內科部過敏免疫風濕科工作手冊intm.vghtc.gov.tw/imd/ch/過敏免疫風濕科工作手冊.pdf · 目錄第一章簡介 1-3 第二章教學訓練計畫 4-10 第三章

1-3

4-10

11-17

18-24

25-34

35-39

40-42

ANA 43-44

45-46

47-52

() 53-62

() 63-67

() 68-78

() 79-82

() 83-85

() 86-87

() 88-90

() 91-94

() 95-98

()

(1) 99-100

(2) 101-103

(3) 104-106

(4) 107-109

(5) 110-112

(6) 113-115

(7) 116-117

118-129

130-137

138-141

142-146

147-150

151-154

155-169

C 170-173

174-179

180-183

184-186

187-189

190-196

197-202

203-204

205-207

208-208

217-219

220-222

223-238

239-240

241-249

250-257

258-260

261-262

263-265

? 266-267

268-275

276-278

279-281

282-283

284-286

287-288

289-290

91 05 25

93 01 10

94 03 01

Editors

Contributors

(Chapter 11)

(Chapter 37,38,40)

(Chapter 6,20)

(Chapter 4,5)

(Chapter 14,16)

(Chapter 15)

(Chapter 17)

(Chapter 12)

(Chapter 32,43)

(Chapter 41,42)

(Chapter 39)

(Chapter 36,44,45)

1

20

4

(1)IFA assay: FIAX ANA fluorometer Fluorecent microscope

2

Inverted fluorecent microscope (2)ELISA: Reader Washer ELISA fully automation Sample handling system (3)FEIA system: Auto CAP system (4)DNA screening: PCR machine EP equipment Image system Incubator-shaker UV cross-linker Sequence equipment (5)Microcytotoxicity assay: Inverted microscope Seradot HLA analyzer (6)Flow cytometry (7)Nephelometry Behring BN II Beckman Image (8)Coagulation system ACL system (9)Polarized microscope (10)Pulmonary function test machine (11)Capillary microscope (12) Protein EP&IFE system Sebia hydrasys and hyrys system (13)West blotting equipment

3

(14)Cell culture system Laminar flow CO2 incubator Autoclave Oven(200C) (15)Ultrahighspeed centrifuge (16)Highspeed centrifuge (17)Refrigerated centrifuge (18)Cytospin (19)Spectrophotometer (20)PH meter (21)(ddH2O)

- 4 -

.

.

.

.

.

. C

.

.

.

.

,

- 5 -

C

:

:

:

ANA pattern recognition

HLA typing

Capillary microscope

- 6 -

1. Rheumatological medicine. Dieppe et al.

2. Textebook of rheumatology (Kelly, Harris, Ruddy, Sledge)

,,

7:30-8:00

19:00-21:30

14:00

18:00-19:30

8:00-12:30

9:00-10:00

*

16:00-17:30

14:30-16:00

,

,

9:00-10:00

16:00-17:30

14:30-16:30

- 7 -

()

Ex

cellent

Good

Fair

SLE

RA

5

: Excellent Good Fair Poor :

- 8 -

()

Ex

cellent

Good

Fair

DMARDs

Capillary

microscopy

SLE

RA

5


- 9 -

Ex

cellent

Good

Fair

X

ANA

Topic:

Journal:

Accepted or

published.


:

- 10 -

Ex

cellent

Good

Fair

CT/MRI

ANA

Topic:

Journal:

Accepted or

published.


:

11

1. ,,, , team: 20G, 1086G, 1219C, 1034A, 961F 892J

2. , ; take over, ; take over, .

3. : 1) progression notes 2) 3): PS: scheduled MTP pulse therapy, ES: Evaluation, B: Biopsy, RBx: Renal biopsy, LBx: Liver biopsy, SBx: Skin biopsy, F: Fresh case; , ()

4. W62 bedside image

5. //

Lupus nephritis 24 hours urine protein WBC/Hb/Platelet

6. / sign on. 7. Cellcept, Ciclosporin,

Ribavirin/ Interferon 8. call 1. Admission note

2. Progression Note

12

Admission note Progression, accept ion note/progression note cosign

3. Methylprednisolone (MTP) pulse therapyactive synovitis, skin rash, positive SI tests positive findings, MTP pulse, C.C admitted for MTP pulse

4. Special Chart

5. Progression Note SOAP 6. procedures: Arthrocentesis, Intraarticular injection, Tendon

injection, Lumbar puncture, CVP insertion, Effusion aspiration/tapping, Procedure Note Cell count, Sugar, Protein, ANA, Gram stain, Acid Fast Stain, KOH, Indian inkinterpretation (ex. Class I inflammatory synovial fluid)

7. Antibiotics, Anti-TB/Fungus agents, DMARDs: MTP, Endoxan, Imuran, Dapsone, Ciclosporin, Cellcept; high dose Prednisolone IndicationsProgression note TPR chart

8. diagnosis, ( antibiotics, HRCT of lung interstitial lung disease, Renal biopsy lupus nephritis)

9. primary care case , assign primary care case, ,

10. primary care case complaint ( CPR), ,, , ,

procedure,

13

1. underlying disease activity , fever antibiotics, immunosuppressants, , fever bedside , evaluation: CXR, Urine routine/culture, Blood culture, Sputum Gram stain , , (ex. Pneumonia, UTI, or Biliary tree infection, or Cellulitis) , TPR chart

2. SLE sulfa drugs flare up, Norcardia, Salmonella, Pneumocystic carinii Baktar/Bactrim sulfa drugs

3. iv form NSAID (ex Ketoprofen, Profenid) 4. prednisolone or DMARDs: Salazopyrin, Plaquenil, Imuran,

Endoxan, MTX, Ciclosporin, Cellcept, D-penicillamine dose

5. H2 blocker, PPI: Lansoprazole, Lipid-lowering agents: Simvastatin (Zocor)/ Benzalip, Albumin indications progression notes.

6. , ,

7. Trial Cellcept, Ciclosporin , CR.

8. Renal biopsy Tapal, Persantin, Coumadin , .

1. , Don't wait for result only. CNS: /, GI: /, Bone & Soft tissue: , Abd & Kidney Sono:() ,

2. Blood culture: Betadine tincture 3, () , , fever episodes 2, 15 30 vein blood culture 10ml blood, anaerobic bottle(), aerobic bottle, 5ml Bacterial Endocarditis case 5 blood culture, 24 persisted bacteremia

3. Gram stain: infection , sputum, synovial

14

fluid, abscess, pleural effusion. progression note. Sputum Gram stain

4. CRP, Ferritin, anti-PLT Ab, Heptoglobulin, anti-HCV Ab () .

5. , ( Dr), combine meeting.

orders 1. OPD immunology, 2. , CBC (

MCV) ( Liver function, renal function, Na/K/Ca/Cl, Fasting blood sugar; proteinuria or hyperlipidemia case TG/Cholesterol)

3. Routine X-ray examination Chest PA & KUB Fresh SLE ()

CBC, Urine routine, Stool OB, Whole set renal/liver function & electrolyte ESR, RPR, PT/Mixing APTT AIR Lab: ANA, DNA Ab, ENA, LE factor, C3/C4 or CH50() Acute phase protein IgG/IgA/IgM, RF-IgM, Lupus anticoagulant :ACA-IgG/ACA-IgM, ACA-IgA, AB2GPI, APTS

Lupus nephritis

Biochemistry TG/Cholesterol For renal biopsy cases: CBC & PT/APTT Check 24 hours urine protein/CCr/Urine IEP(AIR lab) Consult Nephro doctor for renal biopsy renal biopsy: light microscopy, Immonofloresent(IF), Electronic microscopy , biopsy. Biopsy kidney pathology application form Gallium renal scan(): biopsy Biopsy urine routine x1

Lupus cystitis lupus cystitis GU, Dr

15

Rheumatoid arthritis

CBC, Urine routine, Stool OB, Whole set renal/liver function & electrolyte ESR, PT/Mixing APTT AIR Lab: ANA, RF-IgM/RF-IgA(), acute phase protein, ENA:SSA/SSB, IgG/IgA/IgM Uncorrectable Mixing APTT lupus anticoagulant(LA) &:ACA-IgG/ACA-IgM, ACA-IgA, AB2GPI, APTS X-ray: hands/feet & active joint; , active joints

Spondyloarthropathy (AS, Reiter syndrome, psoriatic arthritis,

inflammatory bowel disease) CBC, Urine routine, Stool OB, Whole set renal/liver function & electrolyte ESR, PT/Mixing APTT AIR Lab: Acute phase protein, RF-IgM, HLA B27R , case X-ray: KUB, T-L spine, SI joint C-Spine ROM limitation : C-spine flexion/extension Bone scan

Hemolytic anemia

CBC(MCVRBC/Hb/Hct, Hct x100/RBC) Reticulocyte, PT/mixing APTT, LDH, RPR Coombs tests:Direct/Indirect () AIR Lab: ANA, Heptoglobulin, APS profile:LA (lupus anticoagulant) &:ACA-IgG/ACA-IgM, ACA-IgA, AB2GPI, APTS

Autoimmune hepatitis Check complete liver function profiles: PT, GOT/GPT/AlkP/Bil(T/D), rGT, Albumin/TP AIR Lab: IgG/IgA/IgM ASMA, AMA( AMiA,) APA; AMA positive ama-m2

Lupus Abdominal vasculitis Standing CXR, KUB,

16

Abdominal CT small intestine , R/O abdominal vasculitis ( iv contrast) sucussion splash/post-prandial vomiting: GI for UGI-scopy, (mention duodenum 2nd portion) tenesmus/watery or bloody diarrhea: CRS or GI for Sigmoidoscopy

CNS involvement Lumbar puncture: Cell count, Glucose ( blood sugar), Protein Gram stain/Bacterial culture(aerobic & anaerobic) Cryptococcal Ag/Indian ink/Fungus cuture AFS/TB culture, AIR Lab:TB PCR CSF IgG index ( 3ml blood, CSF AIR Lab) CSF ANA MRI of Brain ( contrast), HMPAO-SPECT

1. //,

2. , 3. , CR VS, .

4. , CR VS, "" CR or VS.

5. , ; , Ditto

6. Gouty arthritis Allopurinol & Benzbromarone, 24Hrs urine Uric Acid underexcretion type .

7. Myofascial pain NSAID, ointment ( well documented)

8. NSAID cases benzoflex muscle relaxant, . 9. URI Danzen SLE:

S/Ss: Malaise? Fever? Hair loss? Skin rash? Arthralgia? Lab: CBC, U/R, DNA, C3/C4 or CH50 ( 3)

RA S/Ss: Active synovitis/Deformity, Morning stiffness?

