RT Journal Article
SR Electronic
T1 Characteristics of patients with early systemic sclerosis and severe gastrointestinal tract involvement.
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 2050
OP 2055
VO 34
IS 10
A1 Emi Nishimagi
A1 Akiko Tochimoto
A1 Yasushi Kawaguchi
A1 Takashi Satoh
A1 Masataka Kuwana
A1 Kae Takagi
A1 Hisae Ichida
A1 Tokiko Kanno
A1 Makoto Soejima
A1 Sayumi Baba
A1 Naoyuki Kamatani
A1 Masako Hara
YR 2007
UL http://www.jrheum.org/content/34/10/2050.abstract
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.