Acute Kidney Failure in Limited Scleroderma Reveals Anti-GBM--Associated Kidney Disease

Although scleroderma renal crisis (SRC) is the most common form of kidney injury in patients with scleroderma, consideration of other autoimmune conditions is warranted in patients with atypical presentation. A 27-year-old woman with a history of limited scleroderma presented to the hospital with 1 month of uremic symptoms and oliguria. Initial laboratory tests revealed significantly elevated serum urea nitrogen and creatinine levels, suggesting kidney failure. Suspicion for SRC was low, and kidney biopsy was performed to evaluate further. Histopathology revealed severe diffuse crescentic glomerulonephritis involving almost all glomeruli. Immunofluorescence revealed a diffuse linear reaction for immunoglobulin G along capillary loop basement membranes, consistent with antiglomerular basement membrane (GBM) disease. Further work-up revealed a positive antineutrophil cytoplasmic antibody panel for antimyeloperoxidase antibodies. Anti-GBM levels were also elevated. Treatment for anti-GBM disease was not warranted in our patient as the severity of kidney injury histopathology suggested unlikely kidney recovery. She was initiated on outpatient dialysis, and discussion regarding kidney transplant was initiated. There is a rare association between scleroderma and anti-GBM disease. Anti-GBM disease should be considered in scleroderma patients presenting with acute kidney failure with atypical features for SRC, especially if they have a history of antineutrophil cytoplasmic antibody positivity.