Abstract: PO1596
Circulating and Depositing Glomerular Antibodies: A Concurrence or Coexistence
Session Information
- Glomerular Diseases: Clinicopathological Features and Outcomes in IgAN, Lupus Nephritis, and Vasculitis
November 04, 2021 | Location: On-Demand, Virtual Only
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Ayanavelli, Deepthi, ESIC Medical College and Hospital Hyderabad, Hyderabad, Telangana, India
- Vurum, Dhanalakshmi, ESIC Medical College and Hospital Hyderabad, Hyderabad, Telangana, India
Introduction
Anti-GBM disease is a systemic autoimmune disorder characterized by circulating IgG antibodies (rarely IgA and IgM), may coexist with pauci-immune antineutrophil cytoplasmic autoantibody-positive glomerulonephritis and membranous glomerulopathy. The concurrent or sequential presentation of anti-GBM disease with IgA nephropathy has been rarely described.
Case Description
We herein report a case of 31-year-old female who had presented with sudden onset of breathlessness, pedal oedema for 15 days and oliguria for 5 days with 1 episode of haematuria. There were no arthralgias, oral ulcers, alopecia, skin rash, sore throat and diarrhoea. Her marital life was of five years with no history of conception or abortions. On examination she had mild pedal edema and her BP was 180/130mm Hg. Blood investigations revealed haemoglobin 9.5g/dl, urea 209 mg/dl, serum creatinine of 17mg/dl. Urine microscopy showed 3+protein and plenty of RBC and protein creatinine ratio 4.3g/g. Serologic test results were strongly positive for IgG anti-GBM antibody (73.97 units) by ELISA; serum C3 and C4 levels were normal. Other serologic tests for ANA, Anti ds DNA antibody, rheumatoid factor, cryoglobulinemia, hepatitis B and C, and ANCA were negative. Kidney biopsy showed 6 glomeruli of which 2 were sclerosed, 2 with cellular crescents, one with fibrocellular crescent and the other fibrous crescent. Interstitium filled with infiltrate of lymphocytes and tubules showed red cell casts. Vessels were unremarkable. IF showed mesangial granular staining with IgA (3-4+), C3c (2+); and kappa (3+) and lambda (3+) light chains.
The patient was initiated on hemodialysis and treated with three pulses of IV methylprednisolone followed by pulse cyclophosphamide. Plasmapheresis was done as there were cellular crescents in the biopsy. Patient progressed to ESRD and continuing on hemodialysis.
Discussion
Presentation of IgAN along with anti GBM without linear deposition on renal biopsy but with positive anti GBM titers makes this case interesting. The association of anti-GBM disease with IgA nephropathy could be a coexistence as IgAN is most common glomerular disease or these IgA mesangial deposits might have role in the pathogenesis of triggering GBM antigens and formation of antibodies in this case.