Introduction:
IgA nephropathy is the most prevalent pattern of glomerular disease characterised by mesangial deposition of IgA +/- C3 deposition. Anti - glomerular basement membrane (GBM) disease is a rare autoimmune disorder with renal biopsy showing linear IgG staining along GBM accompanied by crescentric glomerulonephritis. Anti-GBM antibody is detected in about 95% patients. Though sporadic cases have been reported, it is extremely rare to find the two pathologies to coexist. The authors report 5 cases of this dual pathology association that illustrates the difficulty in diagnosing and managing such patients.
Methods:
This was a prospective study conducted between January 2023 - June 2024 in the Department of Nephrology at a large tertiary care centre hospital in Eastern India. Histopathological examination of renal biopsy was done at Department of Pathology, IPGME&R & SSKMH. All patients with biopsy- proven Anti-GBM disease with coexistent IgA nephropathy with or without Anti-GBM antibodies were included. After obtaining informed consent, data was collected on demography, clinical presentation and course, treatment and outcomes. Investigations were done to look for other systemic involvement on case to case basis. ANCA assay was performed in all cases. All patients received iv pulse methylprednisolone for 3 days followed by oral Prednisolone. Further immunosuppression and plasmapheresis were decided based on individual profile.
Results:
5 patients were found to have coexistent Anti-GBM disease with IgA nephropathy.
All patients were females with age ranging from 19 - 44 years. 1 patient was diabetic, other had no comorbidities.
All presented with rapidly progressive renal failure with Serum Creatinine at presentation ranging from 2.52 - 15.2.
None of them had pulmonary involvement. 2 patients had neurological involvement in the form of PRES. 1 patient had GI bleed, diagnosed with stricture at sigmoid colon with HPE from stricture showing inflammatory cells.
3 out of 5 patients had undetectable Serum Anti GBM antibody. ANCA was negative in all cases.
All patients had 100% crescents on biopsy with IFTA ranging from 20-70%.
4 out of 5 patients did not respond to treatment comprising of plasmapheresis and immunosuppressant (steroid +/- cyclophosphamide) and continued to be hemodialysis dependent. One patient was diabetic, developed septic shock and died on day 15. 3 patients are being managed as CKD-5D. 1 patient was given Inj Rituximab induction to which she responded and renal function improved.
Conclusions:
This series reports 5 female patients of young – middle age seen at a single centre over 1.5 years harbouring concurrently dual pathology of Anti-GBM and IgA Nephropathy – 3 having Atypical anti-GBM disease. The primary pathology of crescents remains unclear. Renal outcomes were poor in this study. The origin of crescents, novel early markers and underlying pathophysiology of the relationship between IgA Nephropathy and anti GBM need further study.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.