hands on a cold day) can cause ischaemiaand sometimes vasculitis" particularly o skin orkidneys.

+ryoglo!ulinaemia is commonly associated %ithhepatitis + or connective tissue disease" such as0&orgren's syndrome. The positive ,/, and highglo!ulins suggest 0&orgren's !ut could also !eassociated %ith chronic inection" such as hepatitis +.

0ince they precipitate at lo% temperatures"cryoglo!ulins should al%ays !e transported to the la!at COP+. ailure to do this %ill result in a alsenegative result as the cryos %ill precipitate and !eremoved %ith the clot.

3. A ;-year-old man with a 1?-year history of rhe#matoid arthritis8 controlled with diclofenac

and s#lphasalaGine has reported rec#rrentheart b#rn and dyspepsia. is doctor askedhim to stop the dicofenac sodi#m and replacesit with celeco%ib 9one of the ',& 2 inhibitors:.6hich of the following is tr#e abo#t ',& 2selecti!e agents(


50/187

A.

They eliminate the chance of non-steroidalanti-inHammatory dr#gs 9JA


51/187

risk o developing signicant upper gastrointestinalcomplications in the rst si months. -o%ever" therisk o 4* !leeding rom nonselective /0,*s is lo% an annual incidence o 18 to C8 and the +DQ5

selective /0,*s are costly. Thereore" it may !e !estto reserve these drugs or patients at highriskpatients to include those over @6 years" thosealready using medications kno%n to increase thelikelihood o upper gastrointestinal adverse events"those %ith serious comor!idity" and those reuiringprolonged use o maimum recommended doses ostandard /0,*s.

,ll /0,*s" including the +DQ5 selective agents" cancause renal toicity" such as acute renal ailure"nephrotic syndrome" and acute interstitial nephritis.,cute renal ailure is a concern particularly inpatients %ith preeisting renal dysunction. /0,*sshould !e avoided also in patients %ith cirrhosis andascites or congestive heart ailure. eriodic

monitoring o renal unction is recommended"particularly in elderly patients.

3>. A 3;-year old woman is referred with a 1-year history of m#scle pain8 tiredness andsleep dist#rbance. he denies fe!er8 weightloss and arthralgia. "%amination re!ealstenderness o!er her occip#t8 trapeGi#s and

l#mbar area. er blood res#lts show a normal"+8 '+P8 B*'8 a weakly positi!e AJA 1/>? andnormal complement. 6hich is the most likelydiagnosis(

A.

Polymyositis


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*.

ystem l#p#s erythemato#s9C":

'

.

FogrenKs syndrome

.

Polymyalgia rhe#matica

". Bibromyalgia

'orrect Answer/ "

'omment

The lack o constitutional symptoms" normalinammatory markers and normal eamination" apartrom evidence o tender points" make aninammatory rheumatological disease unlikely. Thepresence o tender points" history o muscle pain andsleep distur!ance are suggestive o $bromyalgia Ra noninammatory pain disorder.

3. A 2=-year-old woman with a history of 3-second trimester fetal losses is planning afo#rth pregnancy. he has e!idence of theprimary anti-phospholipid syndrome 9stronglypositi!e cardiolipin antibody8 positi!e l#p#santicoag#lant b#t no e!idence of l#p#s:. 6hichof the following treatment regimens oer her

the best chance of ha!ing a s#ccessf#lpregnancy(

A.

teroids alone

* teroids combined with low-dose aspirin


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.

'.

Cow-dose aspirin alone

.

Cow-dose aspirin combined with lowmolec#lar weight heparin

". -year-old woman with ple#ritic chest pain. 6hich ofthe following statements concerning thispatient are tr#e(

A.

Cow white co#nt is consistent with systemicl#p#s erythematos#s 9C":

*.

Cow white co#nt is not consistent witht#berc#losis.

'.

The presence of anticardiolipin antibodies


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". The presence of antin#clear cytoplasmicantibodies 9cAJ'A: at a titre of 1 in 2?s#ggests that 6egener0s gran#lomatosis islikely.

'orrect Answer/ A

'omment

Acti!e C" and t#berc#losis are bothassociated with le#kopenia. ?o% titre antinuclearanti!odies (in the a!sence o /, or 9/,)"antineutrophil cytoplasmic anti!odies (,/+,) and

anticardiolipin anti!odies are nonspecic and arecommonly ound in the presence o inection.

41. A ;;-year-old woman has a 1=-year historyof deforming rhe#matoid arthritis 9+A:. he ismaintained on -Penicillamine. Two weeks agoshe noticed increased diNc#lty in climbingstairs and three days before admission she was

#nable to comb her hair or feed herself.Je#rological assessment is diNc#ltb#t re!eals generalised grade 3= weakness8and the *abiniski sign is positi!e bilaterally.6hich one of the following tests is most likelyto re!eal the diagnosis(

A.

Plain radiograph of the cer!ical spine

*.

"lectromyography 9"I7:

'.

Jer!e cond#ction st#dy 9J':


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.


56/187

-Penicillamine causes various neuromusculardisorders including myathenia gravis.

There is no clinical evidence or primary muscledisease or peripheral neuropathy in this patien"hence 9A4 or /+0 are not necessary.*sotope !one scan may identiy increased activity inthe cervical region" !ut this is not specic and theinvestigation has little role in investigation osuspected spinal cord compression.

42. A ?-year-old woman with a history ofblood transf#sion in the early 1>?s presentswith a 1?-month history of malaise and isnoted to ha!e impaired renal f#nction. er#rine sediment re!eals red cell casts. Theres#lts of imm#nological in!estigations are asfollows/ ser#m


57/187

*.

Antin#clear antibodies

'

.

Antiglomer#lar basement membrane

antibodies

.

Antimyelopero%idase antibodies

". 'ryoglob#lins

'orrect Answer/ "

'omment

The combination of a markedly low '4 9withnormal '3:8 ele!ated rhe#matoid factor8ele!ated ser#m


58/187

.

.

Iethotre%ate

". Penicillamine.

'orrect Answer/

'omment

ydro%ychloroM#ine can e%acerbate psoriasis.0ulphasala=ine tends to only improve &oint symptomsand not improve the psoriasis. 4old and

penicillamine are not commonly used to treat thiscondition.

44. A 4=-year-old woman de!eloped +ayna#d0s2 years ago. he now complains ofbreathlessness and skin tightness aecting her$ngers. A high-resol#tion 'T scan showse!idence of p#lmonary $brosis.

6hich of the following tests is most likely to bepositi!e(

A.

Anticentromere antibody

*.

Anti-do#ble-stranded JAantibody

'.

