An unusual blend; IgA nephropathy and anti GBM disease

Q4 Medicine Journal of Nephropathology Pub Date : 2023-06-19 DOI:10.34172/jnp.2023.17325
N. Jose, E. Indhumathi, M. Bindra, Selvin Sundar Raj, M. Jayakumar
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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.
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不寻常的混合;IgA肾病与抗GBM疾病
抗肾小球基底膜病是一种罕见的疾病,临床表现广泛。在25-60%的病例中,抗GBM的典型表现(90%的病例)是快速进行性肾小球肾炎(RPGN)伴肺部疾病。在其非典型形式中——在10%的病例中可见,抗GBM疾病呈慢性形式,与典型形式的疾病相比,表现为长期肾功能障碍、蛋白尿和更好的肾预后。已知的抗GBM疾病与抗中性粒细胞胞质抗体(ANCA)血管炎和膜性肾病有关。在本病例报告中,一位患有非典型抗GBM疾病的年轻女士被描述为与IgA肾病有最不寻常的关联。这种关联是罕见的,在世界范围内只有少数病例报告描述。还阐明了该病可能的发病机制、临床特点、治疗和转归。
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来源期刊
Journal of Nephropathology
Journal of Nephropathology Medicine-Nephrology
CiteScore
1.30
自引率
0.00%
发文量
35
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