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Summary
The antiglomerular basement membrane (anti-GBM) antibody disease is marked by the presence of specific antibodies against the non-collagenous domain of the type IV collagen’s α3 chain. We describe a case of a 24-year-old Caucasian man, who may have had an atypical presentation of anti-GBM (slow progressive renal insufficiency, massive proteinuria and no detectable circulating anti-GBM antibody). The patient was treated with steroids and cyclophosphamide. This approach failed to attenuate the disease, and so rituximab was initiated with subsequent clinical improvement, normalisation of urinary sediment and marked regression of proteinuria; renal function remained stable. The renal biopsy immunofluorescence was crucial for the diagnosis.
- nephrotic syndrome
- acute renal failure
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Footnotes
Contributors ACT, HP and Nuno Oliveira participated in the treatment patient and continue to follow the patient. Nuno Oliveira performed the kidney biopsy. CM, the pathologist, made the diagnosis based on the results of the biopsy and provided the images of the biopsy. The bibliographical research, as well as the elaboration of the manuscript, was carried out by ACT. HP was responsible for the text formatting. The review of the article was carried out by Nuno Oliveira.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.