Coexistence of anti-glomerular basement membrane disease and IgA nephropathy: an illustrative case and comprehensive literature review

Abstract

Anti-glomerular basement membrane (GBM) disease is a rare autoimmune condition characterized by the presence of positive anti-GBM autoantibodies, linear deposition of immunoglobulin G (IgG) along the GBM and severe kidney injury. In a limited number of cases, the association of anti-GBM disease with other glomerulonephritis has been reported. Herein, we present the case of a 66-year-old female patient with progressive worsen kidney function and decreased urine output. A renal biopsy revealed crescent glomerulonephritis with lineal IgG deposition along the GBM and mesangial IgA deposition, which supported the diagnosis of concurrent anti-GBM disease and IgA nephropathy (IgAN). In an extensive literature review, we identified a total of thirty-nine patients were reported anti-GBM disease combined with IgAN. The clinical characteristics of these patients demonstrate that the anti-GBM disease combined with IgAN tends to be milder with a more indolent course and a better prognosis than the classic anti-GBM disease, and its potential pathogenesis deserves to be further explored.

Keywords: Anti-glomerular basement membrane disease; IgA nephropathy; crescentic glomerulonephritis; immunoglobulin G; prognosis.

Figure 1.

Pathological results of kidney biopsy and clinical course. (A) Light microscopy showing the formation of large cellular crescents (PASM staining, × 200). (B) Immunofluorescence showing linear deposition of IgG along the GBM. (C) IgG subclass shows capillary lineal deposition of IgG3. (D) Immunofluorescence showing lumpy deposition of IgA in the mesangium. (E) Clinical course of patient. GBM, glomerular basement membrane; Ig, immunoglobulin; Scr creatinine; HD, hemodialysis; PE, plasma exchange; MethyI-PD, Methylprednisolone; IV, intravenous; CYC, cyclophosphamide; w. week; m, month.