Presentation and outcomes of anti-GBM disease: a case series

Aim: to describe the clinical course and outcomes of the anti-glomerular basement membrane (anti-GBM) disease. Methods: in this case series we included patients with anti-GBM disease from different regions of the country. The diagnosis was established based on the clinical and laboratory features (rapidly progressive glomerulonephritis, diffuse alveolar hemorrhage (DAH), anti-GBM antibodies levels exceeding the reference range of the local lab) and/or morphological evaluation (extracapillary glomerulonephritis (ECGN) type I) according to the Chapel Hill 2012 consensus conference definition. Results: We enrolled six cases of anti-GBM disease, one male (24 y.o.) and five females (from 27 to 67 y.o.), who were diagnosed between 2016 and 2023. Among them, five had kidney involvement requiring kidney replacement therapy at the disease onset. Four patients had lung involvement, among them two required invasive ventilation due to DAH at the onset. Kidney biopsy was performed in two cases and showed ECGN type I with 100% crescents in both. All patients received glucocorticoids at an initial dose of 50-60 mg q.d. (prednisolone equivalent), five received cyclophosphamide (four of them oral), and four received plasma exchanges. It should be noted that the treatment regimen was in complete compliance with current guidelines only in two cases. Severe adverse events occurred in five patients, leukopenia and infections being the most common. All patients survived and achieved complete remission. Among five patients with kidney involvement four developed chronic kidney disease stage 5 and continued receiving kidney replacement therapy. Conclusion: management of anti-GBM disease presents a challenge in the real-world practice. Kidney survival remains unsatisfactory.

болезнь, ассоциированная с антителами к базальной мембране клубочка, хроническая болезнь почек, иммуносупрессивные препараты, плазмообмен, Anti-glomerular basement membrane (anti-GBM) disease, chronic kidney disease, immunosuppressive drugs, plasma exchange

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