Endo H, Hosono T, Kondo H
Department of Internal Medicine, Kitasato University School of Medicine, Kanagawa, Japan.
J Rheumatol. 1994 May;21(5):864-70.
To elucidate the distribution and frequency of antineutrophil cytoplasmic autoantibodies (ANCA) in systemic sclerosis.
Patient sera were analyzed by indirect immunofluorescence and by enzyme linked immunosorbent assay.
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.
A renal specific subset of systemic sclerosis is suggested.
阐明抗中性粒细胞胞浆自身抗体（ANCA）在系统性硬化症中的分布及频率。
采用间接免疫荧光法和酶联免疫吸附测定法对患者血清进行分析。
核周型ANCA高滴度与肾功能快速恶化相关。患者血压正常，血浆肾素水平正常，但存在贫血、肺出血和血小板减少问题。所有患者均有抗髓过氧化物酶抗体，部分患者有抗nRNP、抗DNA、抗Sm和LE细胞抗体。除1例接受血浆置换和环磷酰胺治疗的患者外，预后均较差。
提示系统性硬化症存在肾脏特异性亚组。