Anti-+o antibody

. Anti-cl-? antibody


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". Anti-Do-1 antibody

'orrect Answer/

'omment This patient has clinical symptoms suggestive odi#se c#taneo#s systemic sclerosis. ulmonary!rosis and anti0clO7 are more common in patients%ith difuse disease. ,nticentromere anti!ody isassociated %ith limited cutaneous systemic sclerosis.,ntidou!lestranded /, anti!ody is associated%ith systemic lupus erythematosus. ,nti#o anti!ody

is associated %ith lupus and primary 0&Hgren'ssyndrome. ,ntiNo1 is associated %ith polymyositis"particularly in patients %ith inammatory lungdisease.


60/187

4=. A =>-year-old woman is referred with painand stiness in her hands and knees. he has afew patches of psoriasis on her arms. erhands are shown in the pict#re. 6hat are the

two most likely diagnoses(

A.

7o#t

*.

Jodal osteoarthritis

'.

Pse#dogo#t

.

ystemic sclerosis

". Psoriatic arthritis

B. ystemic l#p#serythematos#s

7

.


.



61/187

case).*nvolvement o the !ase o the thum! is alsopathognomonic o nodal osteoarthritis" giving thethum! !ase a characteristically suare appearance.

4;. A 3=-year-old man is referred to yo# forin!estigation of rec#rrent infection. e has hadfreM#ent respiratory tract infections for thepast = years8 reM#iring 4-= co#rses ofantibiotics each winter. A month pre!io#sly hewas admitted with pne#mococcal pne#monia.Two months prior to that he had sin#s s#rgery.

owe!er8 this did not impro!e his rec#rrentsin#sitis. 6hich of the following is not in thedierential diagnosis(

A.

Antibody de$ciency

*.


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o lymphoprolierative disease.


63/187

.

Je#trophils

". Platlets

B.


64/187

and ha!e sent the p#r#lent-looking H#id formicroscopy. 6hich of the following is tr#e(

A.

e is likely to ha!e acM#ired this infectionafter an arthroscopy or arthrocentesis.

*.

e!enty to eighty percent of cases will ha!ean accompanying bacteraemia.

'.

+es#lts of the c#lt#re sho#ld be awaitedbefore commencing antibiotics.

.

Antibiotics sho#ld co!er beta-haemolyticstreptococc#s and staphylococcus

infections.". A plain %-ray of the knee will con$rm the

diagnosis.

'orrect Answer/

'omment

This is likely to !e septic arthritis. D!viously"

eamination o the synovial uid %ill help to ecludediferentials such as gout and pseudogout. The mostlikely organisms are beta-haemolyticstreptococci 92?E: and staphlococci 9?E:. Antibiotics should !e started empirically to coverthese i the clinical suspicion is high. *deally theseshould !e intra!eno#s for 2 weeks and then oralfor 4 weeks. ,rthroscopy is a risk actor or septic

arthritis" !ut is rare. Bifty percent of cases %illhave an associated bacteraemia. "arly %-rays arealmost al%ays normal.

4. A 2>-year-old man is has recently beendischarged from hospital after treatment for


65/187

pne#mococcal pne#monia. e has hadrepeated co#rses of antibiotics for sin#s8 earand lower respiratory tract infections8 and hadsin#s s#rgery the pre!io#s year. e is a life

long non-smoker and is not on medication. isblood co#nt prior to discharge was normal.


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deects can oten !e treated %ith appropriatevaccinations and2 or prophylactic anti!iotics.

=?. A 1;-year-old boy is admitted to the AW"

department after collapsing following a waspsting. is blood press#re is recorded at ?=?mmg and e%amination re!eals a widespread#rticarial rash. is blood press#re and rashrespond to ?.=mg i.m. adrenaline8 1??mghydrocortisone i.!. and 1? mgchlorpheniramine i.!. After being seen in clinictwo weeks later8 which two of the following

tests wo#ld be most clinically #sef#l(A.


67/187

'orrect Answer/ *B

'omment

This patient has sufered an anaphylactic reactionto bee !enom. , rise in serum tryptase (i taken%ithin @ hours) can help in identiying cases oanaphylais %here there is diagnostic uncertainty.-o%ever" in this patient the classical eatures andtemporal relationship to a !ee sting esta!lish thediagnosis clinically. -e should !e tested or venomspecic *g9 !y !oth skin prick testing and #,0TG ieither are positive his anaphylactic risk or a urther%asp sting can !e dramatically lo%ered !y venomimmunotherapy

51. A =?-year-old woman presents to cas#altywith a 2-year history of rec#rrent angioedema.er clinical history fails to re!eal an #nderlyingtrigger. er ser#m complement pro$le is as

follows/ ser#m '3 1.2 gl 9ref range ?.=-1.;=:8ser#m '4 ?.?2 gl 9ref range ?.2-?.;:. Themost likely diagnosis is/

A.

food allergy

*.

dr#g allergy

'.


68/187

". !enom allergy.

'orrect Answer/

'ommentKncontrolled activation o the classical complementpath%ay leading to a marked reduction in serum +levels is a hallmark o '1 inhibitor de$ciency. *nthe a!sence o a amily history" it is possi!le that thisrepresents a ne% mutation. ,lternatively" thepossi!ility o acuired +1 inhi!itor deciency due tolymphoprolierative disease should !e considered.

=2. A -year-old man presents with persistenthead ache and progressi!e deafness. ,ne%amination the patient has frontal bossing ofthe forehead and cond#cti!e deafness8 morese!ere in the right ear. is ser#m alkalinephosphatase is signi$cantly raised at >? #C.

6hich of the following statements is mostacc#rate abo#t this disease(

A.


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". *one pain is typically increased with restand on weight bearing.

'orrect Answer/ "

'omment

Paget0s disease o !one is a ocal disorder o !oneremodelling characteri=ed !y an increase in thenum!er and si=e o osteoclasts in afected skeletalsites %hile the rest o the skeleton is spared. aget$sdisease most commonly involves the aial skeleton"the pel!is being the most common" !ut it can

afect any area. *n the ma&ority o patients" thedisease afects at least t%o !ones" !ut in one third opatients only one !one is afected. *n the skull" the:th nerve can !e compressed" resulting in hearingloss. This is one o the more common complaints"!eing present in CO8 o respondents in a recentsurvey o 5777 patients %ith aget's disease . Dthercauses o hearing loss include pagetic involvement o

the middle ear ossicles" %hich dampens the motiono these ossicles.Knlike osteoarthritis" pagetic bone pain #s#allyincreases with rest" on %eight !earing" %hen thelim!s are %armed" and at night. ,n estimated O78 o patients %ho have aget$s disease have nosymptoms. The diagnosis is typically oundincidentally on radiographs and la!oratory

investigations.

