Characteristics of patients with early systemic sclerosis and severe gastrointestinal tract involvement.
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.


CONCLUSIONS
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.
Nishimagi
Emi
E
Institute of Rheumatology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan.
Tochimoto
Akiko
A
Kawaguchi
Yasushi
Y
Satoh
Takashi
T
Kuwana
Masataka
M
Takagi
Kae
K
Ichida
Hisae
H
Kanno
Tokiko
T
Soejima
Makoto
M
Baba
Sayumi
S
Kamatani
Naoyuki
N
Hara
Masako
M
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000974:Antibodies, Antinuclear / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology; D005260:Female; D041981:Gastrointestinal Tract / Q000473:pathology*; D006801:Humans; D007564:Japan; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000276:immunology / Q000473:pathology* / Q000503:physiopathology
D000974:Antibodies, Antinuclear; D001323:Autoantibodies