Atypical anti-GBM disease in pregnancy

A woman in her 20s presented with nephrotic syndrome and hyperemesis in early pregnancy. Pertinent initial investigations revealed a severe acute kidney injury, a serum albumin of 19 g/L, a random protein creatinine ratio of 800 g/mol and microscopic haematuria. All immunological and infection serology testing including anti-glomerular basement membrane (anti-GBM; ELISA) were negative. Kidney biopsy demonstrated diffuse crescentic glomerulonephritis with cellular crescents involving >90% of glomeruli, with immunofluorescence demonstrating intense linear reactivity for IgG consistent with atypical anti-GBM glomerular nephritis. Early pregnancy termination and treatment with immunosuppression were chosen after shared decision-making between the patient and physician. The patient had a poor response to treatment and remained dialysis dependent 12 months later.