Crescentric GlomerulonephritisDr. A.U. Ali Akram

RegistrarWard 49/47B

NHSL

Case• 60 year old mother of 4 children from Marawila,

presented with bilateral purulent ear discharge with reduced hearing and generalized body swelling for one week duration.

• The body swelling was noted on the face, around the eyes as well as bilateral lower limbs.

• Swelling was high in the morning. • There was a trivial feverishness and sorethroat on

first two days of the illness.

• There was associated reduced urine output with frothy urine, but, no change in the colour of urine was noted.

• She didn't have headache or chest symptoms like cough.

• There was no abdominal pain or distension. But she had mild loss of appetite.

• Her bowel habits were normal.• She didn't notice any skin rashes and there were

no joint pain or muscular pain felt.

• There was no history of hepatitis, TB, or other chronic illnesses.

• Her surgical history was unremarkable.

• She was not on any medications including over the counter drugs as well as Ayurvedic medications.

• She is not allergic to any foods or medicines.

• There was no any history of blood transfusions, travel history and she doesn't keep pets.

On examination• Averagely built• Not pale• Not icteric• Not dyspneic• Afebrile• No skin rashes

• Bilateral periorbital oedema was noted

• Bilateral ears reveal inflamed tympanic membranes with exudates

• Oral hygeine was good with normal throat.• There is no finger clubbing, leukonychia or other

peripheral stigmata of chronic liver cell disease.• Bilateral pitting ankle edema upto mid-calf was noted. But

no clinical features of deep vein thrombosis found.

CVS -• PR - 84bpm (regular and good volume)• BP - 150/90mmHg

• Precordial examination was normal.• Auscultation revealed normal S1 and S2 with no murmurs.

RS -• RR - 16cpm• Chest examination revealed no features of pleural effusion

Abdomen -• Not distended• No ascites on clinical examination• There is no hepatosplenomegaly or ballotable masses

Neurology -• Normal

Problems -• Acute - 1. Bilateral suppurative otitis media2. Generalised body swelling with reduced urine

output and frothy urine

Differential Diagnoses -Acute kidney injury due to -1. A glomerular disease2. Otitis media leading to sepsis - unlikely3. Pre-renal renal failure - unlikely

Investigations -1. Full Blood Count -WBC - 9.87 x 103/ulHb - 10.1 g/dl

MCV - 94 flPlt - 158 x 103/ul

2. Trend of Serum Creatinine -

15.09 16.09 17.09 18.09 19.09 20.09 21.09 22.09 23.09 24.09 25.09 26.09 27.09 28.09 29.09 30.090

200

400

600

800

1000

1200

1400

Date

S.Cr

(um

ol/l)

Hemodialysis

Investigations -3. Serum Electrolytes -Na - 135mmol/lK - 3.7mmol/l

4. PT - 12.1s (normal)

5. APTT - 30.8s (normal)

Investigations -6. Urine Full Report -Colour - light yellowAppearance - CloudySpecific gravity - 1.025pH - 5.0Prtoein >300mg/dlSugar - nilKetone bodies - nilUrobillinogen - normalBile pigments - nilNitrite - nil

Centrifuged deposit

Pus cells - 4 - 6/hpfRed cells - >100/hpfEpithelial cells - +/hpfCasts - Granular casts +Crystals - nilOrganisms - ++Others - +

Investigations -7. Urine protein : creatinine ratio -Urine creatinine - 30mg/dlUrine protein - 309mg/dlUPCR - 10.32

>3.5 - Nephrotic range

8. ASO Titre - 200IU/ml

Ref range = <200IU/ml

Investigations -9. CRP - 17.5mg/l (15.09) --> 0.8mg/l (25.09)

10. ESR - 13mm/h

11. Liver enymes -AST - 26U/LALT 80U/LALP - 52U/LTotal Billirubin 3.9umol/l

Investigations -12. Serum Prtoeins -Total - 51g/lAlbumin - 22g/lGlobulin - 29g/l

13. Ear swab culture - no growth

14. Blood culture - no growth

15. Urine culture - no growth

Investigations -16. Phosphorus - 1.7mmol/l (0.8 - 1.5)

Magnesium - 0.75mmol/l (0.8 - 1.1)Total calcium - 1.8mmol/l

17. Ultrasound scan - Abdomen -Bilateral renal cortical echogenecity increased with ill defined cortical demarcation. No hydronephrosis.R/Kidney - 9.5cm; L/Kidney - 10.3cmFeatures of liver parenchymal changes.Mild ascites, no pleural effusion noted.

