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ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,

ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident, PDCC-Renal and Transplant Pathology Department of Histopathology PGIMER, Chandigarh. Clinical history:

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ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,

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  1. ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident, PDCC-Renal and Transplant Pathology Department of Histopathology PGIMER, Chandigarh

  2. Clinical history: • A 40 year old male with no previous comorbidities presented with complaints of facial puffiness and oedema of lower extremeties of 7 months duration. • Found to have proteinuria and was referred to Nephrologist

  3. General Physical examination- • PICKL- Negative • Pedal and periorbital oedema-+ • Normotensive (BP-110/80mmHg) • Systemic examination: NAD

  4. Laboratory investigations- • SCreatinine -1.1mg/dL • 24hr urine protein -6.9g • Urine - 3+ protein with no active sediments • Serologies: • Negative for HCV, HBsAg, HIV, ANA • dsDNA, c-ANCA, p-ANCA • Rheumatoid factor and cryoglobulin • LFT- WNL • CBC- No significant abnormality

  5. Clinical diagnosis • A syndromic diagnosis of nephrotic syndrome was considered and an ultrasound guided percutaneous kidney biopsy was performed. • Clinical possibilities- • Membranous glomerulonephritis • Focal segmental glomerulosclerosis

  6. H/E stain PAS stain

  7. H/E stain PAS stain

  8. Biopsy findings Light microscopic examination Suggestive of Membranous Glomerulonephritis

  9. IgG C3

  10. KAPPA LAMBDA

  11. IgG2 IgG1 IgG3 IgG4

  12. Direct immunofluorescence staining • 8 glomeruli • Granular positivity (3+)along capillary loops • IgG • C3 • Kappa IgA,IgM,C1q, PLAR 2- Negative • IgG Subtyping –IgG3 + Monoclonal IgG3-kappa deposition and C3 along the glomerular capillary loops

  13. Electron microscopy revealed subepithelial electron dense immune deposits.

  14. Diagnosis • Monoclonal immunoglobulin deposition disease with membranous nephropathy • LM: Diffusely thickened GBM • DIF: IgG3,Kappa, C3 deposits • EM: Subepithelial Immune complex type deposits

  15. Post biopsy work-up • SPEP, UPEP, BJP- Negative • Serum free light chain assay- ↑κ : λ ratio (5.2:1) • Bone Marrow: 2% of plasma cells • Serum Ca- WNL • Radiology: No lytic lesions

  16. Monoclonal immunoglobulin deposition disease associated with membranous features

  17. Therapy • Patient was treated with steroids

  18. Follow up • Symptomatic improvement with↓Proteinuria • Serum free light chain assay- Awaited

  19. Till date 13 cases of MIDD with membranous morphology have been described in the English literature. This is the fourteenthsuch case worldwide • Only three (21%) of the 14 patients with available data had a monoclonal protein in serum or urine • Three cases had overt B-cell neoplasms

  20. Definition • The modified criteria for proliferative GN with monoclonal IgG deposits: • The presence of glomerular monoclonal IgG deposits restricted to a single IgG subclass and a single light chain isotype, associated with membranous features without proliferative patterns • The presence of granular (‘immunecomplex type’) deposits by electron microscopy • The absence of clinical and laboratory evidence of cryoglobulinaemia

  21. Clin Exp Nephrol (2012) 16:468–472

  22. Distinct entity or just a different morphological manifestation of the same disease? • Therapy?

  23. Acknowledgements • Prof Kusum Joshi • Dr Ritambhra Nada • Dr Raja Ramchandran • Dr CS Rayat • Dept. of Nephrology

  24. THANK YOU

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