=3. A yo#ng patient is attending therhe#matology monitoring clinic. he de!elops


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le#copaenia. 6hich of the following is #nlikelyto ca#se this abnormality(

A.

AGathioprine

*.

Iethotre%ate

'.


.

'yclosporin A

". "tanercept.

'orrect Answer/

'omment

atients taking aGathioprine8 methotre%ate andetanercept reuire regular monitoring o the ull!lood count to eclude marrow s#ppression.

atients %ith 0?9 oten have a lo% %hite cell count.

=4. A 2-year-old man s#ers a stroke ca#singleft sided weakness. e has !ery little!ol#ntary mo!ement of the left arm and needsassistance to transfer. 6hilst on therehabilitation ward he complains of signi$cantpain aro#nd his left sho#lder. 6hich of the

following statements is correct(A.

Poor handling and positioning of the left armby sta are #nlikely to ha!e contrib#ted tothe pain

* ho#lder pain ca#ses distress b#t does not


71/187

. aect o#tcome

'.

A radiograph of the sho#lder is notnecessary

.

Treatment with simple analgesics is asensible initial approach

".


72/187

=;. A >-year-old woman fo#nd it m#ch easierto manage after the occ#pational therapistpro!ides se!eral aids and appliances for #se inthe kitchen. Brom their appearance8 theproblem aecting her hands 9see image: is/

A.

rhe#matoidarthritis

*.

nodalosteoarthritis

'.

psoriaticarthropathy

. tophaceo#s go#t

". 'harcot Foints.

'orrect Answer/ '

'omment


73/187

This is the classic appearance o arthritis m#tilans in psoriatic arthropathy" %ith a deorming destructivearthritis o the small &oints. Dsteolysis results inshortening o some o the digits. The pattern is not

that o rheumatoid arthritis and the %rists appear to!e spared. There is too much destruction orosteoarthritis. The appearance o gout may !esimilar" though the tophi %ould !e easier to see inlie (as opposed to a photo). +harcot &oints(neuropathic arthropathy) usually afect the oot andankle.

=. A =;-year-old man presents with rec#rrentattacks of polyartic#lar go#t despite treatmentwith allop#rinol 3?? mg daily for the last year.e is adamant that his compliance with hisdr#g treatment regimen is good.

6hich of the following factors is most likely toe%plain his poor response to allop#rinol(

A. 'onc#rrent treatment with

colchicine

*.

Bast metabolism of allop#rinol

'

.

igh alcohol intake

.

Poor #rate clearance !ia thekidney

". igh dietary p#rine intake


74/187

'orrect Answer/ '

'omment

Persistently high alcohol cons#mption is acommon ca#se of poor response to allop#rinol"although the underlying mechanism o this is unclear.B" and 9 are plausi!le ans%ers" !ut are lessimportant in practice. Aost adults %ill respond toallopurinol C77 mg daily" although a small proportion%ill reuire @77 or even F77 mg daily. The aim otreatment should !e to suppress the serum uratelevel to the lo%er end o the normal range or &ust!elo%.

=>. A ;>-year-old woman complained ofprogressi!e pain in her left hip after slippingon ice and s#staining a s#b-capital fract#re ofthe left femoral neck hip. Bollowing reco!eryfrom a hemiarthroplasty8 bone mineral density9*I: meas#rement of the opposite fem#r

con$rmed the diagnosis of osteoporosis with aT score of -2.;. he did not wish to takeoestrogen replacement therapy and was gi!enalendronate ? mg weekly. 6hich of thefollowing statements is tr#e(

A.

Jormally the peak bone mass in a woman isachie!ed F#st before the menopa#se.

*. Plain radiographs are !ery sensiti!e inidentifying minor red#ction in bone mineraldensity and hence a !ery #sef#l screeningtest.

' The T score compares a patientKs *I with


75/187

. the mean !al#e for yo#ng8 healthy ad#lts ofthe same se%.

.

A high ser#m phosphate le!el may be

s#ggesti!e of chronic alcoholism.

". *isphosphonates s#ch as alendronate act bystim#lating bone formation.

'orrect Answer/ '

'omment

Bone mass increases during childhood and

adolescence and peaks !et%een the ages o 57 andC7 years. eak !one mass is inuenced !y age" se"genetic actors" hormonal status" eercise" andcalcium intake. ,s %omen in general have a lo%erpeak !one mass than men" it is understanda!le %hyosteoporosis is predominantly seem in %omen.

lain radiographs are not sensitive enough to

diagnose osteoporosis. #esults o bone mineraldensity 9*I: tests are typically reported as Tscores and X scores.

The T score compares a patient's BA %ith themean value or young" healthy adults o thesame se.

The X score compares a patient's BA %ith themean value or persons o the same age and

se.

Both scores are epressed in terms o standarddeviations rom the mean. The Eorld -ealthDrgani=ation has dened osteoporosis as a BA o atleast 5.6 standard deviations (0) !elo% the mean


76/187

value in young normal adults (i.e. TY-2.=:.

+hronic alcoholism is another important cause o lo%serum phosphate level" in %hich case the liver

en=ymes %ill !e elevated. All bisphosphonates actsimilarly on !one in !inding permanently tominerali=ed !one suraces and inhibitingosteoclastic acti!ity. Thus" they inhi!it !oneresorption and less !one is degraded during theremodelling cycle. They do not stimulate !oneormation.

=. A ;>-year-old man has lost weight. isalkaline phosphatase is raised at 2? C9normal range 3=-12?:. Plain radiographs showsclerotic lesions of bone.

6hat is the likely diagnosis(

A

.

tomach cancer

*.

Prostate cancer

'.

I#ltiplemyeloma

.

C#ng cancer

". ,steomalacia

'orrect Answer/ *

'omment


77/187

'ancer of the prostate is typically associatedwith sclerotic bone lesions in contrast to the lyticlesions seen in multiple myeloma. Dsteomalacia isassociated %ith ?ooser's =ones.

;?. A 33-year-old woman presents with a ;-month history of +ayna#d0s phenomenonaecting her hands and feet. he is pre!io#slywell and takes no medication.

6hich two of the following clinical feat#res orin!estigation res#lts are the strongestpredictors that she will de!elop a connecti!etiss#e disease in the f#t#re(

A.

Age S2= years

*.

Abnormal nail-fold capillarymicroscopy

'.

"le!ated erythrocyte sedimentationrate

.

istory of rec#rrent miscarriage

". Bamily history of +ayna#d0s

B. Anaemia

7

.

trongly positi!e antin#clear

antibody

.

istory of chilblains


78/187

D. ry eyes and dry mo#th

'orrect Answer/ *7

'omment These actors are strongly predictive o a utureconnective tissue disease (+T)" particularlyabnormal nail-fold capillaries. The likelihood odeveloping a +T also increases %ith age o onset o#aynaud's" %ith a particularly high risk in those agedover C6 years. ,ll the other eatures apart rom aamily history (%hich suggests primary #aynaud's)

are associated %ith +T" !ut have not !een sho%n tohave the same predictive value as B and 4.

;1. A 22-year-old man has chronic diarrhoeaand has had se!eral episodes of sin#sitis andpne#monia. A diagnosis of common !ariableimm#node$ciency 9'V


79/187

7.

'ardiomyopathy

.

'hronic renal

fail#re


80/187

*.


'

.


.

Primary complement de$ciency

". '1 inhibitor de$ciency.

'orrect Answer/ '

'omment

This lady has a hypocomplementaemicglomerulonephritis (note active urine sediment) anda purpuric rash accompanied !y a positiverheumatoid actor and an isolated positive ,/,.

The com!ination o renal and skin involvement in thissetting %ith a markedly lo% + suggests miedcryoglo!ulinaemia. -er serology does not support0?9.

The key investigations %hich %ould help conrm thediagnosis %ould !e a %arm clotted sample orcryoglo!ulins and a renal !iopsy.

;3. A ;-year-old man with rhe#matoidarthritis presents with increasedbreathlessness and nose bleeds. 6hich of the

following treatments for rhe#matoid arthritisare #nlikely to ca#se bone marrows#ppression(

A 7old


81/187

.

*.

AGathioprine

'.

Jon-steroidal anti-inHammatory dr#gs9JA


82/187

rec#rrent attacks of red congested eyes with asensation of grittiness. The most likely ca#seof her red eyes is likely to be/

A.

scleritis

*.

episcleritis

'.

keratitis

.

keratoconF#ncti!itis

sicca". choroiditis.

'orrect Answer/

'omment

,pproimately 56 8 o patients %ith rheumatoidarthritis (#,) %ill have ocular maniestations.

keratocon&unctivitis sicca" scleritis" episcleritis"keratitis" peripheral corneal ulceration" and other lesscommon entities such as choroiditis" retinalvasculitis" episcleral nodules" retinal detachments"and macular edema.

)eratoconF#ncti!itis sicca8 or dry eye syndrome"is the most common ocular maniestation o #, and

has a reported prevalence o 16 to 56 8. Thepatient reports a gritty sensation in the eyes .

0cleritis and episcleritis as causes o recurrent redeyes are distinguished on the !asis o anatomy andappearance. 0ymptoms may !e similar" !ut the pain


83/187

in scleritis is more evident and severe. Tenderness topalpation o the glo!e can help diferentiate the t%o.,ter asking the patient to look do%n %ith eyelidsclosed" the physician gently presses the glo!e.

atients %ith scleritis have tenderness on palpation"%hile those %ith episcleritis do not.. Knlike scleritis"patients %ith episcleritis do not complain o !lurredvision or photopho!ia. The importance o correctlydiagnosing and distinguishing !et%een scleritis andepiscleritis is !ased on the potential ocular andsystemic complications associated %ith scleritis.0tudies have sho%n that patients %ith #,associated

scleritis have more %idespread systemic disease anda higher mortality rate than those episcleritis.

;=. A 2?-year-old man with common !ariableantibody de$ciency presents to the "mergencyepartment with a 3-day history of co#ghprod#cti!e of green sp#t#m. is temperat#re

is 3.=Q'8 p >4min8 respiratory rate 12min8and breath so#nds are !esic#lar. 'hestradiograph is #nremarkable. 6hich two of thefollowing actions do yo# recommendimmediately(

A.

Prescribe a 14 day co#rse of antibiotics

*.

Prescribe neb#lised salb#tamol

'.

7i!e an inf#sion of intra!eno#simm#noglob#lin


84/187

.

Take a sp#t#m sample to look for acid fastbacilli

". Prescribe a =-day co#rse of antibiotics

B. 'heck his ser#m imm#noglob#lin le!els

7.

'hoose an antibiotic regimen s#itable forpossible pse#domonas infection

.

,rder a high resol#tion 'T scan

day co#rse of antibiotics.

'orrect Answer/ A<

'omment

,nti!ody decient patients need prompt treatment o presumed !acterial inection. Treatment should !econtinued or slightly longer than normalG 1 days or

an uncomplicated chest inection %ould !eappropriate. Aost inections are caused !y commonorganisms such as haemophil#s orpne#mococc#s. suedomonas is unusual andmyco!acterial disease rare in common varia!leimmunodeciency. +ultures are invalua!le i there isa poor response to treatment and or guiding utureanti!iotic choices. or inections causing ever"

routine anti!ody replacement should !e deerred or5: hours until there is a clear response totreatment" as adverse reactions are much morecommon in the presence o ever.


85/187

;. A ;=-year-old woman with a mitral !al!ereplacement presents to the "mergencyepartment with pyre%ia and fainting. he is#nwell8 hypotensi!e8 anaemic and pyre%ial. he

has a !ag#e history of s#ering from areaction to penicillin in her childhood. Aftertaking blood c#lt#res she is started on broad-spectr#m antibiotics. 'ardiac !al!#lar!egetations are seen on echocardiography andher blood grows methicillin-sensiti!eStaphylococci [ the microbiologist s#ggestsna$cillin as the most appropriate antibiotic8

b#t is concerned that she may ha!e allergy tobeta lactam-based antibiotics.

6hich of the following is most appropriate toin!estigate her history of possible penicillinallergy(

A

.

ser#m tryptase

*.

skin prick test topenicillin

'.

ser#m penicillin speci$c


86/187

, history o penicillin allergy is relatively common inhospital patients" and is in most not due to type <hypersensiti!ity reactions. , diagnosis openicillin allergy crucially reuires a detailed history

o the drug reaction" and can !e conrmed !y apositi!e skin prick test to the ma&or and minordeterminants o penicillin. 0kin prick testing is carriedout i there is a clinical need or penicillin treatmente.g. treatment o inective endocarditis. , patient isunlikely to develop anaphylais %ith a negativepenicillin skin prick test. The detection o penicillinspecic *g9 in the serum is unrelia!le.

;>. A 3;-year-old man presents with a 2-dayhistory of se!ere pain in the left knee. e hasrecently ret#rned from a holiday in pain."%amination shows low grade fe!er 93.=Q':and marked syno!itis of the left knee with atense e#sion. 6hich are the two most likelydiagnoses(

A.

Cyme disease

*.


'.

Pse#dogo#t

.

7o#t

". eptic arthritis

B. ,steosarcoma

7 ,steoarthritis


87/187

.

.

+eacti!e arthritis


88/187

white cell co#nt ;.4 % 1?C 9normal range 4-11:8 platelet co#nt 32? % 1?C 9normal range1=?-4??:8 "+ > mmho#r8 '+P ; mgdC9normal ;:8 ser#m -4.?: and


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persistently elevated 90#. /ote that her +reactiveprotein (+#) is normal" %hich reects the act that+# production is not inuenced !y changes inserum immunoglo!ulin levels.

?. A 2>-year-old woman presented withfatig#e and e%treme tiredness. Physicale%amination re!ealed facial skin rash andtenderness across the small Foints of thehands. he was concerned that she might ha!esystemic l#p#s erythematos#s 9C":. 6hich ofthe following tests when J"7AT


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diseases.

Antibodies to m antigen are highly speci$c fora diagnosis of C" 9SE:. -o%ever" only a!out

568 o patients %ith 0?9 have anti0m anti!odies.Anti-JA anti!odies are diagnostic o 0?9(specicity >FF8). -o%ever" only @78 o patients%ith 0?9 %ill have these anti!odies. Thereore"a!sence o anti/, or anti0m anti!odies should noteclude 0?9 as a diagnosis. ,nti#o200, anti!odiesare ound in C78 o patients %ith 0?9. Anti-histoneantibodies are identied in small proportion o 0?9

patients. They are more oten seen %ith druginduced lupus.


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92/187


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31. A 3?-year-old man has a swollen wrist8 lowback pain with early morning stiness lasting 2ho#rs and mo#th #lcers. e does not ha!e arash. 6hat is the most likely diagnosis(

A.

+he#matoid arthritis 9+A:

*.

Psoriatic arthritis

'.



94/187

.

7o#t

". systemic l#p#s erythematos#s

9C":'orrect Answer/ '

'omment

The history is suggestive o a seronegativespondyloarthropathy.

+A is classically a peripheral8 symmetricalpolyarthritis.

7o#t can aect the wrist b#t is #n#s#al in ayo#ng person.atients %ith 0?9 can eperience mouth ulcers !ut0?9 is uncommon in young males.


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tho#ght that the most likely ca#se of hiscollapse was an anaphylactic reaction tohidden0 n#ts contained in the meal.

6hich of the following mast cell-deri!edmediators is it most #sef#l to meas#re tocon$rm the clinical s#spicion of anaphyla%is(

A.

Iast cell - deri!ed growthfactor

*.

'hymase

'.


97/187

shows a !ertebral cr#sh fract#re. 6hich two ofthe following red#ce an indi!id#al0s risk forde!eloping osteoporosis(

A.

"arly menarche

*.

"arly menopa#se

'.

moking

.

igh alcohol intake

". Prolonged treatment withcorticosteroids

B. +he#matoid arthritis

7.

'rohn0s disease

.

Asthma


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*t is most likely that the cause o osteoporosis %ould!e postmenopausal in this %oman" !ut many medicalactors inuence the likelihood o this condition.

The following tests sho#ld be considered in apatient presenting with an osteoporoticfract#re (although not all need to !e perormed inall cases)< ull !lood count" renal 2 liver 2 !onechemistry" immunoglo!ulins and serum 2 urineelectrophoresis (to eclude myeloma)" thyroidunction" testosterone (in men)" investigations or+ushing's syndrome.

3=. This patient was trying to stand #p. ,ne%amination he has periorbital oedema with a


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faint p#rple h#e. The ser#m creatinine kinase9'): le!els were 1?-times the #pper limit ofnormal. 6hich of the following manifestationsis least likely to be associated with this

disorder(

A.

bl#e and white color change in the $ngerson e%pos#re to cold

*.

diNc#lty in swallowing liM#ids

'

.

do#ble !ision on looking to the sides

.

radial p#lse of 4? beats per min#te

". persistent co#gh8 haemoptysis and weightloss of 12 kg in the last three months

'orrect Answer/ '

'omment

This patient is not a!le to stand up rom a chairunaided. This indicates pro%imal m#scleweakness. The skin rash and the raised levels omuscle en=yme point to%ards an inammatorymyopathy" namely dermatomyosytis 9I:.A is easily recogni=ed and diagnosed !y a

characteristic rash


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"rythema of the kn#ckles accompanied !y araised8 !iolaceo#s scaly er#ption 97ottron0ssign: is also characteristic" and may precede orollo% muscle %eakness. +ayna#d0s phenomenon

%ith characteristic color change (pallor" cyanosis andru!or) on eposure to cold is encountered in patient%ith A" more oten %hen there is overlap %ith otherconnective tissue disease such as scleroderma ormied connective tissue disease.*nammation o the skeletal muscles o theoropharyn and upper oesophagus lead todysphagia especially or liuids in the initial stages

o the disease %hich progresses to diJculty ins%allo%ing solids as the disease progresses.9lectrocardiographic evidence o cond#ctiondefects and arrhythmias occur reuently inchildhood and adult A" although overt clinicalsymptoms are uncommon.

There is an increased incidence o underlyingmalignancy in adult A" ranging rom 68 to 168.

'arcinoma of the bronch#s8 o!aries and breastare the most common.,c#lar m#scles remain normal" even inadvanced" untreated cases" and i these muscles areafected" the diagnosis o inammatory myopathyshould !e in dou!t.

3;. A 34-year-old woman presents to yo#r

clinic complaining of cold hands8 partic#larly inthe winter months. ,n e%amination8 she hascold d#sky hands and a petechial rash.


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- normalO alb#min 3; gCO glob#lin ? gCOprotein electrophoresis - polyclonal increase ingammaglob#linsO antin#clear antibodiespresent 911;?8 speckled pattern:O complement

- '3 ?. 9J+ ?.=-1.2=:8 '4 ?.?4 9J+ ?.14-?.;:. 6hich of the following statements is tr#e(

A.

Acti!e systemic l#p#s erythemato#s 9C": is#nlikely if JA antibodies are present.

*.

A blood sample sent to the lab on ice mayshow cryoglob#lins.

'

.

ForgrenKs syndrome is #nlikely if

rhe#matoid factor is present.

.

ForgrenKs syndrome is likely if +o and Cae%tractable n#clear antigens are present.

". epatitis ' is #nlikely in this case.

'orrect Answer/

'omment

The symptoms and signs and low '4 ares#ggesti!e of cryoglob#linaemia.

0ludging o proteins at reduced temperatures (oreample hands on a cold day) can cause ischaemiaand sometimes vasculitis" particularly o skin orkidneys.

+ryoglo!ulinaemia is commonly associated %ithhepatitis + or connective tissue disease" such as0&orgren's syndrome. The positive ,/, and highglo!ulins suggest 0&orgren's !ut could also !eassociated %ith chronic inection" such as hepatitis +.


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0ince they precipitate at lo% temperatures"cryoglo!ulins should al%ays !e transported to the la!at COP+. ailure to do this %ill result in a alsenegative result as the cryos %ill precipitate and !e

removed %ith the clot.

3. A ;-year-old man with a 1?-year history of rhe#matoid arthritis8 controlled with diclofenacand s#lphasalaGine has reported rec#rrentheart b#rn and dyspepsia. is doctor askedhim to stop the dicofenac sodi#m and replacesit with celeco%ib 9one of the ',& 2 inhibitors:.

6hich of the following is tr#e abo#t ',& 2selecti!e agents(

A.

They eliminate the chance of non-steroidalanti-inHammatory dr#gs 9JA


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/0,*s inhi!it prostaglandin synthesis !y inhi!itingthe en=yme cyclooygenase (+DQ). The eistence ot%o diferent orms o +DQ isoen=ymes (+DQ1 and+DQ5) has no% !een recognised. +DQ1 has a

constitutive 'housekeeping' role and +DQ5upregulation is associated %ith inammation. The+DQ5selective /0,*s are not more efective thanordinary /0,*s" they are not the primary treatmentor rheumatoid arthritis" and they do not eliminatethe chance o /0,*associated dyspepsia. Knlikeaspirin and most traditional /0,*s" they have noantiplatelet efect" so they are not cardioprotective.

*n clinical trials +DQ5 selective /0,*0 reduced therisk o developing signicant upper gastrointestinalcomplications in the rst si months. -o%ever" therisk o 4* !leeding rom nonselective /0,*s is lo% an annual incidence o 18 to C8 and the +DQ5selective /0,*s are costly. Thereore" it may !e !estto reserve these drugs or patients at highrisk

patients to include those over @6 years" thosealready using medications kno%n to increase thelikelihood o upper gastrointestinal adverse events"those %ith serious comor!idity" and those reuiringprolonged use o maimum recommended doses ostandard /0,*s.

,ll /0,*s" including the +DQ5 selective agents" can

cause renal toicity" such as acute renal ailure"nephrotic syndrome" and acute interstitial nephritis.,cute renal ailure is a concern particularly inpatients %ith preeisting renal dysunction. /0,*sshould !e avoided also in patients %ith cirrhosis andascites or congestive heart ailure. eriodic


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monitoring o renal unction is recommended"particularly in elderly patients.

3>. A 3;-year old woman is referred with a 1-

year history of m#scle pain8 tiredness andsleep dist#rbance. he denies fe!er8 weightloss and arthralgia. "%amination re!ealstenderness o!er her occip#t8 trapeGi#s andl#mbar area. er blood res#lts show a normal"+8 '+P8 B*'8 a weakly positi!e AJA 1/>? andnormal complement. 6hich is the most likelydiagnosis(

A.

Polymyositis

*.

ystem l#p#s erythemato#s9C":

'.

FogrenKs syndrome

. Polymyalgia rhe#matica

". Bibromyalgia

'orrect Answer/ "

'omment

The lack o constitutional symptoms" normal

inammatory markers and normal eamination" apartrom evidence o tender points" make aninammatory rheumatological disease unlikely. Thepresence o tender points" history o muscle pain andsleep distur!ance are suggestive o $bromyalgia Ra noninammatory pain disorder.


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3. A 2=-year-old woman with a history of 3-second trimester fetal losses is planning a

fo#rth pregnancy. he has e!idence of theprimary anti-phospholipid syndrome 9stronglypositi!e cardiolipin antibody8 positi!e l#p#santicoag#lant b#t no e!idence of l#p#s:. 6hichof the following treatment regimens oer herthe best chance of ha!ing a s#ccessf#lpregnancy(

A

.

teroids alone

*.

teroids combined with low-dose aspirin

'.

Cow-dose aspirin alone

.

Cow-dose aspirin combined with low

molec#lar weight heparin". -year-old woman with ple#ritic chest pain. 6hich of


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the following statements concerning thispatient are tr#e(

A.

Cow white co#nt is consistent with systemicl#p#s erythematos#s 9C":

*.

Cow white co#nt is not consistent witht#berc#losis.

'.

The presence of anticardiolipin antibodies


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stairs and three days before admission she was#nable to comb her hair or feed herself.Je#rological assessment is diNc#ltb#t re!eals generalised grade 3= weakness8

and the *abiniski sign is positi!e bilaterally.6hich one of the following tests is most likelyto re!eal the diagnosis(

A.

Plain radiograph of the cer!ical spine

*.

"lectromyography 9"I7:

'.

Jer!e cond#ction st#dy 9J':

.


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decreased endurance" gait diJculty" paresthesias othe hands" and loss o ne deterity. Aultipleneurological signs may !e elicited on physicaleamination" including difuse hyperreeia" lo%er

etremity spasticity" a spastic gait" and Ba!inski'ssign.,lthough plain radiography o the cervical spine isregarded as the initial imaging assessment tool orneck pain in patient %ith #," those %ith symptoms orsigns o cord compression should undergo immediateA#* and !e sent or surgical consultation. A#* isconsidered the most sensitive imaging modality or

diagnosing spinal cord compression" evaluating itsetent" and assessing sot tissues (panus) as %ell as!one destruction.

-Penicillamine causes various neuromusculardisorders including myathenia gravis.

There is no clinical evidence or primary muscle

disease or peripheral neuropathy in this patien"hence 9A4 or /+0 are not necessary.*sotope !one scan may identiy increased activity inthe cervical region" !ut this is not specic and theinvestigation has little role in investigation osuspected spinal cord compression.

42. A ?-year-old woman with a history ofblood transf#sion in the early 1>?s presentswith a 1?-month history of malaise and isnoted to ha!e impaired renal f#nction. er#rine sediment re!eals red cell casts. Theres#lts of imm#nological in!estigations are asfollows/ ser#m


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-4.?:8 4


110/187

most important test in esta!lishing a denitivediagnosis in this patient.

43. A patient with psoriatic arthritis has acti!e Foints and tro#blesome plaM#e psoriasis. 6hichof the following will impro!e both the Foint andskin problems(

A.

#lphasalaGine

*.

ydro%ychloroM#ine

'.

7old

.

Iethotre%ate

". Penicillamine.

'orrect Answer/

'omment

ydro%ychloroM#ine can e%acerbate psoriasis.0ulphasala=ine tends to only improve &oint symptomsand not improve the psoriasis. 4old andpenicillamine are not commonly used to treat thiscondition.

44. A 4=-year-old woman de!eloped +ayna#d0s2 years ago. he now complains ofbreathlessness and skin tightness aecting her$ngers. A high-resol#tion 'T scan showse!idence of p#lmonary $brosis.


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6hich of the following tests is most likely to bepositi!e(

A

.

Anticentromere antibody

*.

Anti-do#ble-stranded JAantibody

'.

Anti-+o antibody

. Anti-cl-? antibody

". Anti-Do-1 antibody

'orrect Answer/

'omment

This patient has clinical symptoms suggestive odi#se c#taneo#s systemic sclerosis. ulmonary

!rosis and anti0clO7 are more common in patients%ith difuse disease. ,nticentromere anti!ody isassociated %ith limited cutaneous systemic sclerosis.,ntidou!lestranded /, anti!ody is associated%ith systemic lupus erythematosus. ,nti#o anti!odyis associated %ith lupus and primary 0&Hgren'ssyndrome. ,ntiNo1 is associated %ith polymyositis"particularly in patients %ith inammatory lungdisease.


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4=. A =>-year-old woman is referred with painand stiness in her hands and knees. he has afew patches of psoriasis on her arms. erhands are shown in the pict#re. 6hat are thetwo most likely diagnoses(

A.

7o#t

*.

Jodal osteoarthritis

'.

Pse#dogo#t

.

ystemic sclerosis

". Psoriatic arthritis

B. ystemic l#p#serythematos#s


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7.


.



114/187

.

'.

*ronchiectasis secondary to rec#rrentinfection

.

'omplement '; de$ciency

". moking = cigarettes per day.

'orrect Answer/

'omment

Antibody de$ciency is typically associated %ithrespiratory tract inections. ,sk a!out diarrhoea and!acterial skin inections %hich are also common. Takea careul drug history and !ear in mind the possi!ilityo lymphoprolierative disease.


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4. An infant presents with widespreadlymphadenopathy8 hepatosplenomegaly andfe!er one month after recei!ing a *'7 !accine.

A lymph node biopsy re!eals acid-fast bacilli.6hich 2 of the following components of theimm#ne system is it most important to assess(

A.

T-cells

*.

*-cells

'.

'3

.

Je#trophils

". Platlets

B.


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characterised !y gran#lomas composed of T-cellsand macrophages8 whose de!elopmentcritically in!ol!es interferon gamma8 . A ;=-year-old man is admitted as anemergency with a !ery hot8 swollen left knee.,n e%amination he is #nwell and pyre%ial. ehas marked loss of range of mo!ement8

secondary to pain. o# ha!e aspirated his kneeand ha!e sent the p#r#lent-looking H#id formicroscopy. 6hich of the following is tr#e(

A.

e is likely to ha!e acM#ired this infectionafter an arthroscopy or arthrocentesis.

*.

e!enty to eighty percent of cases will ha!ean accompanying bacteraemia.

'.

+es#lts of the c#lt#re sho#ld be awaitedbefore commencing antibiotics.

.

Antibiotics sho#ld co!er beta-haemolyticstreptococc#s and staphylococcus infections.

". A plain %-ray of the knee will con$rm thediagnosis.

'orrect Answer/

'omment

This is likely to !e septic arthritis. D!viously"eamination o the synovial uid %ill help to eclude


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diferentials such as gout and pseudogout. The mostlikely organisms are beta-haemolyticstreptococci 92?E: and staphlococci 9?E:. Antibiotics should !e started empirically to cover

these i the clinical suspicion is high. *deally theseshould !e intra!eno#s for 2 weeks and then oralfor 4 weeks. ,rthroscopy is a risk actor or septicarthritis" !ut is rare. Bifty percent of cases %illhave an associated bacteraemia. "arly %-rays arealmost al%ays normal.

4. A 2>-year-old man is has recently been

discharged from hospital after treatment forpne#mococcal pne#monia. e has hadrepeated co#rses of antibiotics for sin#s8 earand lower respiratory tract infections8 and hadsin#s s#rgery the pre!io#s year. e is a lifelong non-smoker and is not on medication. isblood co#nt prior to discharge was normal.


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".


119/187

.

Antin#clear antibodies 9AJA:

". Thyroid-stim#lating hormone 9T:

B. 6asp !enom skin prick test

7.

rinary catecholamines

.

Ci!er f#nction test 9CBT:


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follows/ ser#m '3 1.2 gl 9ref range ?.=-1.;=:8ser#m '4 ?.?2 gl 9ref range ?.2-?.;:. Themost likely diagnosis is/

A.

food allergy

*.

dr#g allergy

'.

? #C.


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6hich of the following statements is mostacc#rate abo#t this disease(

A.


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the middle ear ossicles" %hich dampens the motiono these ossicles.Knlike osteoarthritis" pagetic bone pain #s#allyincreases with rest" on %eight !earing" %hen the

lim!s are %armed" and at night. ,n estimated O78 o patients %ho have aget$s disease have nosymptoms. The diagnosis is typically oundincidentally on radiographs and la!oratoryinvestigations.

=3. A yo#ng patient is attending the

rhe#matology monitoring clinic. he de!elopsle#copaenia. 6hich of the following is #nlikelyto ca#se this abnormality(

A.

AGathioprine

*.

Iethotre%ate

'.


.

'yclosporin A

". "tanercept.

'orrect Answer/

'omment

atients taking aGathioprine8 methotre%ate andetanercept reuire regular monitoring o the ull!lood count to eclude marrow s#ppression.atients %ith 0?9 oten have a lo% %hite cell count.


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=4. A 2-year-old man s#ers a stroke ca#singleft sided weakness. e has !ery little!ol#ntary mo!ement of the left arm and needs

assistance to transfer. 6hilst on therehabilitation ward he complains of signi$cantpain aro#nd his left sho#lder. 6hich of thefollowing statements is correct(

A.

Poor handling and positioning of the left armby sta are #nlikely to ha!e contrib#ted tothe pain

*. ho#lder pain ca#ses distress b#t does notaect o#tcome

'.

A radiograph of the sho#lder is notnecessary

.

Treatment with simple analgesics is asensible initial approach

".


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eclude racture or dislocation. Treatment usuallystarts %ith simple analgesics or nonsteroidals.hysiotherapists may treat su!luation %ithstrapping.* the pain persists intraarticular steroid

in&ections and T9/0 may help.

