全身性強皮症の病像と臨床経過 : 混合性結合組織病との比較

概要

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To clarify the clinical features and course of systemic sclerosis(SSc)based on antinuclear antibodies, we studied 136 patients with SSc and compared them with those with mixed connective tissue disease(MCTD), which were studied by the MCTD Research Committee. The SSc patients were observed for more than 2 years and excepted for overlap syndrome. The 136 patients were classified by antinuclear antibody(ANA)pattern into 28 patients with anti-Ul-RNP antibody, 24 with anticentromere antibody(ACA)and 84 without these antibodies. The clinical features linked to these ANA patterns were low frequency of diffuse scleoderma in ACA and anti-Ul-RNP antibody groups, and high frequency of renal crisis in anti-Ul-RNP groups. The frequencies of diffuse scleoderma, renal crisis and pulmonary hypertension were increased during the follow-up periods(7.2±4.7 years). Twenty-nine patients(21.3%)died. Survival rates from the time of diagnosis calculated by the life table method were 91.3% at 5 years. Patients with SSc and anti-Ul-RNP antibody had a low survival rate(85.4% at 5 years), whereas MCTD patients had a high survival rate(93.1% at 5 years). This finding suggests that these 2 diseases with the same antibody may belong to different categories.
日本結合組織学会の論文
1998-03-25