Systemic sclerosis and antineutrophil cytoplasmic autoantibady-associated renal failure

We report three patients who developed antineutrophil cytoplasmic autoantib ody (ANCA)-associated crescentic glomerulonephritis, two of whom showed cli nical features of limited scleroderma and one whose results of serological tests were suggestive of limited scleroderma without cutaneous features. Al l had anticentromere antibodies and antimyeloperoxidase antibodies. No pati ent showed the features of typical scleroderma renal crisis such as acceler ated hypertension or microangiopathy. Our patients were normotensive at the time of onset of renal failure, and the clinical picture was characterised by only modest features of limited scleroderma. All three patients had cre scentic glomerulonephritis at various stages of chronicity. One patient res ponded to immunosuppressive therapy with improvement in renal function; the other two patients rapidly developed end-stage renal failure. These patien ts and others recently described may represent a newly described form of sc leroderma renal disease.