Gopambuj Singh Rathod*, Atanu Pal, Pallavi Mahato, Aakash Roy, Debroop Sengupta and Muzzamil Ahmad
Published on: 13th September, 2024
Anti-glomerular basement membrane (GBM) antibody glomerulonephritis is an extremely rare glomerular disease. Around 90% of the patients are positive for serum anti-GBM antibodies while up to 10% can be negative. In such patients, only a kidney biopsy can reveal the anti-GBM disease it is then labeled as an atypical anti-GBM disease. Though anti-GBM disease can be associated with Anti Neutrophil Cytoplasmic Antibodies (ANCA) positivity, it is extremely rare to find atypical anti-GBM with ANCA positivity so much so that till now there are very few such cases reported from across the world.The case presented here is one such case where the patient presented with adult-onset nephrotic syndrome features with active urinary sediments and mildly deranged renal function. Myeloperoxidase (MPO) ANCA was positive and it was considered ANCA-associated crescentic glomerulonephritis (GN) but after the renal biopsy the picture was of anti-GBM disease. She was treated with pulse methylprednisolone but her creatinine increased in the meantime and considering anti-GBM she was put on Plasma Exchange (PLEX). She received 5 sessions of PLEX after which her renal function improved. She was also planned for Rituximab which could not be given due to local infection. As there are no protocols for treating such cases because of the extremely rare nature of the presentation, this case will increase the understanding of such presentations for the clinicians. This will help to plan for building the approach for such cases.
Double-Positive Patients (DPPs), characterized by the simultaneous presence of Anti-Neutrophil Cytoplasmic Antibody (ANCA) and anti-Glomerular Basement Membrane (anti-GBM) antibodies, represent a rare subset in systemic vasculitis. We present two cases of DPPs with renal involvement and review the existing literature to elucidate the clinical characteristics, histopathological findings, management strategies, and prognostic outcomes associated with this condition. Both cases exhibited renal involvement with rapidly progressive glomerulonephritis, requiring renal replacement therapy. Renal biopsies confirmed crescentic glomerulonephritis with features of both anti-GBM disease and ANCA-associated vasculitis. Management included high-dose glucocorticoids, cyclophosphamide, and consideration of plasma exchanges. Double-positive ANCA and anti-GBM vasculitis pose challenges in management and prognosis. Further research is essential to improve therapeutic strategies for this rare and heterogeneous condition.
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