Investigations -18. Renal biopsy -Morphological features are consistant with "Cresentric Glomerulonephritis".Some features are suggestive of lupus nephritis.

19. ANA - Negative20. C3/C4 – Normal 21. Hepatitis screening - Negative22. Cryoglobulin levels - pending23. Retroviral studies - Negative

Cant afford –C-ANCA and P-ANCAAnti-GBM antibodies

Management at ward -1. IP/OP monitoring.

IP = Last day OP + 400ml

2. Antibiotics IV Ceftriaxone 1g bd for 14daysOral Flucloxacillin 500mg 6hourly for 14days

3. Oral Frusamide 40mg bdDiltiazem 30mg tds

Management at ward -4. ENT opinion on otitis media -

Continue the antibioticsAntibiotic ear drops added

5. Nephrology opinion on AKI (RPGN) -Urgent hemodialysis via neck line on first 2 daysIV Methyl Prednisolone pulse (500mg daily/3d)followed by oral Prednisolone 40mg dailyLeft renal biopsy done on 17.09.2015IV Cyclophosphomide 500mg one dose given.

Management at ward -6. Daily urine ward test for protein and dry weight

7. Daily CBS chart

8. Supportive therapy-Alendronate 75mg weeklyVitamin A&D one tab dailyCaCO3 1 tab daily

Glomerulopathy

Pure Nephrotic Syndrome

(Nephrotic syn-drome with blant

sediments)

Pure Nephritic Syn-drome

Mixed Nephrotic and Nephritic Syn-

drome(Nephrotic syn-

drome with active sediments)

Any type can present as RPGN

Nephrotic Syndrome with Blant Sedi-ments

Nephrotic Syndrome with Active Sed-iments

Nephritic Syndrome

Spectrum of Glomerulopathies

Rapidly Progressive Glomerulonephritis - RPGN

• It is a clinical syndrome, characterized by;1. Glomerular disease in the urine and2. Progressive loss of renal function over a short period

and3. Morphologically extensive crescent formation

• The commonest histological diagnosis in the el-derly who presents with acute nephritis.

Rapidly Progressive Glomerulonephritis - RPGN

• The prognosis depends on the:– Degree of crescent formation and– Circumferential involvement

• Crescent formation appears to represent a non-specific response to severe injury to the glomerular capillary wall.

Types of RPGN

1. Type 1 : Anti-GBM –– Idiopathic Anti-GBM or Goodpasture’s syndrome

2. Type 2 : Immune-complex –– IgA Nephropathy –– Post-infectious GN –– Lupus nephritis – – Mixed cryoglobulinemia -

Types of RPGN

3. Type 3 : Pauci-immune –– ANCA associated vasculitis

4. Double antibody-positive –– Type 1 and 3 positive

5. Idiopathic –– Immune-complex disease with no identifiable causes or– Pauci-immune disease with negative ANCA

Clinical Presentation

• Presenting complaints resemble severe post-strep-tococcal GN.

• More than 3 x normal Cr• Dysmorphic red cells• Red cell and other casts• Variable proteinuria (nephrotic range implies less severe renal

damage)

• Systemic features of particular disease, if any!

Evaluation

• ANA• Anti-GBM antibodies• ANCA• Complements• Cryoglobulins

• Renal biopsy

Empiric Treatment

1st • Pulse methylprednisolone

2nd• Oral prednisolone• Oral / IV Cyclophosphamide

3rd• Plasmapharesis, if patient has hemopty-

sis