17

Lab: WBC, Hb, Platelet, ESR/CRP, U/R, Cr, GPT, RF-IgM/RF-IgA (,), 2-3.

X-ray of both hand/feet Active 6 Active synovitis DMARDs, fellows SAE: AS, Reiter's, Psoriatic arthritis

S/Ss:Buttock pain/Lumbasacral pain/Cough pain/Sneezing pain/Inguinal pain/Neck ROM Hip internal rotation limitation/Sausage toes/fingers, active synovitis Lab: CBC, ESR, CRP, Cr, U/R (For NSAID nephropathy) 3 1 X-ray of KUB/SI joint (), T-L spine (C-spine) oligo-/poly-"active synovitis over , seronegative RA still should be suspected" 3 RF-IgM check X-ray F/U, miss seronegative RA.

Gouty arthritis S/Ss: acute attack ? uric acid, Cr, U/R? benzbromarone 24hrs urine uric acid ? synovial fluid finding prove Gouty arthritis? revise diagnosiscrystal arthritis Lab: 3 check uric acid, Cr, U/R

- 18 -

(-)

() (1)

- 19 -

(2)

(3)"" ""

""

(4)

Reiter

() (1)

- 20 -

(2)

(3)

(4) LymeB C Lyme DNA B19

Lyme (5)

(6) Crohn

(hypertrophic osteoarthropathy)

(7) () hydralazineprocanamide

"statin" ( NSAIDs Gold D-penicillamine)

- 21 -

()

Marfan

()

()

(Health Assessment Questionnaire, HAQ)(Arthritis Impact Meseaurement Scale)

()

- 22 -

()

(1)

(2) ()

(rotator cuff)

(3)

(trigger Points)(referred Pain)

(4)

(5)

- 23 -

(6)

() (stiffness)

(polymyalgia rheumatica)

() (swelling)

() (weakness)

() (constitutional symptoms)

38.5C

() (Raynauds phenomenon)

- 24 -

CREST

1. Kelly, Harris, Ruddy, Sledege, Textbook of Rheymatology, 5th Ed., Saunders

2. Klippel, Dieppe, Rheumatology, 2nd Ed. Mosby 3. Simons, Travell, Myofascial pain and dysfunction the trigger point

manual, 2nd Ed. Williams & Wilkins 4. , ,

- 25 -

Doherty GALS 3-4GALS

() GALS

GALS (Gait, Arms, Leg, Spine)

(1) (2) (3)

GALS

- 26 -

GALS /

,

/

- 27 -

() (1)()

(2)

(3) 45 45 45

60 (4) 70-80 20-30 25-35

35-45

() (1)

(2)

- 28 -

(2) 90

90 45 160 60

(3) 90 60 55

90 120 45 75

(4)

() (1) 50 10-15

45

(2)

- 29 -

(3)

150 0 5

(4) 90

() (1)

123

(2)

70 80 30 20

()

(1)

Marfan

- 30 -

(2)

(Dupuytren's contracture)

(3)

(ulnar deviation)

Heberden

() : (1)

60% 40% (2)

(antalgic gait)

(3)gluteus medius Trendelenburg

- 31 -

5-10cm

(4)

(5) 90-120

(6) 90

30-40 40-60 30-40

(7)( 10-15 )( 30-50 )( 30 )

() :

(1)

"X " "O "(hamstring)

(2)

(3)

- 32 -

(4)()()()

(5)

(6)

() : (1)

(2) 20 45

30 20

() : (1) (hammer toes) (hallux valgus)

(2)

- 33 -

(3)(MTP) Morton

() (1)

(2) 10 cm 10 cm

() (1)

(2) (3)

(4)

- 34 -

(5)

()

(1)

(2)




- 35 -

RF 1% RF 33.1% RF 0.3%30% RF 95.5%RF 9.7%

5% 14%23% 30%

(acute phase reactant)

- 36 -

C(CRP)(ESR)ESR (giant cell arteritis)(polgmyalgia rheumatica)ESR 50mm/ 10%ESR ESRRSR ESRHenoch-scholein ESRESR(Spondyloarthropathy)

(ANA)ANCAHLA (lupus anticoagulant)

DNA

- 37 -

(Antinuclear Antibodies) Sm

DNA 95%

Ro (SSA) CH50 Ro

(Antineutrophil cytoplasmic antibodiesANCA) ANCA Wegener

ANCAANCA (c-ANCA) (p-ANCA)c-ANCA proteinase 3 (PR3)Wegener 70%-90% p-ANCA myeloperoxidasc (MPO)

p-ANCA MPO (inflammatory bowel disease) ANCA ANCA

- 38 -

(Rheumatoid factor) G Fc

75%-90%

B27 (HLA-B27) HAL-B27

95%80%50%70% HAL-B27 6.5% HLA-B27 HLA-B27

(complement) CH50 (Nephelometry)

C3C4 CH50 C1C2

ANA ANA Ro(SSA) CH50

- 39 -

RF

: 1. Doherty M, Hazleman BL, Hutton CW, Maddison PJ, et al. Rheumatology

Examination and Injection Techniques. (Second edition) London), W.B. Saunders, 1999

2. Cash JM. Evaluation of the Patient. A. History and Physical Examination. Primer on the Rheumatic Diseases. 11 edition Arthritis Foundation (Georgia), 89-94, 1997

3. Shmering RH, Liang MH. Evaluation of the Patient. B. Laboratory Assessment. Primer on the Rheumatic Diseases. 11 edition. Arthritis Foundation (Georgia), 94-97,1997

- 40 -

C

IgG Y(Fab) IgG Y IgG (Fc)

IgG IgG

- 41 -

()

1. : 80% 20%

2. :

15 8%

()

1. 30% 80%

2. :

()

1. C:

C80%

- 42 -

2. :

()

()


2. Klippel, Dieppe, Rheumatology, 2nd Ed. Mosby 3. , ,

43

ANA

- 45 -

BC BC

() Blood type compatible:

O, A, B, AB A A, AB B B, AB AB AB

() HBs (+) donor recipient HBs (+), or anti-HBs (+), or anti-HBc(+)

[HBs (+) donor cannot be given to HBs (-), anti-HBs (-), anti-HBc (-) recipient]

() Anti-HCV (+) donor anti-HCV (+) recipients

- 46 -

:

UNOS

() UNOS

Level

( 0.5) 0.5

HLA

0ABDR mismatch 0BDR mismatch 0AB mismatch 1BDR mismatch 2BDR mismatch 3BDR mismatch More mismatch

10 7 6 3 2 1 0

>=80%

- 47 -

,

- 48 -

butterfly rash of SLE acne rosacea lupus pernio lupus vulgaris seborrheic dermatitis

discoid rash bullous LE reverse Gottron sign digital and palmar vasculitis

.Gottron sign, Shawl sign, mechanic hand

.sclerodactyly, digital pitting, telangiectasis, calcinosis

.nail pitting, salmon patch, onycholysis

.:rheumatoid nodules

. livedo reticularis atrophy blanche purpura, palpable purpura erythema nodosum Raynauds phenomenon (two phase, three phase)

- 49 -

() (Trapezius) : (Sternocleidomastoid muscle) :

(Levator scapulae muscle) : ? (Splenius capitis and splenius cervicis) : ( Posterior cervical muscles : multifundi, semispinalis) :

( Suboccipital muscle ) : (Temporalis muscle) : : () (Scalene Muscles):

(Supraspinatus Muscle):

(Infraspinatus Muscle):

(Teres Minor): (Latissmus Dorsi Muscle): (Teres Major):

- 50 -

(Rhomboideus Muscles): .(Deltoid Muscle):

(Pectoris Major & Minor): .(Sternalis):

(Serratus Anterior, Posterior): () (Biceps brachii):

(Hand extensors):

(Quadriceps femoris):

(Hamstring muscle):

(Gastrocnemius muscle): (Soleus muscle): () (Thoracolumbar Paraspinal Muscles) (Quadratus Lumborum) (Iliopsoas Muscle) (Gluteus Minimus) (Gluteus Medius) (Gluteus Maximus),

(Pyriformis muscle)

() ()

- 51 -

Goodpastures Wegerners

()

()

(Tubulointerstitial)

(ANCA)

( NSAIDs, Gold D-penicillamine)

()

()

- 52 -

()

()


2. Klippel, Dieppe, Rheumatology, 2nd Ed. Mosby 3. Simons, Travell, Myofascial pain and dysfunction the trigger point manual,

2nd Ed. Williams & Wilkins 4. Daniel J. Wallace, Bevra Hannahs Hahn, Edmund L. Lupus Erythematosus

Dubois, Dubois' Lupus Erythematosus, Lippincott Williams & Wilkins Publishers

5. , ,

- 53 -

()

( SLE) 75/100,000SLE

SLE

SLE SLE

SLE

SLE(bullous) Ro (SSA)(subacute cutaneous lupus erythematosus, SCLE) (discoid lupus)(hypertrophy)(verrucous DLE)(lupus profundus)SLE 30% 10% SLE

SLE SLE 20%

- 54 -

(Raynauds phenomenon) 30% SLE

SLE

SLE SLE SLE

Jaccoud deformity ischemic necrosis of bone avascular necrosis SLE 4%~10%SLE SLE

X X

SLE SLE

SLE (Cushingoid syndrome) 85% SLE 45%

- 55 -

: 1.

Zizic prednisolone 40mg prednisolone 30mgprednisolone 20mg 5% SLE

2.

3.

