Antineutrophil cytoplasmic autoantibodies in 6 patients with renal failure and systemic sclerosis.
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.


CONCLUSIONS
A renal specific subset of systemic sclerosis is suggested.
Endo
H
H
Department of Internal Medicine, Kitasato University School of Medicine, Kanagawa, Japan.
Hosono
T
T
Kondo
H
H
D058186:Acute Kidney Injury / Q000097:blood / Q000150:complications / Q000276:immunology*; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies / Q000097:blood*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000097:blood / Q000150:complications / Q000276:immunology*
D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies