Objective: To elucidate the distribution and frequency of antineutrophil cytoplasmic autoantibodies (ANCA) in systemic sclerosis.
Methods: Patient sera were analyzed by indirect immunofluorescence and by enzyme linked immunosorbent assay.
Results: High titers for perinuclear ANCA correlated with rapid deterioration of renal function. Patients were normotensive with normal plasma renin levels, but anemia, pulmonary hemorrhage and thrombocytopenia were problematic. All patients had anti-myeloperoxidase antibodies and some had anti-nRNP, anti-DNA, anti-Sm and LE cell antibodies. Outcome was poor except for one patient treated by plasmapheresis and cyclophosphamide.
Conclusion: A renal specific subset of systemic sclerosis is suggested.