5%~11%

SLE

15%(cytoid bodies) (macular degeneration) SLE SLE(central retinal artery occlusion)(certral retinal vein thrombosis)(optic neuritis)(proliferative retinopathy)

- 56 -

SLE

SLE14~56%5~17% 14~46% SLE

6~45% 21~49% 60~83%

10% SLE (mitral valve prolapse) SLE SLELibman-Sacks

SLE

SLE

42~60%16~40% X(lupus pneumonitis) (fibrosing alveolitis) (alveolar hemorrhage)(pulmonary embolism)(pulmonary edema):

Gallium

- 57 -

3gm%SLE

SLE

2~6%

8~10% SLE (pseudopyloric obstruction);(rebound pain)(board like rigidity) X

SLE

SLE

(malabsorption) X

SLE

10~30%30%~60%

(lupoid hepatitis), (autoimmune hepatitis)

- 58 -

B Cm

SLE

SLE

(lupus cystitis)(hydroureter)(Hydronephrosis) X (sacroilitis)

SLE

SLE

38%~65% SLE SLE5~16% SLE

;

(creatinine)

C3 double strand DNA

5 10 86% 75%

- 59 -

85%

SLE

SLE

NSAID SLE

mononeuropathy multiplexacute psychosis

SLESLE

SLE SLE

SLE

- 60 -

SLE

2mg/dl

SLE C3 C3 C3SS-ALupus auticoagulant

Aspirin SSARo

SLE

SLE

SLE

16% Coombs

5%(ITP) SLERo (SSA) 5 SLE

- 61 -

1982 , ()()()()(Smithdouble strand DNA)

SLE DR2

SLE

SLE SLE

- 62 -

1. Kelly, Harris, Ruddy, Sledege, Textbook of Rheymatology, 5th Ed.,

Saunders 2. Klippel, Dieppe, Rheumatology, 2nd Ed. Mosby 3. Simons, Travell, Myofascial pain and dysfunction the trigger point

manual, 2nd Ed. Williams & Wilkins 4. Daniel J. Wallace, Bevra Hannahs Hahn, Edmund L. Lupus

Erythematosus Dubois, Dubois' Lupus Erythematosus, Lippincott Williams & Wilkins Publishers

5. , ,

- 63 -

()

:

: 1 2 3 4 5

:Paracetaminophen NSAID :NSAID :NSAID

- 64 -

:

: nifedipine :NSAIDs : : Danazol

() : : :Propranolol

"""" () (NSAIDs)

NSAIDs Salicylate NSAIDsNSAID NSAIDs NSAIDs(corticosteroid) NSAID NSAIDs NSAIDs

(prostaglandin) NSAIDs NSAIDs NSAID NSAIDs

- 65 -

NSAIDs Ibuprofen

() (Corticosteroid)

5mg prednisolone prednisolone NSAIDprednisolone

methylprednisolone :

prednisolone prednisolone Methylprednisolone

- 66 -

() (Antimalarials)

() (Immunosuppressive drugs)

Chlorambucil cyclophosphamide Azathioprine cyclophosphamide1.0~4.0mg/kg/day 0.5~1.0gm/m2 60 24 Chlorambucil 0.1~0.2mg/kgAzathioprine 1.0~4.0mg/kg

- 67 -

() (Plasma exchange)

40ml/kg5%


2. Klippel, Dieppe, Rheumatology, 2nd Ed. Mosby 3. Daniel J. Wallace, Bevra Hannahs Hahn, Edmund L. Lupus


4. , ,

- 68 -

()

SLE

24 C3DAN

- 69 -

585%

BBTBDNADNA (in situ formation)

WHOWHO

1. ()

2. ()

- 70 -

3. ()()

(cresecents)(fibrinoid necrosis) (pyknosis)( karyorrhexis )

(wire loop)(hyaline thrombi) (hematoxylin bodies) (LE body) LE

(membranoproliferative)

4. ()

IgGIgAIgM IgG C3C4C1q properdin C1q

- 71 -

(fibrin) (fibrinogen)

(tubuloreticular inclusions)

(tubulointerstitial)

CD8

(noninflammatory necrotizing vasculopathy)(thrombotic microangiopathy)(necrotizing vasculitis)

(activity and chronicity index)

03+024(endocapillary proliferation)(fibrinoid necrosis)

- 72 -

(karyorrhexis) ( cellular crescent)(interstitial|inflammation)2 03+ 0 12(glomerulosclerosis)(fibrous crescents)(tubular atrophy)(interstitial fibrosis) 124

WHO

: (1) WHOWHO

(2) WHO

: (1) WHOWHOWHO

(2) WHOWHOWHO

- 73 -

(1)

(2)

(3)

(4) (5)

(6) (7)

1.

2.

3.

GFR 4.

- 74 -

GFR

5.

GFR

1. ()

2. ()

3. ()

cyclophosphamide azathioprine prednisolone cyclophophamide NIH methylprednisolone cyclophosphamide () cyclophosphamide cyclophosphamide () prednisolone methylprednisolone (creatinine )25% cyclophosphamide 25% cyclophosphamide 10%

- 75 -

cyclophosphamide cyclophosphamide 24

4. ()

ciclosporine

double strand DNA C3C4 DNA C3C4 24C

SLESLE 1/4

- 76 -

SLE

2mg/dl

prednisolone15mg

Azathioprineciclosporinecyclophosphamide

40012

14

- 77 -

1.

2.

3.

1. 461-466

2. Wallace DJ, Dubois: Dubois lupus erythematosus, 3rd ed. Philadelphia: Lea and Febigrer, 1997

- 78 -

WHO ()

A. B.

WHO

I.() II. A. / B. /

III. A. B. C.

IV. A. B. C. D.

V. A. B.IIaIIb

VI.

- 79 -

()

23

HMPAO

HMPAO

SLE

NSAID SLE

mononeuropathy multiplexacute psychosis

(cognition dysfunction)

- 80 -

(

) NSAIDs

1. 2 6

2.

3. PET(positron emission tomography)

PETHMPAO SPECT (single-photon-emission computed tomography)

1.

- 81 -

2. SLE

3. SLE

CSF IgG index 4. HMPAO

5. MRI

6. PET

cyclophosphamide, azathioprine, cyclosporine , Aspirin,

(transverse myelopathy)(mononeuropathy multiplex) acute inflammatory demyelinating polyneuropathyAIDP, chronic inflammatory demyelinating polyneuropathyCIDP

cyclophosphamide

- 82 -

1. (hydroxychloroquine)

2. DHEA 3. 4.

5.

1. Daniel J. Wallace, Bevra Hannahs Hahn, Edmund L. Lupus Erythematosus Dubois, Dubois' Lupus Erythematosus, Lippincott Williams & Wilkins Publishers



- 83 -

()

azathioprine

- 84 -

(migratory pain)

succussion splash

(pseudopyloric obstruction)

peritoneal sign

X

(Double contrast

Abdominal CT scan)

- 85 -

""

cyclophosphamide

(Endoxan)




- 86 -

()

76

DNA

hydralazine()procanamide()methyldopa()isoniazide()chloropromazine()D-penicillamine()

1. 2. 60( 86)

- 87 -

3. 4. 5.




- 88 -

()

1 : 9

1.

2.

LH, FSH

3.

- 89 -

4.

1.

2.

3.

(Plaquenil)

- 90 -

4. (Endoxan)




- 91 -

()

SSA 45%SSB 15%98% SSA SSB SSA SSB 6 8

20% 3%-5%

SSA SSBSSA SSB 40%

()

- 92 -

() :

()

() ()

() (AV block)

18 24

30 82% 30 18% SSA SSB

20% SSA SSB

SSASSB SSA 60KDa 52KDa SSA 48KDa

- 93 -

? SSA 52KDa SSB 48KDa

SSA/SSB

SS(Sjogren Syndrome) SSASSB SSA SSB

: 1. SSA 52KDa SSB 48KDa

2. 1820212223 24 24

:

1.

2. Dexamethasore 4mg

3. 4.

- 94 -

1. Daniel J. Wallace, Bevra Hannahs Hahn, Edmund L. Lupus




- 95 -

()

minocycline() (Drug-induced lupus) 1951 hydralazine

hydralazine () procanamide () methyldopa ()isoniazid ()chlorpromazine ()D-penicillamine () 90% 9%99% 1%

1.

- 96 -

hydralazineprocanamidemethyldopaD-penicillamine

2. isoniazid (INH)chlorpromazine (Thorazine) sulfasalazineQuinidineCarbamazepine ()griseofulvin ()(minocycline)

3. (phenytointrimethadioneprimidoneethosuximide) (captopril , prazosin) (propylthiouracilmethimazole) (practololacebutololatenolollabetalol) (mevacorlopidpravachol)

1. 2. 60 30

3.

hydrazine procanamide quinidine minocyclineminocycline methyldopa

- 97 -

: IgG(H2A-H2B)-DNA

hydralazinequinidine minocycline

1.

2.

3. 4. IgG(H2A-H2B)-DNA

:

1. 2.

3.

:

- 98 -





- 99 -

() (1)

1. :

(1) (2) (3) (progesterone)(progesterone)

(1)

2.

3. ()

: 1960

- 100 -

(tartrazine)

(estrogen)(ethinyl estradiol) 35(progestogens)

(1) (2) (3) (4) (5) (6) (7) (8)





- 101 -

() (2)

1: 9

: 1.:

2.: (Endoxan) 24 20%-50% 30 80%

- 102 -

3.:

: 1. (mg%) 80%

2. 3.

4.

1. 2. :

heparin Aspirin

3. SSA/SSB Ro/La

Dexamethasone

- 103 -




- 104 -

() (3)

2~6

()

: 1. : 2. :

- 105 -

3. :()

4. :

15~30 1~2 5

(Dental floss)(Dental tape)(Rubber tips)(interproximal) 3

25%

!


Erythematosus Dubois, Dubois' Lupus Erythematosus, Lippincott

- 106 -

Williams & Wilkins Publishers 2. , ,

- 107 -

() (4)

1992 Petri

21%31%(cephalosporin)57% 14% 2.6 1.8

1976 Gold man IgE1985 73% 37%SLE

? 1989 WHO ()

- 108 -

ciprofloxacin

40%

Amoxicillinampicillin

1.

2.

3.

- 109 -

4. 2 Amoxicilin 4-6

5.




- 110 -

() (5)

2714

X

10-15%

- 111 -

80% 1.

2. 3. 4. 5.

X

X

1.

2.

- 112 -

1. 2. 3. 4. 5. 6. 7.




- 113 -

() (6)

---(premature atherosclerosis)

5 30% 40%

15-30

1.

- 114 -

35 2.

(HDL-C) A1(APO-A1) 1

3.

10 prednisolone

4. 5.

6. 7. (homocystine) 8.

35

- 115 -

1. 2.

3. (hydroxychloroquine)

4.

