RPGN

(loss of real futio over days to weeks, hypertension, edea )

SAQ/LQ. A 20-year-old female who had previously been well, presented with a one-weak history of painless macroscopic haematuria, reduced urine output and body swelling. She denied any history of skin rash, painful swollen joints or haemoptysis. There was no family history of renal disease. On physical examination she had anasarca and blood pressure (BP) of 150/100 mmHg. Bedside urine dipstick showed blood 3+ and protein 2+. The initial laboratory findings were as follows: serum creatinine 619 umol/L, urea 30 mmol/L, serum sodium 139 mmol, serum potassium 6.07 mmol/l, hemoglobin 9.6 g/dl, platelets 4,00,000/L. There were no blasts or schistocytes on the peripheral smear.

SAQ.LQ. A 30-year-old man presented with decreased volume of urine with both leg swelling for 10 days. On examination, his blood pressure was 160/100 mm Hg and bilateral pedal oedema was present. Urine RME showed +++ albumin, RBC 15-20/ HPF with RBC casts. His current S. creatinine level was 7 mg/dL which was 3 mg/dL 4 days ago.

1. What is your diagnosis?
2. Mention 5 differential diagnoses for this patient.
3. List 5 other important investigations to reach the etiological diagnosis.
4. What other history do you want to take from the patient?
5. How will you plan to treat the patient?

Diagnosis: Rapidly progressive glomerulonephritis (RPGN)

Etiological differential:

1. PIGN
2. Anti-GBM disease
3. Small-vessel vasculitides
4. Lupus nephritis 
5. IgA nephropathies

Investigations:

Management:

• Plasma exchange to remove circulating antibodies
• Steroids to suppress inflammation from antibody already deposited
• Cyclophosphamide to suppress further antibody synthesis

Rapidly Progressive Glomerulonephritis (RPGN)

Definition:

• RPGN is rapid loss of renal function over days to weeks, usually in association with 

• hypertension and oedema.
• non visible hematuria (glomerular haematuria →RBC cast or dysmorphic RBC), 
• variable proteinuria, 
• renal biopsy shows crescentic lesions 

Types of RPGN:

• Linear immunofluorescence pattern (anti GBM RPGN)

• Idiopathic anti-GBM antibody mediated RPGN
• Good pasture’s syndrome

• Granular immunofluorescence pattern (Immune complex mediated RPGN)

• Idiopathic immune complex mediated RPGN
• Associated with other primary GN

                            Mesangiocapillary GN

                            Ig A nephropathy

                            Membranous nephropathy

• Associated with secondary GN

                           Post-infectious GN

                           SLE

                           HSP

                           Cryoglobulinaemia 

• Negative immunofluorescent pattern (pauci-immune RPGN)

                ANCA associated systemic vasculitidies