Abstract
Pauci-immune necrotizing glomerulonephritis is a form of a rapidly progressive glomerulonephritis that is associated with the anti-neutrophil cytoplasmic antibodies (ANCA). ANCA-associated pauci-immune glomerulonephritis may be secondary to rheumatic diseases. We report on a patient with limited cutaneous systemic sclerosis who presented with subacute onset of nephritic syndrome and renal insufficiency. Her myeloperioxidase ANCA titer was elevated and renal biopsy confirmed pauci-immune crescentic glomerulonephritis, which was stabilized with high-dose glucocorticoid and cyclophosphamide. ANCA-associated necrotizing glomerulonephritis is rarely reported in systemic sclerosis and a systematic review is presented.
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Chan, P.T., Mok, C.C. Pauci-immune crescentic glomerulonephritis in limited cutaneous systemic sclerosis. Clin Rheumatol 31, 1273–1277 (2012). https://doi.org/10.1007/s10067-012-2010-8
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DOI: https://doi.org/10.1007/s10067-012-2010-8