Pauci-immune crescentic glomerulonephritis in limited cutaneous systemic sclerosis.
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.
Department of Medicine, Tuen Mun Hospital and Center for Assessment and Treatment of Rheumatic Diseases, Pok Oi Hospital, New Territories, Hong Kong, SAR, China.
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Chan
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Mok
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D018501:Antirheumatic Agents / Q000008:administration & dosage; D001379:Azathioprine / Q000008:administration & dosage; D001706:Biopsy; D003520:Cyclophosphamide / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005260:Female; D005921:Glomerulonephritis / Q000150:complications* / Q000188:drug therapy; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage; D051437:Renal Insufficiency / Q000209:etiology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy
D019268:Antibodies, Antineutrophil Cytoplasmic; D018501:Antirheumatic Agents; D005938:Glucocorticoids; D003520:Cyclophosphamide; D011239:Prednisolone; D001379:Azathioprine