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Case 3 55-year man presenting with nephrotic syndrome and RPRF

Case 3 55-year man presenting with nephrotic syndrome and RPRF. Dr. Vinita Agrawal Additional Professor Department of Pathology SGPGIMS, Lucknow. Clinical presentation. 55 year gentleman Not a known diabetic/ hypertensive Edema- 1month Decreased urine output- 2weeks

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Case 3 55-year man presenting with nephrotic syndrome and RPRF

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  1. Case 355-year man presenting with nephrotic syndrome and RPRF Dr. Vinita Agrawal Additional Professor Department of Pathology SGPGIMS, Lucknow

  2. Clinical presentation • 55 year gentleman • Not a known diabetic/ hypertensive • Edema- 1month • Decreased urine output- 2weeks • Fever with chills- 2weeks • Breathlessness-2weeks • Anorexia, nausea, recurrent vomiting- 2weeks

  3. Clinical presentation • No history of • Hematuria • systemic symptoms: cough, expectoration, joint pains, rashes, sore throat

  4. On examination • PR-110/min • BP- 180/90 mm Hg • Pallor+, edema+ • Chest- bilateral crepitations • No organomegaly

  5. Investigations

  6. Investigations

  7. Clinical diagnosis • 55 year-male patient with nephrotic range proteinuria • Recent onset • Renal failure • Active sediments RPRF with nephrotic range proteinuria ? Crescentic GN

  8. Renal histology

  9. Salient histological features • Enlarged glomeruli • Diffuse endocapillary proliferation • Neutrophilic exudation • Widespread splitting of GBM • Capillary BM thickening (wire loop-like) • Hyaline thrombi • Acute tubular necrosis

  10. Histological diagnosis and differential diagnosis Proliferative Glomerulonephritis (MPGN) • MPGN type I • C3 glomerulopathy • Cryoglobulinemia • Lupus Nephritis • Secondary GN- HCV or HBV related?

  11. IgG (3+) IgA (4+) • IgM, C1q negative • K, λ equal intensity C3 (4+)

  12. EM findings • Focal foot process effacement • Extensive electron dense deposits: sub-endothelial and in capillary lumens • Glomerular basement membrane duplication

  13. Further work-up

  14. Investigations

  15. Final Diagnosis • Cryoglobulinemia • S. Cryoglobulins negative • No hyper-gammaglobulinemia • Lupus Nephritis • No systemic manifestations • ANA negative • Full-house on IF absent • Secondary causes- HCV or HBV • Serology negative • C3 glomerulopathy and dense deposit disease • Presence of IG’s on IF • S. C4 low (marginally) • No dense deposits on ultrastructure • MPGN type I

  16. Final Diagnosis • IgA Nephropathy with MPGN-like pattern (M1E1S0T0)/HAAS Class IV • ? Features • Extensive sub-endothelial deposits • Hyaline thrombi and wire loop-like lesions • MPGN type I (IgA-dominant)

  17. IgA Nephropathy with MPGN-like pattern • Rare; few case reports • Usually reported in children* • IgA-dominant post-infectious GN may have a MPGN-like pattern and hyaline thrombi ǂ * Iitaka, K. IgA-associated GN with MPGN-like pattern in 2 children. Clin.Exp. Nephrol. 2003; 7:284-9. * Akira K et al. Infantile IgAN showing features of MPGN. Tohoku J Exp. Med. 2012;228:253-58. ǂWorawichawong S et al. IgA-dominant postinfectious GN. Hum. Pathol. 2011; 42: 279-84  Satoskar A et al. Staph. infection-associated GN mimicking IgAN. Clin J Am SocNephrol. 2013;1: 1179.

  18. No description of MPGN-like pattern • Peripheral capillary deposits (23%) had no prognostic significance

  19. Summary • Diagnostic dilemma- IgAN with MPGN- like pattern/ MPGN (IgA-dominant) • Limited description in literature • No association with the common secondary causes

  20. Follow-up Intensive treatment- Steroids (3 months) Remission Maintenance on Azoran + ACE receptor blockers • Recent visits (Feb. and August 2013) • S. creatinine- 1.2mg%, no proteinuria or active urinary sediments

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