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MR Vol.14 No.3 indexに戻る
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MODERN RHEUMATOLOGY
Vol.14 No.3 |
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Classification of systemic sclerosis in the Japanese
population based on rapid progression of skin thickening |
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| Emi Nishimagi1, Yasushi Kawaguchi1 , Eiichi Tanaka1,
Masako Hara1 and Naoyuki Kamatani1 |
| (1) Institute of Rheumatology, Tokyo Womens Medical
University, 10-22 Kawada-cho, Shinjuku-ku, Tokyo, 162-0054, Japan |
Received: 11 July 2003 Accepted: 27 February
2004 |
| Abstract |
| In general, patients with systemic sclerosis
(SSc) have been classified in two clinical subsets (diffuse and limited)
based on the extent of skin thickening, and the extent of skin fibrosis
is closely related to the severity of organ involvement and mortality
rates. In addition, there is a rapid progression of skin thickening
in diffuse cutaneous SSc (dcSSc). In this study, we classified a
novel subset of SSc based on the rate of skin thickening progression,
and evaluated the relationship of the subset to disease severity
and laboratory markers. Thirty-three patients with dcSSc were included
in the study. Participants who had a modified Rodnans total skin
thickness score higher than 15 points within a year from the first
symptoms were defined as having rapidly progressive SSc (RPSSc; group
1), while all the other dcSSc patients were defined as having non-RPSSc
(group 2). The frequencies of interstitial lung disease and renal
crisis were significantly higher in group 1 than in group 2. The
frequencies of antitopoisomerase I antibody (anti-topo I) and anti-SS-A
antibody were significantly higher in group 1 than in group 2. Conversely,
the frequency of anticentromere antibody was significantly higher
in group 2 than in group 1. This study demonstrates the clinical
significance of a new subset of SSc (defined as RPSSc), which is
characterized by a markedly rapid progression of skin thickening.
This new classification may be useful for predicting the prognosis
of SSc at an early stage, so that patients who should receive aggressive
therapy with immunosuppressants might be identified. |
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| Key words |
| Classification - Systemic sclerosis (SSc) - Total
skin score |
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