Anti-glomerular Basement Membrane Disease: epidemiological, clinical, laboratory, morphological, and immunophenotypic characteristics

Abstract

Introduction: Anti-glomerular basement membrane (anti-GBM) disease is a rare condition, classified as rapidly progressive glomerulonephritis (RPGN), with an annual incidence of less than 2 cases per million inhabitants. It is confirmed by renal biopsy showing crescents on light microscopy, which is associated with diffuse linear Ig Immunostaining along the glomerular basement membrane immunofluorescence. Prognosis depends on the severity of renal dysfunction at initial presentation, and timely diagnosis and early therapy are responsible for renal survival rates. Methods: A cross-sectional observational study was conducted using data collected from renal biopsy reports of 124 patients with RPGN. Results: The collected sample enabled the study to obtain a p-value <0.05 with significant associations. Patients with anti-GBM RPGN are predominantly male (72%). The mean age found was 46 years (Type I). Regarding the distribution of crescents, Class I showed crescents predominantly within the same evolutionary stage (proliferative or sclerosing). Regarding vascular thickness, vessels of normal thickness or slightly thickened prevailed. The degree of fibrosis was pronounced in 44% in Type I. Conclusion: New scientific data on this rare and poorly understood disease, especially in Brazil, were obtained. The morphological pattern showed that anti-GBM disease presents with severe evolutionary stages and pronounced tubulointerstitial involvement, requiring prompt treatment to improve renal and overall survival.