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Characteristics of Patients with Early Systemic Sclerosis and Severe Gastrointestinal Tract Involvement EMI NISHIMAGI, AKIKO TOCHIMOTO, YASUSHI KAWAGUCHI, TAKASHI SATOH, MASATAKA KUWANA, KAE TAKAGI, HISAE ICHIDA, TOKIKO KANNO, MAKOTO SOEJIMA, SAYUMI BABA, NAOYUKI KAMATANI, and MASAKO HARA
ABSTRACT. Methods. Three hundred two consecutive Japanese patients with SSc were investigated: Group 1 comprised 14 patients with severe GIT involvement (malabsorption syndrome and/or pseudo-obstruction) within 2 years of onset of SSc; group 2 consisted of all patients without severe GIT involvement (n = 288); and group 3 consisted of 117 patients without severe GIT involvement within 2 years of onset of SSc. Autoantibodies were evaluated using double immunodiffusion, ELISA, and immunoprecipitation. Results. We found significant differences in clinical features among the 3 groups. Diffuse cutaneous type, erosive esophagitis, and myositis were more common in group 1 than in group 2 (p = 0.007, 0.003, and 0.003, respectively) or group 3 (p = 0.04, 0.002, and 0.01, respectively), whereas interstitial lung disease (ILD) was more frequent in group 2 (p = 0.005) and group 3 (p = 0.02) versus group 1. Antinuclear antibodies showed a nucleolar pattern significantly more frequently in group 1. Myositis-related autoantibodies, including anti-U1RNP, anti-U3RNP, anti-Ku, and anti–signal recognition particle antibodies, were observed in 57% of group 1. Conclusion. Our findings strongly suggest the existence of a subgroup of SSc patients with severe GIT involvement in the early stage. Among the Japanese individuals, these patients never developed severe ILD, even though they were classified as having diffuse cutaneous SSc. (J Rheumatol 2007;34:2050-5) Key Indexing Terms:
SYSTEMIC SCLEROSIS From the Institute of Rheumatology, Tokyo Women's Medical University School of Medicine, and Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan. Dr. Kawaguchi was supported by a systemic sclerosis research grant from the Ministry of Health, Labour and Welfare. E. Nishimagi, MD, PhD; A. Tochimoto, MD; Y. Kawaguchi, MD, PhD, Assistant Professor; K. Takagi, MD, PhD; H. Ichida, MD; T. Kanno, MD; M. Soejima, MD; S. Baba, MD; N. Kamatani, MD, PhD, Professor; M. Hara, MD, PhD, Professor, Institute of Rheumatology, Tokyo Women's Medical University; T. Satoh, MD; M. Kuwana, MD, PhD, Associate Professor, Rheumatology, Keio University School of Medicine. Address reprint requests to Dr. Y. Kawaguchi, Institute of Rheumatology, Tokyo Women's Medical University School of Medicine, 10-22 Kawada-cho, Shinjuku-ku, Tokyo 162-0054, Japan. E-mail: y-kawa@ior.twmu.ac.jp Accepted for publication July 5, 2007.
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