![Page 1: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/1.jpg)
ISRTPCON and CMEAIIMS
NEW DELHI12-14 Sept,2013
Dr Kiran KSenior Resident,
PDCC-Renal and Transplant PathologyDepartment of Histopathology
PGIMER,Chandigarh
![Page 2: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/2.jpg)
Clinical history:o A 40 year old male with no previous comorbidities
presented with complaints of facial puffiness and oedema of lower extremeties of 7 months duration.
o Found to have proteinuria and was referred to Nephrologist
![Page 3: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/3.jpg)
General Physical examination-o PICKL- Negativeo Pedal and periorbital oedema-+o Normotensive (BP-110/80mmHg)
Systemic examination: NAD
![Page 4: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/4.jpg)
Laboratory investigations-o SCreatinine -1.1mg/dLo 24hr urine protein -6.9g o Urine - 3+ protein with no active sedimentso Serologies:
• Negative for HCV, HBsAg, HIV, ANA• dsDNA, c-ANCA, p-ANCA• Rheumatoid factor and cryoglobulin
o LFT- WNLo CBC- No significant abnormality
![Page 5: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/5.jpg)
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
![Page 6: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/6.jpg)
H/E stain PAS stain
![Page 7: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/7.jpg)
H/E stain PAS stain
![Page 8: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/8.jpg)
![Page 9: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/9.jpg)
Biopsy findings
Light microscopic examination Suggestive of Membranous Glomerulonephritis
![Page 10: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/10.jpg)
IgG C3
![Page 11: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/11.jpg)
KAPPA LAMBDA
![Page 12: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/12.jpg)
IgG3 IgG4
IgG1 IgG2
![Page 13: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/13.jpg)
Direct immunofluorescence staining
![Page 14: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/14.jpg)
![Page 15: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/15.jpg)
![Page 16: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/16.jpg)
• Electron microscopy revealed subepithelial electron dense immune deposits.
![Page 17: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/17.jpg)
Diagnosis
• Monoclonal immunoglobulin deposition disease with membranous nephropathy– LM: Diffusely thickened GBM– DIF: IgG3,Kappa, C3 deposits– EM: Subepithelial Immune complex type deposits
![Page 18: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/18.jpg)
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
![Page 19: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/19.jpg)
• Monoclonal immunoglobulin deposition disease associated with membranous features
![Page 20: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/20.jpg)
Therapy
• Patient was treated with steroids
![Page 21: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/21.jpg)
Follow up
• Symptomatic improvement with↓Proteinuria• Serum free light chain assay- Awaited
![Page 22: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/22.jpg)
• Till date 13 cases of MIDD with membranous morphology have been described in the English literature. This is the fourteenth such 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
![Page 23: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/23.jpg)
![Page 24: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/24.jpg)
![Page 25: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/25.jpg)
Definition• The modified criteria for proliferative GN with
monoclonal IgG deposits:1. 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
2. The presence of granular (‘immunecomplex type’) deposits by electron microscopy
3. The absence of clinical and laboratory evidence of cryoglobulinaemia
![Page 26: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/26.jpg)
Clin Exp Nephrol (2012) 16:468–472
![Page 27: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/27.jpg)
![Page 28: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/28.jpg)
![Page 29: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/29.jpg)
• Distinct entity or just a different morphological manifestation of the same disease?
• Therapy?
![Page 30: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/30.jpg)
Acknowledgements
• Prof Kusum Joshi• Dr Ritambhra Nada• Dr Raja Ramchandran• Dr CS Rayat• Dept. of Nephrology
![Page 31: ISRTPCON and CME AIIMS NEW DELHI 12-14 Sept,2013 Dr Kiran K Senior Resident,](https://reader035.vdocument.in/reader035/viewer/2022070410/568146a9550346895db3c476/html5/thumbnails/31.jpg)
THANK YOU