5. B12B6




- 116 -

() (7)

- 117 -





- 118 -

(antiphospholipid syndrome)

12 HLA HLA

- 119 -

()

(1)

(prostacyclinPGI 2)

A2 (thromboxane A2) A2

III (antithrombin III) (thrombomodulin) (2)2GP1

2 1 (2GP1)

2 1 (3)

1 (placental anticoagulant 1)

1

()

(1)

- 120 -

30% 70%

(2)(thrombosis)

(3)

(Budd-Chiari )

(4)

(5)

- 121 -

(6) (catastrophic antibody syndrome)

()

(7)

(Libman-Sachs)

() 1988 Asherson 1989 Alarcon-Segovia 1998

1952 Moore SLE

FeinsteinRapaport

Harris

- 122 -

(cardiolipin) ELISA

1990 2 1 (2 GP1)2 GP12 GP1

(heparin) () (BFP-STS) VDRL

5% - 19%

() (lupus anticoagulant) 1972 Feinstein Rapaport

IgG IgM

(APTT) (KCT) (RVVT) KCT

2 GP1 C S () (antiphospholipid antibody) (anticardiolipin

antibody)

ELISA IgGIgAIgM

- 123 -

2 GP1X ELISA 2 GP1

1. 2.

3. 4.

5 Lediden C S III

(dysfibrinogenemia) Burger

- 124 -

1.

warfarin INR

2. 75heparin

warfarin 3.

dapsone dapsone 4.

- 125 -

1.Harris EN, Baguley E, Asherson RA, Hughes GRV. Clinical and

serological features of the antiphospholipid syndrome (APS).Br J Rheumatol, 1987,26:19

2.Bick RL. The antiphospholipid thrombosis( APL-T) syndromes Characteristics and recommendations for classification and treatment. Am J Clin Pathol, 1991,96:424

3.Asherson RA, Cervera R. Antiphospholipid antibodies and the heart: Lessons and pitfalls for the cardiology, Circulation. 1991,84:920

4.Asherson RA ,Cervera R. Primary, 'Secondary' and other variants of the antiphospholipid syndrome. Lupus, 1994,3:293

5.Hinton RC. Neurological Syndrome associated with antiphospholipid antibodies. Semin thromb Hemost, 1994,20(1):46

6.Cervera R, Asherson RA, Lie JT. Clinicopathologic correlations of the antiphospholipid syndrome. Semin Arthritis Rheum, 1995,24(5):262

7.Lockshin MD. Answers to the antiphospholipid antibody syndrome. N Eng J Med. 1995,332:1025

8.Donato Alarcon-Segovia, Cabral A R. The antiphospholipid/cofactor syndrome. J Rheumatol. 1996,23:1319-1321

9.Harris EN, Pierangeliss, Birch D. Anticardiolipin wet workshop report: 5th International Symposium on Antiphospholipid Antibodies. Am J Clin Pathol. 1994,101:616-624

10.Khamashta MA, Cuadrado MJ, Mujic F, et al. The management of thrombosis in the antiphospholipid-antibody syndrome. N Engl J Med 1995,332:993-997

11.Khamashita MA : Hughs Syndrome Antiphospholipid Syndrome. London : Springer-Verlag London Limited, 2000

- 126 -

. (1998Asherson) 1.

2. 3. 4.

1. IgG APL(/) 2. IgM APL(/) 3.

1. 1 1 2. APL 3. 5 SLE

- 127 -

. (1989Alarcon-Segovia)

(-) 1. 2. 3. 4. 5. 6. 7.

()(IgG IgM>5 SD)

(IgG IgM2-5 SD)

- 128 -

.SAPORO(1998)

1. 2. 3.

1.

2. 34

3.

(Anticardiolipin Ab) (aCL)

1.IgG/ IgM aCL

( ELISA2 -I)

1.

- 129 -

2.Activated partial thromboplastin timeKaolin clotting time, diluted Russel Viper venom time

3.

4.

5.

- 130 -

(Rheumatoid Arthritis, RA)

(DMARDs)

(RA)

EBRetrovirus, parvovirus rubella

(molecular mimicry)

(superantigen) HLA-DR T

- 131 -

()

10

()

PIPMCP

Baker's 12

Swan-neck deformity, PIP hyperextensionDIP hyperflexion Boutonniere deformity, PIP flexion DIP extensionZ deformity Radial deviation ulnar deviation. phalanges palmar subluxationMallet deformity, DIP flexion hallux valgus lateral deviation dorsal subluxation

()

1. (Rheumatoid nodule)

extensor surface 20 30

2.

- 132 -

3.

4.

5.

Amyloidosis 6.

( 1987 ) 1. 2. 3. 4. 5. 6. 7. X

1990

- 133 -

():

88.9% 93.3% 1. 15 2. (

) 3.

4. 5. 6. X

()

1. : IgG IgG IgG

C

71%

2. :

- 134 -

1:160

3. : IgG 40%

4. HLA: HLA-DR4(040104050406) HLA-DR4 HLA-B27

5. :

6. 2003

C 2mg/dl HLA-DR4

- 135 -

()()()

()()

()

()

1.

COX2

2.

prednisolone 7.5mg

- 136 -

7.5mg

4. Hydroxychloroquine

(SulfasalazineMethotrexateMTXAvaraLeflunamide

ACyclosporin A

5.

Enbrel

(DMARDs) 1 3 2 3 4 40mm C 1.0 mg /dl

- 137 -

()

()




- 138 -

(Progressive Systemic Scleroderma, PSS)

(Scleroderma)

30-50 3-4

;

()

(1):

- 139 -

(2):

(3):

(4):

()

(1):

(2)

(3)

- 140 -

(4)

1.:

-70 (Scl-70 antibody) (Nucleolar

antibody)(Centromere)

2. : (Capillary

microscopy)

( D-penicillamin,

Cyclosoporin )

():

PPI

- 141 -

(): (Acute fibrosing alveolitis)

(Interstitial lung disease)





- 142 -

, 2-3:1, 10-15 45-55

....

(anti-SRP)(antisynthetase), picornavirus, coxackievirus 131, encephalomyocarditis virus-221A

()

IgGIgMC3C56-C9

Arahata Engel

B

- 143 -

perifascicular atrophy

,

(endomysial infiltration),

T B, CD8+T,

CD4+T B

()

1)

2)

3)

4)

5)

,

,

- 144 -

--Gottron

--(Heliotropes rash)(V-sign )(Shawal sign)(Mechanics hand)(Periungal erythema)(dilated capillary loops)....

(subcutaneous calcification)

8%

(Amyotrtophic dermatomyositis)

(GOTCPKLDHAldolase) CPK 34

56

triad 1) increased insertional activity, fibrillations, and sharpe positive waves; 2) spontaneous, bizarre, high-frequency discharges

; 3) polyphasic motor-unit potentials

- 145 -

of low amplitude and short duration

Anti-Jo-1 20% aminoacyl-tRNA synthetaseanti-Jo-1 histidyl-tRNA synthetase(anti-PL-7anti-PL12amti-EJantiOJ) Anti-Jo-1 anti-Jo-1ANAAnti-SRP (signal recogniton particle)

5 30%Anti-Mi-2 510%

Anti-PM-Scl Anti-Mi-2 (5 95%) Anti-PM-Scl (5 95%)

IgGIgM Ig

C SLE

prednisolone, 1-1.5mg/kg/day 1-1.5mg/kg/day()

azathioprine, methotrexate, cyclophosphamide, cyclosporine,cellcecpt, chlorambucilHydroxychloroquine 3(methylprednisolone) 15mg/kg/mg (750-1000mg/day)(IVIG(plasmapheresis)

- 146 -

- 147 -

(Raynauds Phenomenon)

?

15-40;

()

- 148 -

: () () ()

?

()

: 1. 2. 3.

4. 5. 6. 7. 8.

9.

10. 11. 12.

- 149 -

13. 14. 15. 16.

()

1. 2. 3. ok

4. 5.

6. 7.

8.

()

()

(calcium channel blocker)(nitrate)

- 150 -

()



- 151 -

(mixed connective tissue disease, MCTD)

(RNP)

8 15

()Raynaud phenomenon

scleroderma ()Swollen fingers/toes/hands/feet

/(sausage finger/toe)()

() SLE RA

()

- 152 -

10-20%

()

Scleroderma (DLCO )MCTD

() Scleroderma 65%

() 20% 10-30%

()

SLE ()

MCTD 25%U1-RNP

scleroderma SLE

()Skin and mucous membrane sclerderma SLE(typical malar rash or discoid rash)(periungal erythema)

Achilles

- 153 -

() 75% 60% Coombs test

SLE

overlap syndrome

() CBC/DCESRCRPCPKAldolase

() ANAENA (SSA/SSB/RNP/SM) U1 RNP

() XEMG/NCVcapillary microscopy

steroid

(SLE scleroderma RA) ()

90.5 % 82.1%

( 35%)

- 154 -

1. , , , 2002; 50:376-380 2. Kelly, Harris, Ruddy, Sledege, Textbook of Rheymatology, 5th Ed.,

Saunders 3. Klippel, Dieppe, Rheumatology, 2nd Ed. Mosby 4. Simons, Travell, Myofascial pain and dysfunction the trigger point


- 155 -

(Behets Disease)

1937 Behcet(Behcets disease)(Behcets syndrome)(ocular-oral-genital syndrome)

""

80-370/10 13.5-20/10 14/10 0.12-0.33/10

1992 30.5/10 9.9/10 3

1982 310 89%

30 40

- 156 -

65%

()

HLA-B5 B51 HLA-B51 72%, 26% HLA-B51 57% 12% 53.3% HLA-B51 10.9% HLA HLA B51 81% 13%HLA B51 HLA-B51

()

1. : EB

Cparvovirus B19

2. :

heat shock protein 3. :

- 157 -

1950

() :

1. : 60%

2. :

C9 CH50 C3 (A) T :

T

(B) : :1; 2

; 3 3. :

PGI2

- 158 -

()

()

()

()

- 159 -

()

24-482-10cm 1-2

() 70%24-48

1-3

()

60-80%

1. :

6

- 160 -

5

2. :

4

3. :

4. :

5. :

(parthergy reaction)

(A) :

- 161 -

(B) :

(C) :

48 ; 48 62.2% 4% 0 75% 10%HLA-B51

:1 48 ; 2; 3

()

1/3 2/3

- 162 -

()

(neuro-Behcets disease) 30% : 1; 2 1-3 ; 3; 4

; 5 ;

()

(vasculo-Behcets disease, angio-Behcets disease) 10-37%

()

- 163 -

()

C 2

VIII

ENA X

CT

1987 ()

- 164 -

1987 1.

