![]() These antibodies will cause glomerular capillary wall injury by local complement activation and polymorphonuclear leukocytes. In anti-GBM disorder, there are circulating antibodies usually, IgG directed against an antigen present in the GBM and/or alveolar basement membrane, specifically the non-collagenous domain of alpha-3 chain of type IV collagen. Each disease leads to this pathway in different ways: The primary feature of crescentic glomerulonephritis is the rupture of the basement membrane followed by extra capillary fibrin precipitate, and this, followed by the proliferation of parietal cells and formed capsular proliferate in a crescent shape. RPGN is a very rare cause of end-stage kidney disease (ESKD) in various case studies. The younger population is more associated with this kind of presentation, but once immunosuppressive treatment started, the infection is the commonest cause for mortality. The cause of mortality in RPGN is usually pulmonary involvement in ANCA-associated disease. Pauci-immune disorder: most common type of crescentic glomerulonephritis 65 to 70%, mainly white patients, with peak age 60 to 85 years, the majority of patients have anti neutrophilic antibody ANCA positive. Immune complex glomerulonephritis comprises 25 to 30% of all cases of rapid progressive glomerulonephritis. It has a slight male predominance M: F 3:2 and has a bimodal distribution, and the mean age is around 30 years and the second peak in the late sixty to seventies. įor anti-glomerular basement membrane (GBM) disease: 10 to 15% of all diffuse crescentic glomerulonephritis, with an incidence of 0.5-0.9/million/year, mainly seen in white patients. But the prevalence has been reported from the age group 2 years to 92 years, although it is very rare in the pediatric population. In most of the series, the mean age is around 30 years and the second peak in the late sixty to seventies. The disease represents a bimodal distribution related to the mean age for clinical manifestation. The male-to-female ratio in most of the studies is approximately 1:1. It is relatively uncommon in African Americans. It is more common among the white population, and some reported incidences in the Asian population. There are other reported clusters all over the world, suggesting a possible environmental influence on the pathogenesis. The incidence in the United States of America is around 7 cases per 1 million person-years, while it is 2 cases per 1 million person-years reported in the United Kingdom. Rapidly progressive glomerulonephritis is very rare worldwide. There are various drugs associated with the GN (renal limited or systemic) Eosinophilic granulomatosis with polyangiitis – EGPA, Churg-Strauss syndromeĤ. Granulomatosis with polyangiitis, previously called Wegener granulomatosisģ. Membranoproliferative glomerulonephritisĬ) Pauci-immune disorder: almost 80 to 90% of cases are positive for ANCA.ġ. Immunoglobulin A nephropathy without vasculitisħ. Henoch-Schönlein purpura – there is immunoglobulin A deposits and associated systemic vasculitisĥ. Postinfectious GN, especially after a Streptococcus infectionĤ. or associated with the positive anti-neutrophil cytoplasmic antibody (ANCA), sometimes called “dual antibody disease” or “double positive in which patient has crescentic GN and positive for both ANCA and anti-GBM antibody, some literature showed 10% to 50% of patients with the anti-GBM disease have detectable ANCA (usually recognizing myeloperoxidase ), and up to 10% of patients with ANCA also have circulating anti-GBM antibodies.ī) Granular immune complex disorder: it can be idiopathic or secondary to the following:ġ. or with pulmonary hemorrhage The combination of glomerulonephritis and pulmonary hemorrhage referred to as Goodpasture syndromeģ. Crescentic glomerulonephritis alone (renal limited variant)Ģ. It can be presented as one of the following features:ġ. It is approximately 10% to 15% of all diffuse crescentic GN. Rapidly progressive glomerulonephritis is broadly classified based on the histopathology and immune complex deposition as follows:Ī) Linear antibody deposition, anti-glomerular basement membrane (GBM) disease: it is circulating antibodies IgG directed against an antigen normally present in the GBM and/or alveolar basement membrane, specifically the non-collagenous domain of alpha-3 chain of type IV collagen. ![]()
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