Nisha Jose
1 , Elayaperumal Indhumathi
2* , Mandeep Bindra
3, Selvin Sundar Raj
1, M Jayakumar
11 Department of Nephrology, Christian Medical College and Hospital, Vellore, India
2 Department of Nephrology, Saveetha medical college and hospital, Chennai, India
3 Departement of General Pathology, Naruvi Hospitals, Vellore, India
Abstract
Anti-glomerular basement membrane (anti-GBM) disease is a rare illness with a wide spectrum of clinical manifestations. The typical presentation (90% of cases) of anti-GBM is with a rapidly progressive glomerulonephritis (RPGN) in conjunction with pulmonary disease in 25-60% of cases. In its atypical form – seen in 10% of cases, anti GBM disease takes on a chronic form, presenting with long standing renal dysfunction, proteinuria and better renal prognosis when compared to the typical form of the disease. The known associations of anti-GBM disease are with anti-neutrophil cytoplasmic antibody (ANCA)vasculitis and membranous nephropathy. In this case report, a young lady with atypical anti-GBM disease is described with a most unusual association with IgA nephropathy. This association is rare and only described in few case reports worldwide. The possible pathogenesis, clinical features, treatment and outcome of this disease are also elucidated.
Implication for health policy/practice/research/medical education:
Anti-glomerular basement membrane (Anti-GBM) disease is commonly associated with anti-neutrophil cytoplasmic antibody (ANCA) vasculitis and membranous nephropathy. It is association with IgA nephropathy is very unconventional. Traditional anti-GBM assays often do not detect the same from plasma since the antibodies are directed against a different antigenic target. The disease is less aggressive and renal prognosis is better than conventional anti-GBM disease.
Please cite this paper as: Jose N, Indhumathi E, Bindra M, Sundar Raj S, Jayakumar M. An unusual blend; IgA nephropathy and anti GBM disease. J Nephropathol. 2023;x(x):e17325. DOI: 10.34172/jnp.2023.17325.