(1) (2)

(3) (4)

2. (1) (2) (3) (4) (5)

3. (1) (2) 3 2 2

2 (3)

(4) 4.

(1) (2) CRP

(3) HLA-B51

- 165 -

1. 1 3

2.

3. ()

4. ()

5. 20 24-48

4 2 ()()

()

Tsank

()

HLA-B27 HLA-B51

() (Reiters syndrome)

- 166 -

;

() (sarcoidosis)

X

() (inflammatory bowel disease)

()

- 167 -

(80mg/)

() 3-4

()

1.

prednisolone 30-60mg 1 3 prednisolone 60-100mg/ 10-15mg/ Prednisolone 10-60mg/ Prednisolone

- 168 -

2.

3. (chlorambucil)

50-100mg/ 2-4mg/ 4

4. A (cyclosporine A):

(chlorambucil) A A 3-5mg/kg 2

5. (cyclophosphamide)

cyclophosphamide

A 1g/m2

6. FK506 A FK506

7. : levamisoleD-penicillamine

- 169 -

8. (colchicine)

0.5mg/ 2

9. (NSAID)

()



- 170 -

C

C~

C

61C

70%13131

(Cryoglobulin)

60 Wintrobe Bull

1947 Lemer Watoson

()

(Lympho-proliferative)

()

IgG IgM

()

IgG IgM

- 171 -

(essential mixed cryoglobulinemia)

C 36%

127 C 54%17%

34%

C 14.2

7.6

C

()(

)()()(

)

IgM IgM

C

C RNA

- 172 -

(1)

(2)

(3) C C

81

C

- Ribavirin

-

C

48

24 RNA

C

70-75 C RNA

-

C

C

C

C

- 173 -

1. Meltzer M, Franklin EC, Elias K, et al. cryoglobulinemiaclinical and laboratory studycryoglobulins with rheumatoid

factor activity. Am J med 1996;40837-42

2. Abel G, Zhang O-X, Angello V, Hepatitis C virus infection in type mixed cryoglobulinemia.Arthtitis Rheum 1993;361341-9.

3. Angello V. HypothesisThe etiology of mixed cryoglobulinemia associated with hepatitis C virus infection.Scand J Immunol

1995;42179-84.

4. Polzien F. SchottP. MihmS. Ramacoria. Hartmann H. Inteferon-treatment of hepatitis C virus-associated mixed

cryoglobulinemia. J Hepatol.1997;2763-71.

- 174 -

(Ankylosing Spondylitis)

(Ankylosing Spondylitis)

510.5 ~ 1%

()

()

()

()

- 175 -

CRPESR(A)B27(HLA-B27)

()

()

1984Dr. van der Linden (Modified New York criteria1984)

1. 3 2. 3. 4. 23 41~3

() 1.NSAIDs

- 176 -

, COX2 Meloxicam MobicRofecoxibVioxxCelecoxibCelebrex

2.

(Salfasalazine)MTX (Methotrexate116234 5 ~ 10

()

(1)

(2)

()

- 177 -

(1)

(2)

(3)

(4)

(5)

() (1)

(2)

(3)

(4)

- 178 -

(5)

(6)

(7)

(8)

(9)

()

(10)

(11)

(12)

- 179 -

(13)

(14)Sulfasalazine

NSAIDs

(15)

(16)



- 180 -

(Chlamydial Arthritis)

1. :

- 181 -

8-15

281

2. :

() 3. :

4. :

5. :

6. :

- 182 -

7. :

() :

() :

IgM IgA

(1):

(2): DNADNA PCR

(3)(HLA): HLA-B27HLA-B27

- 183 -

1.

2. Quinolone

3. 4.



- 184 -

(Gonococcal Arthritis)

15 30 5 9

- 185 -

1.:

2.: 60-90% 5

3.:

90%

4.:

1. 4-5

2. 3.

:

80-90%50-75% 25% :

- 186 -

: 1.:

3 20

2.:

3.PCR:

PCR DNA

:



- 187 -

(Myofascial Pain Syndrome)

(Trapezius) :

(Sternocleidomastoid muscle) :

- 188 -

(Levator scapulae muscle) : ? (Splenius capitis and splenius cervicis) : (Posterior cervical muscles : multifundi, semispinalis) :

(Suboccipital muscle) : (Temporalis muscle) : :

(Scalene Muscles):

(Supraspinatus Muscle):

(Infraspinatus Muscle):

(Teres Minor): (Latissmus Dorsi Muscle): (Teres Major):

(Rhomboideus Muscles): . (Deltoid Muscle): (Pectoris Major & Minor):

. (Sternalis):

(Serratus Anterior, Posterior):

- 189 -

(Biceps brachii):

(Hand extensors):

(Quadriceps femoris):

(Hamstring muscle):

(Gastrocnemius muscle): (Soleus muscle):

(Thoracolumbar Paraspinal Muscles): (Quadratus Lumborum): (Iliopsoas Muscle): (Gluteus Minimus): (Gluteus Medius): (Gluteus Maximus):,

(Pyriformis muscle)



- 190 -

Nonsteroid Anti-inflammatory Drugs; NSAIDS

()

Cox-1Cox-2Cox-1

Cox-2 Cox-2

Leukotriene

- 191 -

()

()

()

phenylbutazone Indomethacin

()

Indomethacin

- 192 -

IbuprofenTolmetin Sulindac

()

pyrazole

misprostol (Corticosteroids, )

-

- 193 -

1949 (Mayo Clinic) Hench 1950

Hench

( prednisolone 7.5mg)

()

prednisolone

1mg/kg 40mg prednisolone

()

20-40mg

prednisolone

prednisolone

()

2-20mg prednisolone

methotrexate

- 194 -

prednisolone 2 3

()

1.

2.

3.

4.

() :

1. H2 2.

3.

4. 5. 1()

6. 7. 8.

- 195 -

9.

DMARDs

(DMARDs, Disease Modifying Anti-rheumatic Drugs)DMARDs: 1. (Sulfasalazine) 2. (Hydroxychloroquine) 3. (Methotrexate, MTX) 4. (Arava, Leflunomide)

FDA

MTX MTX

5. (Enbrel) FDA

MTX

6. A (Ciclosporin A) A

T MTX DMARDs A

- 196 -

DMARDs

DMARD

DMARDs


2. Klippel, Dieppe, Rheumatology, 2nd Ed. Mosby 3. Daniel J. Wallace, Bevra Hannahs Hahn, Edmund L. Lupus


4. , ,

- 197 -

1976-1986 (therapeutic plasma exchange) 1983-1992

1992 86 86 prednisolone cyclophosphamide 40 DNA 20% 17%

T cyclophosphamide T 1980 (60ml/kg albumin) cyclophosphamide cyclophosphamide

- 198 -

14 8 56 14 4 1 17

(1). SLE (thrombotic thrombocytopenic prupura, TTP) (2). SLE (3). SLE (4). ()

(1). (2). 1 1.5 (plasma volume) (3). 10

(antiphospholipid antibody) (lupus anticoagulant) 34% 44% 65%

"" (catastrophic antiphospholipid antibody syndrome) 60%

(DIC)(heparin)

- 199 -

(1). 14 34(2). 24 29

(thrombotic microangiopathy)

(1)(2)

47cc/kg

- 200 -

1992

(ANCA)

NIH

Churg-Strauss

prednisolone cyclophosphamide prednisolone cyclophosphamide Wegeners

IgM

22.5

- 201 -

(1). (2). (3). 1

1. Lewis EJ, Hunsicker LG, Lan SP, Rohde RD, Lachin JM. Lupus Nephritis Collaborative Study Group. A controlled trial of plasmapheresis therapy in severe Lupus nephritis. N Engl J Med 1992;326:1373-1379.

2. Euler HH, Schroeder JO, Harten P, Zeuner RA, Gutschmidt HJ. Treatment-free remission in severe systemic lupus erythematosus following synchronization of plasmapheresis with subsequent pulse cyclophosphamide. Arthritis Rheum 1994;34:1784-1794.

3. Euler HH, Schwab UM, Schroeder JO, Hasford J. The Lupus Plasmapheresis Study Group: rationale and updated interim report. Artif Organs 1996;20:356-359.

4. Fulcher D, Stewart G, Exner T, Trudinger B, Jeremy R. Plasmaexchange and the anticardiolipin syndrome in pregnancy. Lancet 1989;2:171.

5. Neuwelt CM, Daikh DI, Linfoot JA, Pfister DA, Young RG, Webb RL, London SS, Asherson RA. Catastrophic antiphospholipid syndrome: response to repeated plasmapheresis over three years. Arthritis Rheum 1997;40:1534-1539.

6. Dwosh IL, Giles AR, Ford PM, Pater JL, Anastassiades TP and the

- 202 -

Queens University Plasmapheresis Study Group. Plasmapheresis therapy in rheumatoid arthritis: a controlled, double blind, crossover trial. N Engl J Med 1983;308:1124-1129.

7. Brubaker DB, Winkelstein A. Plasma exchange in rheumatoid vasculitis. Vox Sang 1981;41:295-301.

8. Madore F, Lazarus JM, Brady HR. Therapeutic plasma exchange in renal disease. J Am Soc Nephrol 1996;7:367-386.

9. Miller FW, Leitman SF, Cronin ME, Hicks JE, Leff RL, Wesley R, Fraser DD, Dalakas M, Plotz PH. Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis. N Engl J Med 1992;326:1380-1384.

10. McCune M, Winkelmann RK, Osmundson PJ, Pineda AA. Plasma exchange: a controlled study of the effect in patients with Raynauds phenomenon and scleroderma. J Clin Apheresis 1983;1:206-214.

11. Endo H, Hosono T, Kondo H. Antineutrophil cytoplasmic autoantibodies in 6 patients with renal failure and systemic sclerosis. J Rheumatol 1994;21:864-870.

12. Guillevin L, Lhote F, Cohen P, Jarrousse B, Lortholary O, Genereau T, Leon A, Bussel A. Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus corticosteroids plus pulse cyclophosphamide alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome patients with factors predicting poor prognosis. A prospective, randomized trial in sixty-two patients. Arthritis Rheum 1995;38:1638-1645.

13. OReilly MJG, Talpos G, Roberts VC. Controlled trial of plasma exchange in treatment of Raynauds syndrome. Br Med J 1979; 1:1113-1115.

14. Frankel AH, Singer DRJ, Winearls CG, Evans DJ, Rees AJ, Pusey CD. Type II essential mixed cryoglobulinemia: presentation, treatment outcome in 13 patient. Q J Med 1992; 82: 101-124

- 203 -

1. methotrexate, ketoconazole

2.

