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Case 6

Case 6. Dr Manoj Jain Additional Professor Department of Pathology Sanjay Gandhi Postgraduate Institute of Medical Sciences Lucknow. 17 Yrs / Male C/O Abdominal Pain - 2 weeks Skin Rash (Lt & Rt LL, red,flat, mildly pruritic) - 2 weeks

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Case 6

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  1. Case 6 Dr Manoj Jain Additional Professor Department of Pathology Sanjay Gandhi Postgraduate Institute of Medical Sciences Lucknow

  2. 17 Yrs / Male C/O • Abdominal Pain - 2 weeks • Skin Rash (Lt & Rt LL, red,flat, mildly pruritic) - 2 weeks • Polyarthralgia with arthritis of Lt elbow joint - 10 days • Heamturia - 1 week O/E • Flat erythemaous rash, varying size all over body, no ulceration or scaling • No palor/ icterus • Rest Examination - WNL

  3. Hb– 10.4 Gm/DL • TLC 9.8 • DLC : P72, L28,E0 M0 • Reticulocyte 3.5% • Platelet 266

  4. Urine Examination • Proteins (+++), Glucose (–) • M/E (RBcs 40-45/hpf, Leucocytes 10-12/hpf, Casts hyaline++) • S. Glucose (R) 70mg% • S. Creatinine 0.91 mg% • S. Proteins, Tot 6.37 • S. Albumin 3.24 Gm% • ANA - Negative • ANCA- Negative (MPO- 2.17, PR3- 2.59 RU/ML)

  5. Presented with - Fever, arthritis, muscle swelling, palpable purpura, abdominal pain Clinical Diagnosis: • Henoch Schonlein Purpura with Subnephrotic Proteinuria • Skin & Renal Biopsy performed

  6. IgA IgA

  7. IgA

  8. Skin Biopsy M/E Dermis- neutrophilic infiltration of blood vessel, perivascular neutrophilc karyorrhetic debris, extravasation of RBC and foci of fibrinoid necrosis. I/F- Granular IgA in the dermal blood vessels (1-2+). IgG, IgM and C3 are negative. LEUKOCYTOCLASTIC VASCULITIS

  9. KIDNEY BIOPSY • M/E • Single renal core, 23 glomeruli with lobular accentuation & focal endocapillary proliferation. • Mild mesangial proliferation in 40% glomeruli & partial cellular crescent in one glomerulus • The tubules, interstitium and blood vessels unremarkable. • Morphological Diagnosis • Focal Proliferative Glomerulonephritis (Possibility of HSP)

  10. IgA

  11. IgA

  12. IgM

  13. C3

  14. Immunoflourescence: two cores, 16 glomeruli • IgM 2+ granular mesangial deposits • IgA 3+ granular mesangial deposits • C3 1+ granular mesangial deposits • IgG nonspecific & C1q negative

  15. DIAGNOSIS • Henoch Schonlein Purpura with Focal Proliferative Glomerulonephritis (Class III -ISKDC Classification of HSP Nephritis)

  16. Follow-up • Started on Wysolone 1 mg/ day • During hospital stay-complaints subsided with decrease in abdominal pain,hematuria, arthritis and skin rash. • Discharged on steroids to be tapered over 6 months

  17. ISKDC Histologic Classification of HSP Nephritis

  18. D/D of HSP • Microscopic PAN • SLE • Mixed Cryoglobulinemia • IgA dominant Post-infectious GN

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