=;. A >-year-old woman fo#nd it m#ch easierto manage after the occ#pational therapistpro!ides se!eral aids and appliances for #se inthe kitchen. Brom their appearance8 theproblem aecting her hands 9see image: is/

A

.

rhe#matoid

arthritis

*.

nodalosteoarthritis

' psoriatic


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. arthropathy

.

tophaceo#s go#t

". 'harcot Foints.

'orrect Answer/ '

'omment

This is the classic appearance o arthritis m#tilans in psoriatic arthropathy" %ith a deorming destructivearthritis o the small &oints. Dsteolysis results in

shortening o some o the digits. The pattern is notthat o rheumatoid arthritis and the %rists appear to!e spared. There is too much destruction orosteoarthritis. The appearance o gout may !esimilar" though the tophi %ould !e easier to see inlie (as opposed to a photo). +harcot &oints(neuropathic arthropathy) usually afect the oot andankle.

=. A =;-year-old man presents with rec#rrentattacks of polyartic#lar go#t despite treatmentwith allop#rinol 3?? mg daily for the last year.e is adamant that his compliance with hisdr#g treatment regimen is good.

6hich of the following factors is most likely toe%plain his poor response to allop#rinol(

A. 'onc#rrent treatment with

colchicine


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*.

Bast metabolism of allop#rinol

'

.

igh alcohol intake

.

Poor #rate clearance !ia thekidney

". igh dietary p#rine intake

'orrect Answer/ '

'omment

Persistently high alcohol cons#mption is acommon ca#se of poor response to allop#rinol"although the underlying mechanism o this is unclear.B" and 9 are plausi!le ans%ers" !ut are lessimportant in practice. Aost adults %ill respond toallopurinol C77 mg daily" although a small proportion%ill reuire @77 or even F77 mg daily. The aim otreatment should !e to suppress the serum uratelevel to the lo%er end o the normal range or &ust!elo%.

=>. A ;>-year-old woman complained ofprogressi!e pain in her left hip after slippingon ice and s#staining a s#b-capital fract#re ofthe left femoral neck hip. Bollowing reco!eryfrom a hemiarthroplasty8 bone mineral density

9*I: meas#rement of the opposite fem#rcon$rmed the diagnosis of osteoporosis with aT score of -2.;. he did not wish to takeoestrogen replacement therapy and was gi!enalendronate ? mg weekly. 6hich of thefollowing statements is tr#e(


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A.

Jormally the peak bone mass in a woman isachie!ed F#st before the menopa#se.

*

.

Plain radiographs are !ery sensiti!e in

identifying minor red#ction in bone mineraldensity and hence a !ery #sef#l screeningtest.

'.

The T score compares a patientKs *I withthe mean !al#e for yo#ng8 healthy ad#lts ofthe same se%.

.

A high ser#m phosphate le!el may be

s#ggesti!e of chronic alcoholism.". *isphosphonates s#ch as alendronate act by

stim#lating bone formation.

'orrect Answer/ '

'omment

Bone mass increases during childhood and

adolescence and peaks !et%een the ages o 57 andC7 years. eak !one mass is inuenced !y age" se"genetic actors" hormonal status" eercise" andcalcium intake. ,s %omen in general have a lo%erpeak !one mass than men" it is understanda!le %hyosteoporosis is predominantly seem in %omen.

lain radiographs are not sensitive enough to

diagnose osteoporosis. #esults o bone mineraldensity 9*I: tests are typically reported as Tscores and X scores.

The T score compares a patient's BA %ith themean value or young" healthy adults o thesame se.


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The X score compares a patient's BA %ith themean value or persons o the same age andse.

Both scores are epressed in terms o standarddeviations rom the mean. The Eorld -ealthDrgani=ation has dened osteoporosis as a BA o atleast 5.6 standard deviations (0) !elo% the meanvalue in young normal adults (i.e. TY-2.=:.

+hronic alcoholism is another important cause o lo%serum phosphate level" in %hich case the liver

en=ymes %ill !e elevated. All bisphosphonates actsimilarly on !one in !inding permanently tominerali=ed !one suraces and inhibitingosteoclastic acti!ity. Thus" they inhi!it !oneresorption and less !one is degraded during theremodelling cycle. They do not stimulate !oneormation.

=. A ;>-year-old man has lost weight. isalkaline phosphatase is raised at 2? C9normal range 3=-12?:. Plain radiographs showsclerotic lesions of bone.

6hat is the likely diagnosis(

A.

tomach cancer

*.

Prostate cancer

'.

I#ltiplemyeloma


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.

C#ng cancer

". ,steomalacia

'orrect Answer/ *

'omment

'ancer of the prostate is typically associatedwith sclerotic bone lesions in contrast to the lyticlesions seen in multiple myeloma. Dsteomalacia isassociated %ith ?ooser's =ones.

;?. A 33-year-old woman presents with a ;-month history of +ayna#d0s phenomenonaecting her hands and feet. he is pre!io#slywell and takes no medication.

6hich two of the following clinical feat#res orin!estigation res#lts are the strongestpredictors that she will de!elop a connecti!e

tiss#e disease in the f#t#re(

A.

Age S2= years

*.

Abnormal nail-fold capillarymicroscopy

'

.

"le!ated erythrocyte sedimentation

rate.

istory of rec#rrent miscarriage

". Bamily history of +ayna#d0s


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B. Anaemia

7.

trongly positi!e antin#clearantibody

.

istory of chilblains


131/187

'.

yperparathyroidism

.

Atheroma

". "pilepsy

B. "nteropathy

7.

'ardiomyopathy

.

'hronic renal

fail#re


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cell casts8 proteinZ8 AJA 1>?8 anti-JAnegati!e8 anti-"JA negati!e8 ser#m '3 1.?2 gl9J+ ?.=-1.;=:8 '4 ?.?2 gl 9?.2?-?.;=:8 '+P=mgl 9J+ =:8 creatinine 14= 9J+ =?-14?:.

6hich is the most likely diagnosis(

A.


*.


'.


.

Primary complement de$ciency

". '1 inhibitor de$ciency.

'orrect Answer/ '

'omment

This lady has a hypocomplementaemicglomerulonephritis (note active urine sediment) anda purpuric rash accompanied !y a positiverheumatoid actor and an isolated positive ,/,.

The com!ination o renal and skin involvement in thissetting %ith a markedly lo% + suggests miedcryoglo!ulinaemia. -er serology does not support0?9.

The key investigations %hich %ould help conrm thediagnosis %ould !e a %arm clo

Documents

Rhumatology masterclass 2010