3.

4.

- 204 -

5.

6.

7. 8. 9. spirolactone 10.

1.

2. 3. 4. 5. 6. 7.

8.

1. , ,

- 205 -

()(Alfalfa)

(L-canavanine)

- 206 -

T (suppressor-inducer T cell)

()(Echinaccea)

- 207 -

1. , ,

- 208 -

1. Monoarticular or pauciarticular symptoms 2. Polyarticular joint symptoms 3. Diagnostic algorithm for polyarticular disorders 4. Inflammatory or non-inflammatory arthritis 5. Approach to management in difficult fibromyalgia syndrome 6. Spondyloarthropathy 7. Diagnostic algorithm for spinal disorders 8. Cryoglobulinemia

- 209 -

Arthralgia limited to oneOr several joints

Complete History & Physical Examination

Significant Trauma or Focal Bone Pain

Effusion or Signs of Inflammation?

X-ray

FRACTURE, YUMOR or METABOLIC BONE

Joint Aspiratio

Probable Inflammatory

Process

Re-evaluate Bloody Bone Marrow

Elements Present >2000 WBCs? >75% PMNs?

COAGULOPATHY, PSEUDOGOUT,

TUMOR, TRAUMA, or CHARCOT JOINT

Check: PT/PTT Platelet Count Bleeding Time

INTRA- ARTICULAR FRACTURE

Cystals Identified

Positive Culture*

Sterile Inflammatory

Joint Fluid

MONOSODIUN URATE (gout)

CALCIUM PYTOPHOSPHATE

DIHYDRATE (pserfogout)

INFECTIOUS ARTHRITIS

*Synovial Fluid Culture as well as

cervical, urethal,pharyngeal, and/or

rectal evalusttions for Gonococcus and

Chlamydia when suspected

*

Check: CBC, ESR, RF Consider: LFTs, LHA-B27, ANA, Lyme serologies, and Pelvis Radiographs

Point Tenderness or Trigger Points

BURSITIS, TENDINITIS, or FIBROMYALG1

OSTEOARTHRITIS, INTERNAL

DERANGEMENT, SOFT TISSUE

INJURY, or VIRAL (+)

(-)

(-)

(+)

(+)

(-) (+)

adnormal

normal

successful

unsuccessful

Supspect: RA, JRA, VIRAL, SLE, LYME, SARCOIDOSIS, OR SPONDYLOART

- 210 -

?

?

Check: Blood Count Liver Function Tests Consider: Hepatitis B and C Serology Parvovirus Serology

Check: CBC, ESR, RF, and/or

ANA; Creatinine, Urinalysis, Joint Aspiration (if effusion present; see previous figure for analysis

Consider: Liver Function Tests Hepatitis B and C Serology Radiographs Thyroid Stimulation Hormone Calcium Albumin Alkaline Phosphatase

(-) (+)

(-)

(+) (-)

(+)

- 211 -

- 212 -

- 215 -

- 216 -

- 217 -

Clinical Pathway

( outcomes management )

- 218 -

( cost-effective )

( length of hospital stay )

4 4 103

12 12

- 219 -

_____________________________________

____/____/_____ ____/____/____ ___

1 2 3 4-5 B/R,U/R,S/R

BUN,Creat,GOT,GPT ESR,CRP Rheumatoid factor HLA-B27R X-ray : C-spine

T-spine L-spine SI joint KUB Hip ______________

bone scan CT scan of spine

unstable spinal fracture fusion stable spinal fracture bracing

NSAIDs Sulfasalazine MTX Plaquenil

spinal fracture, active discitis start methylprednisolone pulse therapy

MTP pulse therapy

Vital signs,, Hot compression On TENS, 15,min,

qid

On bracing

30

,

,

MTP pulse

- 220 -

85

""

1. 2. () 3. ()

- 221 -

1. 1 2 3

2. 1

3. 2

4. 3

1. ()()()

2. ()()()()

3. ()()

- 222 -

()

4.

223

Systemic lupus erythematosus Rheumatoid arthritis Sjgrens syndrome Systemic sclerosis Behets disease Ankylosing spondylitis Reiters syndfome Relapsing Polychondritis

224

The 1982 revised criteria for classification of systemic lupus erythematosus

Criterion Definition 1. Malar rash Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds

2. Discoid rash Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions

3. Photosensitivity Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation

4. Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed by physician

5. Arthritis Nonerosive arthritis involving 2 or more peripheral joints, characterized by tenderness, swelling, or effusion

6. Serositis

a) Pleuritis--convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion

OR b) Pericarditis--documented by ECG or rub or evidence of pericardial effusion

7. Renal disorder

a) Persistent proteinuria greater than 0.5 grams per day or grater than 3+ if quantitation not performed

OR b) Cellular casts--may be red cell, hemoglobin, granular, tubular, or mixed

8. Neurologic disorder

a) Seizures--in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance

OR b) Psychosis--in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte imbalance

9. Hematologic disorder

a) Hemolytic anemia--with reticulocytosis OR

b) Leukopenia--less than 4,000/mm3 total on 2 or more occasions OR

c) Lyphopenia--less than 1,500/mm3 on 2 or more occasions OR

d) Thrombocytopenia--less than 100,000/mm3 in the absence of offending drugs

10. Immunologic disorder

a) Positive LE cell preparation OR

b) Anti-DNA: antibody to native DNA in abnormal titer OR

c) Anti-Sm: presence of antibody to Sm nuclear antigen OR

d) False positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test

11. Antinuclear antibody An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with "drug-induced lupus" syndrome

Updating the American College of Rheumatology Revised Criteria for the Classification of Systemic

Lupus Erythematosus 1. Delete item 10(a) ("Positive LE cell preparation"), and 2.Change item lO(d) to "Positive finding of antiphospholipid antibodies based on

1) an abnormal serum level of IgG or IgM anticardiolipin antibodies, 2) a positive test result for lupus anticoagulant using a standard method, or 3) A false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by

Treponema pallidum immobilization or fluorescent treponemal antibody absorption test.

225

1987 Criteria for the Classification of Acute Arthritis of Rheumatoid Arthritis

Criterion Definition 1. Morning stiffness Morning stiffness in and around the joints, lasting at least 1 hour before maximal improvement

2. Arthritis of 3 or more joint areas

At least 3 joint areas simultaneously have had soft tissue swelling or fluid (not bony overgrowth alone) observed by a physician. The 14 possible areas are right or left PIP, MCP, wrist, elbow, knee, ankle, and MTP joints

3. Arthritis of hand joints At least 1 area swollen (as defined above) in a wrist, MCP, or PIP joint

4. Symmetric arthritis

Simultaneous involvement of the same joint areas (as defined in 2) on both sides fo the body (bilateral involvement of PIPs, MCPs, or MTPs is acceptable without absolute symmetry)

5. Rheumatoid nodules Subcutaneous nodules, over bony prominences, or extensor surfaces, or in juxtaarticular regions, observed by a physician

6. Serum rheumatoid factor Demonstration of abnormal amounts of serum rheumatoid factor by any method for which the result has been positive in

226

Criteria for the Diagnosis of Sjogren's Syndrome Primary Sjogren's Syndrome: all of the following

(1) keratoconjunctivitis sicca (see chapter on ophthalmology) (2) xerostomia (see chapter on dentistry and oral medicine) (3) no concurrent connective tissue disease

Secondary Sjogren's Syndrome (1) keratoconjunctivitis sicca and/or xerostomia (2) connective tissue disorder

Rheumatoid arthritis Systemic Lupus Erythematosus Progressive Systemic Sclerosis, Scleroderma Dermatomyositis, Polymyositis Primary Biliary Cirrhosis Wegener's Granulomatosis Polyarteritis Nodosa

Possible or Incipient Sjogren's Syndrome (1) keratoconjunctivitis sicca or xerostomia (2) one of the following conditions associated with an immune

dysfunction in the absence of a diagnosed connective tissue disorder: pulmonary lymphocytic interstitial infiltrates interstitial nephritis and/or renal tubular acidosis hypergammaglobulinemia vasculitis with purpura polymyopathy chronic noncirrhotic liver disease neuropathy

References: 1. Daniels TE. Chapter 6: Benign lymphoepithelial lesion and Sjogren's syndrome. pages 83-106. IN:

Ellis GL, Auclair PL, Gnepp DR. Surgical Pathology of the Salivary Glands.Volume 25 in Major Problems in Pathology. WB Saunders Company. 1991. Table 6-1 page 97.

2. Manthorpe R, Oxholm P, et al. The Copenhagen criteria for Sjogren's syndrome. Scand J Rheumatology. 1986; Supplement 61: 19-21.

3. Prause JU. Manthorpe R, et al. Definition and criteria for Sjogren's syndrome used by the contributors to the First International Seminar on Sjogren's Syndrome - 1986. Scand J Rheumatology. 1986; Supplement 61: 17-18.

227

1980 Criteria for the Classification of Systemic Sclerosis

Glossary of clinical terms used in description or classification of systemic sclerosis

1. Typical sclerodermatous skin changes: tightness, thickening, and non-pitting induration, excluding the localized forms of scleroderma (morphea or linear scleroderma)

a) Sclerodactyly: above-indicated changes limited to (fingers and toes).

b) Proximal scleroderma: above-indicated changes proximal to the metacarpophalangeal or metatarsophalangeal joints, affecting other parts of the extremities, face, neck, or trunk (thorax or abdomen); usually bilateral, symmetrical and almost always including sclerodactyly

2. Other skin manifestations attributable to systemic sclerosis or comparison disorders

a) Digital pitting scars or loss of substance from the finger pad: depressed areas at tips of digits or loss of digital pad tissue as a result of digital ischemia rather than trauma or exogenous causes

b) Bilateral finger or hand edema: firm but pitting edema, especially involving fingers (includes puffy sausage-like swelling of fingers) or the dorsal aspect of the hands

c) Abnormal skin pigmentation: hyperpigmentation often containing areas of punctate or patchy hypopigmentation or depigmentation ("pepper and salt")

d) Raynaud's phenomenon: at least two-phase color change in fingers and often toes consisting of pallor, cyanosis, and/or reactive hyperemia in response to cold exposure or emotion, as determined by patient's history or physician's observation

3. Visceral manifestations

a) Bibasilar pulmonary fibrosis: bilateral reticular pattern of linear or lineonodular densities which are most pronounced in basilar portions of the lungs on standard chest roentgenogram; may assume appearance of diffuse mottling or "honeycomb lung," and should not be attributable to primary lung disease

b) Lower (distal) esophageal dysphagia: substernal discomfort on swallowing or sensation of food holdup in the retrosternal location

c) Lower (distal) esophageal dysmotility: hypoperistalsis or aperistalsis, as demonstrated by either cine esophagram or fluoroscopy or by manometric study, often accompanied by evidence of decrease in lower esophageal sphincter tone with reflux of gastric contents into the esophagus

d) Colonic sacculations: wide-mouthed diverticula of colon located along the antimesenteric border; found on barium enema examination; these sacculations may also occur in ileum and jejunum

229

New York Criteria for Ankylosing Spondylitis Overview: The New York criteria for ankylosing spondylitis is a modification of the

Rome criteria as proposed at the CIOMS Symposium in New York during 1966. It combines both clinical criteria and radiographic findings of the sacroiliac joint.

