PT  - JOURNAL ARTICLE
AU  - Emi Nishimagi
AU  - Akiko Tochimoto
AU  - Yasushi Kawaguchi
AU  - Takashi Satoh
AU  - Masataka Kuwana
AU  - Kae Takagi
AU  - Hisae Ichida
AU  - Tokiko Kanno
AU  - Makoto Soejima
AU  - Sayumi Baba
AU  - Naoyuki Kamatani
AU  - Masako Hara
TI  - Characteristics of patients with early systemic sclerosis and severe gastrointestinal tract involvement.
DP  - 2007 Oct 01
TA  - The Journal of Rheumatology
PG  - 2050--2055
VI  - 34
IP  - 10
4099  - http://www.jrheum.org/content/34/10/2050.short
4100  - http://www.jrheum.org/content/34/10/2050.full
SO  - J Rheumatol2007 Oct 01; 34
AB  - OBJECTIVE:To clarify the clinical features of patients with systemic sclerosis (SSc) who developed severe gastrointestinal tract (GIT) involvement in the early stage of the disease. 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.