Clinical criteria (1) Limitation of motion of the lumbar spine in all 3 planes (anterior flexion, lateral

flexion, extension). Skin markings to aid in the examination are shown in the Figure on page 356 of Moll (1973).

(2) A history of pain or the presence of pain at the dorsolumbar junction or in the lumbar spine.

(3) Limitation of chest expansion to 1 inch (2.5 cm) or less, measured at the level of the fourth intercostal space.

Radiographic Changes in the Sacroiliac Joint(s) Grade normal 0 suspicious 1 1 minimal sacroiliitis 2 2 moderate sacroiliitis 3 3 ankylosis 4 4 Radiographic Change Clinical Criteria Present Interpretation bilateral grade 3-4 sacroiliitis 1 or more definite unilateral grade 3-4 sacroiliits criterion #1 definite bilateral grade 2 sacroiliitis criterion #1 definite unilateral grade 3-4 sacroiliits criterion #2 or #3 definite bilateral grade 2 sacroiliitis criterion #2 or #3 definite bilateral grade 3-4 sacroiliitis 0 probable References:

1. Bennet PH, Wood PHN. Population studies of the rheumatic diseases. Amsterdam, Excerpta Medica Foundation. 1968; 456-457. Eck JC, Humphreys C. Diagnosis and treatment of common metabolic spinal disorders in the geriatric population. Southern Medical Journal. 1998; 91: 1090-1097.

2. Goei The HS, Steven MM, et al. Evaluating of diagnostic criteria for ankylosing spondylitis: A comparison of the Rome, New York and modified New York criteria in patients with a positive clinical history screening test for ankylosing spondylitis. Br J Rheumatol. 1985; 24: 242-249. Moll JMH, Wright V. New York clinical criteria for ankylosing spondylitis. Ann Rheum Dis. 1973; 32: 354-363.

3. Moll JMH. New criteria for the diagnosis of ankylosing spondylitis. Scand J Rheumatology. 1987; Supplement 65: 12-24. van der Linden S, Valkenburg HA, Cats A. Evaluation of diagnostic criteria for ankylosing spondylitis. Arthritis Rheumatism. 1984; 27: 361-368.

230

Modified New York Criteria for Ankylosing Spondylitis Overview: van der Linden et al proposed a modification to the original New York criteria

for ankylosing spondylitis. This replaces the original New York pain criterion with the pain criterion from the Rome criteria. The authors are from the Erasmus University in Rotterdam.

Clinical criteria (1) low back pain and stiffness for more than 3 months which improves with exercise

but which is not relieved by rest. (2) Limitation of motion of the lumbar spine in both the sagittal and frontal planes. (3) Limitations of chest expansion relative to normal values corrected for age and sex. Measurement of chest expansion needs to be standardized to minimize variability.

measured in the 4th intercostal space van der Linden measured the expansion twice, taking the larger of the 2. The result is compared with tables broken down by age and sex (Moll and

Wright, 1972; Moll and Wright, 1973). Using 1 inch (2.5 cm) as the measure was specific but insensitive.

Radiographic criteria:

(1) bilateral sacroiliitis >= 2 (2) unilateral sacroiliitis >= 3

Definite anklyosing spondylitis (1) >= 1 clinical criterion AND 1 radiologic criteria

Probable ankylosing spondylitis

(1) 3 clinical criteria AND no radiologic criteria (2) 1 radiologic criterion is present AND no clinical criteria

References: 1. Dougados M. Editorial: Diagnostic features of ankylosing spondylitis. Br J Rheumatol. 1995; 34:

301-305. 2. Goei The HS, Steven MM, et al. Evaluating of diagnostic criteria for ankylosing spondylitis: A

comparison of the Rome, New York and modified New York criteria in patients with a positive clinical history screening test for ankylosing spondylitis. Br J Rheumatol. 1985; 24: 242-249.

3. Moll JMH, Wright V. An objective clinical study of chest expansion. Ann Rheum Dis. 1972; 31: 1-8. 4. Moll JMH, Wright V. The pattern of chest and spinal mobility in ankylosing spondylitis: an objective

clinical study of 106 patients. Rheumatol Rehab. 1973; 12: 115-134. 5. Moll JMH. New criteria for the diagnosis of ankylosing spondylitis. Scand J Rheumatology. 1987;

Supplement 65: 12-24. 6. van der Linden S, Valkenburg HA, Cats A. Evaluation of diagnostic criteria for ankylosing spondylitis.

Arthritis Rheumatism. 1984; 27: 361-368.

231

American College of Rheumatology

1977 Criteria for the Classification of Acute Arthritis of Primary Gout

1. More than one attack of acute arthritis

2. Maximum inflammation developed within 1 day

3. Monoarthritis attack

4. Redness observed over joints

5. First metatarsophalangeal joint painful or swollen

6. Unilateral first metatarsophalangeal joint attack

7. Unilateral tarsal joint attack

8. Tophus (proven or suspected)

9. Hyperuricemia

10. Asymmetric swelling within a joint on x ray*

11. Subcortical cysts without erosions on x ray

12. Monosodium urate monohydrate microcrystals in joint fluid during attack

13. Joint fluid culture negative for organisms during attack

232

Criteria for the Diagnosis of Behcets Disease from the International Study Group for Behcets Disease

Recurrent oral ulcerations, having all of the following features:

minor aphthous ulcers, major aphthous ulcers, or herpetiform lesions observed by physician or patient recur at least 3 times in one 12 month period

AND Two or more of the following

recurrent genital ulcerations: aphthous ulceration or scarring observed by physician or patient

eye lesions anterior uveitis posterior uveitis cells in vitreous humor on slit lamp examination retinal vasculitis observed by ophthalmologist

skin lesions: erythema nodosum observed by physician or patient pseudofolliculitis papulopustular lesions acneiform nodules observed by physician in postadolescent patients not on

corticosteroid treatment positive pathergy test

Pathergy (Behcetine) test: skin needle pricks or intradermal injection of saline read by physician at 24-48 hours positive reaction: indurated erythema or pustular formation Performance of criteria sensitivity 91-95% specificity 96-98% References: International Study Group for Behcets Disease. Criteria for diagnosis of Behcets disease. Lancet. 1990; 335: 1078-1080.

233

ACR Clinical Classification Criteria for Osteoarthritis of the Hand Overview:

The American College of Rheumatology (ACR) published criteria for the diagnosis of osteoarthritis involving the hand based on clinical findings.

10 selected joints evaluated for hard tissue enlargement and deformity:

(1) second distal interphalangeal (DIP) joint, left hand (2) third distal interphalangeal (DIP) joint, left hand (3) second distal interphalangeal (DIP) joint, right hand (4) third distal interphalangeal (DIP) joint, right hand (5) second proximal interphalangeal (PIP) joint, left hand (6) third proximal interphalangeal (PIP) joint, left hand (7) second proximal interphalangeal (PIP) joint, right hand (8) third proximal interphalangeal (PIP) joint, right hand (9) first carpometacarpal (CMC) joint, left hand (10) first carpometacarpal (CMC) joint, right hand

Traditional format: (1) hand pain, aching or stiffness, AND (2) 3 or 4 of the following:

(a) hard tissue enlargement in >= 2 of the 10 selected joints (b) deformity of >= 1 of the 10 selected joints (c) hard tissue enlargement of >= 2 DIP joints (d) swelling of 0 2 (< 3) metacarpophalangeal (MCP) joints

Classification tree format: all of the following (1) hand pain, aching or stiffness (2) hard tissue enlargement in >= 2 of the 10 selected joints (3) swelling of 0 2 (< 3) MCP joints (4) one or both of the following

(a) hard tissue enlargement of >= 2 DIP joints (b) deformity in >= 1 of the 10 selected joints

NOTE: In Table 6 (page 1607), the last item is "deformity of 2 or more of the 10 selected joints." However, in Figure 1 page 1606, which is graphical representation of the table, >= 1 is used. This matches the traditional format.

Performance characteristics:

traditional format has a sensitivity of 94% and specificity of 87% classification tree format has a sensitivity of 92% and specificity of 98%

References: Altman R, Alarcon G, et al. The American College of Rheumatology criteria for the classification and reporting of osteoarthritis of the hand. Arthritis Rheumatism. 1990; 33: 1601-1610.

234

Criteria for Reiter's Syndrome Criteria of Calin et al for Reiter's Syndrome

(1) seronegative (rheumatoid factor negative) asymmetric arthropathy predominantlyaffecting the lower extremity. Usually this is a polyarthropathy but may be a monoarthropathy.

(2) one or more of the following: urethritis, or cervicitis in women dysentery inflammatory eye disease (conjunctivitis, iridocyclitis, uveitis) mucocutaneous disease (balanitis in the male, oral ulcerations, and/or keratodermia)

(3) none of the following primary ankylosing spondylitis psoriatic arthropathy other rheumatic diseases

Other findings: (1) nail lesions (2) aortitis (3) tendonitis

Associations (1) Infection with Chlamydia trachomatis can be found in over 70% of men with

untreated nondiarrheal Reiter's syndrome. (2) There is evidence of exaggerated cell-mediated and humoral responses to

chlamydial antigens. (3) A similar syndrome may occur after enteric infection with Salmonella, Shigella or

Campylobacter. (4) HLA-B27 is present in over 80% of affected patients.

References: 1. Calin A, Fox R, et al. Prognosis and natural history of Reiter's syndrome. Ann Rheumatic Dis. 1979;

38 (supplement): 29-31. 2. Stamm WE, Holmes KK. Chapter 140: Chlamydial infections. pages 759-768 (page761 and 764).

IN: Isselbacher KJ, Braunwald E, et al. Harrison's Principles of Internal Medicine, Thirteenth Edition. McGraw-Hill. 1994.

235

Criteria for the Diagnosis of Relapsing Polychondritis Initial criteria: 3 or more of the following

(1) recurrent chondritis of both auricles (swelling, pain and erythrema of the helix, antihelix, tragus and/or external auditory canal, with sparing of the inferior lobule)

(2) nonerosive inflammatory arthritis (3) chondritis of the nasal cartilage (4) ocular inflammation (5) chondritis of the larynx, trachea, or both (6) damage to the cochlea, vestibule or both, with hearing loss and tinnitus, or vertigo,

or both

Modified criteria (1) 1 or more of the above criteria, with histologic confirmation (2) chondritis in 2 or more anatomic locations, with a response to corticosteroids,

dapsone or both where: In the implementation, I counted the following as separate anatomic locations for

the second modified criteria: one or both ears, nasal cartilage, larynx and/or trachea, inner ear. Joint and eye involvement were not included as written.

References: 1. Damiani JM, Levine HL. Relapsing polychrondritis Rport of ten cases. Laryngoscope. 1979; 89:

929-946 (Table I, page 930). 2. Maddison PJ. Chapter 5.17.3: Diseases of bone, cartilage, and synovium. pages 1617-1629 (pages

1626-1627). IN: Maddison PJ, Isenberg DA, et al (editors). Oxford Textbook of Rheumatology, Second Edition. Oxford Medical Publications. 1998.

3. McAdam LP, O'Hanlan MA, et al. Relapsing polychrondritis. Prospective study of 23 patients and a review of the literature. Medicine. 1976; 55: 193-215. Scully RE, Mark EJ, et al (editors). Weekly clinicopathological exercises: Case 32-1999. N Engl J Med. 1999; 341: 1292-1299. (Table 2, page 1295)

236

Classification Tree for the Diagnosis of Takayasu Arteritis Criteria Comment Age of disease onset 10mmhg

Difference of > 10 mm Hg in systolic blood pressure between arms

Bruit over subclavian artery or aorta Bruit audible on auscultation over one or Both subclavian arteries or abdominal aorta

Arteriographic abnormalities

Narrowing, aneurysms or occlusion of the Entire aorta, its primary branches, or large Arteries in the proximal upper or lower Extremities, not due to arteriosclerosis, Fibromuscular dysplasia or similar causes; Changes usually focal or segmental

237

Cirteria for Polymyalgia Rheumatica, 1982

Criteria for diagnosis all of the following: (1) Age of the patient >= 50 years (2) Erythrocyte sedimentation rate > 40 mm/h (3) Bilateral aching and stiffness for >= 1 month involving >= 2 of the 3

target body regions (4) Exclusion of all other diagnosis except giant cell arteritis

Body regions: (1) Neck or torso (2) Shoulder or proximal regions of the arms (3) Hips or proximal aspects of the thighs

239

A.

B.

C.

D.

(1)

(2)

(3)

E.

(1)

(2) ()

(3)

(4)

(5)

F.

COX-2

(60%)

240

(tenosynovitis)

(ceftriaxone ceftizoxime

) 24 36

ciprofloxacin500

amoxicillin/clavulanate500-850

(chlamydia)

B

parvo virus

()

(HIV)

(septic bursitis) olecranon prepatella

(housemaids kneewriters elbow)

( knee pads)

Borrelia burgdorferi

(erythema migrans)

241

(Gout)

1% 7 9 1;

3.3%

50~60 30~40 2.7

(Hyperuricemia)

(purine)

(uricase)

7mg/dl 6mg/dl 37

7mg/dl 6mg/dl

2-18% 80%

( 10-20%)(

80-90%)(

)(

)

()

9mg/dl

4.9%; 7 8.9 0.5%;

7mg/dl 0.1%

242

(cytokine)(Interleukin)-1-6-8

(Tumor necrosis factor, TNF)

;

2 (cycloxygenase-2)

()

():(1)

;(2) TNF-

;(3)

transforming growth factor (TGF)-;(4)

Peroxisome proliferators-activated reaptor (PPARr)

:

(1)

(2)

()

(3)

(4)

10

(strongly negatively birefringence)

X-

1977():

:: 12

X :1.

2.3.4.5.

243

6.7.8.9.10.

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;

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Weir (2003)( B)

10mg/dl

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NSAIDs ()

NSAIDs( 2 -Cox-2 inhibitors)

NSAIDs( Indomethacin)( B)

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/

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244

()

( 15~30 mg3-7

) Daxamethasone(5 mg1-2 )

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()

:

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2.(3 /) 3.()

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:

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hypoxanthine()

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400mgAllopurinol

Allopurinol

Steven-Johnsons Allopurinol Steven-Johnsons

2. (Uricase):Rasburicase (Urate oxidase)

Allantoin ( 10~100 )

245

( B)

3. (Uricosuric drugs) probenecid(Benemid)Sulfinpyrazone

Benzbromarone(Nacaricin)(fenofibrate)

(Losartan) Allopurinol

probenecidSulfanpyrazone(50

/)(

)Benzbromarone

( C)

Allopurinol

Fenofibrate(Lipanthyl)

( C)

Losartan (Cozaar)

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5~10%

10~20%

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246

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249

1. Terkeltaub RA. Clinical practice: gout. NEJM 2003;349:1647-55. 2. Rott KT, Agudelo CA. Contempo updates: gout. JAMA 2003;289:2857-60. 3. Wortmann RL. Gout and hyperuricemia. Curr Opin Rheumatol 2002;14:281-6. 4. Matsukawa A, Yoshimura T, Maeda T, Takahashi T, Ohkawara S, Yoshinaga M.

Analysis of the cytokine network among tumor necrosis factor , interleukin-1, interleukin-8, and interleukin-1 receptor antagonist in monosodium urate crystal-induced rabbit arthritis. Lab Invest 1998;78:559-69.

5. Schweyer S, Hemmerlein B, Radzun HJ, Fayyazi A. Continous recruitment, co-expression of tumor necrosis factor and matrix metalloproteinases, and apoptosis of macrophages in gouty tophi. Virchows Arch 2000;437:534-9.

6. Yamamoto T, Moriwaki Y, Takahashi S, Tsutsumi Z, Ka T, Fukuchi M, et al. A simple method of selecting gout patients for treatment with uricosuric agents, using spot urine and blood samples. J Rheumatol 2002;29:1937-41.

7. Segal JB, Albert D. Diagnosis of crystal-induced arthritis by synovial fluid examination for crystals:lessons from an imperfect test. Arthritis Care Res 1999;12:376-80.

8. Shekarriz B, Stoller ML. Uric acid nephrolisthiasis: current consepts and controversies. J Urology 2002;168:1307-14.

9. Park YB, Park YS, Lee SC, Yoon SJ, Lee SK. Clinical analysis of gouty patients with normouricemia at diagnosis. Ann Rheum Dis 2003;62:90-92.

10. Emmerson BT. Drug therapy: the management of gout. NEJM 1996;334:445-51. 11. Umpierrez A, Cuesta-Herranz J, De Las Heras M, Liuch-Bernal M, Figueredo E,

Sastre J. Successful desensitization of a fixed drug eruption caused by allopurinol. J Allergy Clin Immunol 1998;101:286-7.

12. Takahashi S, Moriwaki Y, Yamamoto T, Tsutsumi Z, Ka T, Fukuchi M. Effects of combination treatment using antu-hyperuricemic agents with fenofibrate and/or losartan on uric acid metabolism. Ann Rheum Dis 2003;62:572-5.

13. Hepbum AL, Kaye SA, Feher MD. Long-term remission from gout associated with fenofibrate therapy. Clin Rheumatol 2003;22:73-6.

14. Perez-Ruiz F, Alonso-Ruiz A, Calabozo M, Herrero-Beites A, Garcia-Erauskin G, Ruiz-Lucea E. Efficacy of allopurinol and benzbromarone for the control of hyperuricemia. A pathogenic approach to the treatment of primary chronic gout. Ann Rheum Dis 1998;57:545-9.

15. Scott JT. Comparison of allopurinol/probenecid. Ann Rheum Dis 1996;25:623-6. 16. Fam AG. Treating acute gouty arthritis with selective COX-2 inhibitors. BMJ

2002;325:980-1.

250

(Vasculitis)

()

(1)-

(giant cell arthritis)(Takayasus disease); (2)

-(polyarteritis nodosa)

(Kawasakis disease); (3)-

(Wegeners granulomatosis)Churg-Strauss

(microscopic polyangiitis) (4)

-Henoch-Schonlein

(cryoglobulinemia)

(ethanol)

C-ANCA

P-ANCAC-ANCA Cytoplasmic ANCA

azurophilic granule proteinase 3(PR3) P-ANCA Perinuclear

ANCA

Myeloperoxidase(MPO) elastase, lactoferin,cathepsin G

P-ANCAANA

251

formalin

P-ANCA

C-ANCA ANCA

Churg-Strauss

Microscopic polyangiitis

Primary pauci-immune necrotizing and crescentic

glomerulonephritisRapidly

progressive glomerulonephritisRPGN C-ANCA

13652

C-ANCA 66

98 P-ANCA

Churg-Strauss

C

ELISAs

C-ANCAproteinase 3PR3p-ANCA

MyeloperoxidaseMPO

,

C-ANCAPR3

Sensitivity73Specificity

99ANCA

ANCA

(1)Induction therapyNIH

252

Cyclophosphamide 2mg/kg/day

Cyclophosphamide

Cyclophosphamide

(plasmapheresis)

751

IVIG0.4gm/kg/day

Cyclophosphamide

2Anti-thymocyteglobulin

T3anti-CD4anti-CDw52

4

(2)Cyclosphosphamide

Cyclo

Documents

內科部 過敏免疫風濕科工作手冊intm.vghtc.gov.tw/imd/ch/過敏免疫風濕科工作手冊.pdf · 目 錄 第一章 簡介 1-3 第二章 教學訓練計畫 4-10 第三章

內科部過敏免疫風濕科工作手冊intm.vghtc.gov.tw/imd/ch/過敏免疫風濕科工作手冊.pdf · 目錄第一章簡介 1-3 第二章教學訓練計畫 4-10 第三章