Presentation: Characteristics of Patients with Early Systemic Sclerosis and Severe Gastrointestinal Tract Involvement (2007)

18 Characteristics of Patients with Early Systemic Sclerosis and Severe Gastrointestinal Tract Involvement

Purpose: Systemic sclerosis (SSc) is a multisystem connective tissue disorder of unknown origin that is characterized by tissue fibrosis, endothelial injury, and autoimmunity. Approximately 90% of patients with SSc develop gastrointestinal tract (GIT) manifestations related to dysmotility. Although the mechanisms of impaired GIT motility are not fully understood, it has been hypothesized that alteration in neural function in the GIT is present along with fibrosis in the early stage, leading to smooth muscle atrophy and degeneration that cause severe motility disturbance. Severe manifestations such as malabsorption, repeated episodes of pseudo-obstruction, severe constipation, rectal prolapse, megacolon, and multiple diverticula are rarely observed in both early and late stages. However, we have sometimes encountered patients who developed severe GIT involvement as a main symptom of early SSc, and in some cases the symptom preceded the skin manifestation. In the present study, we examined the clinical features of patients who developed severe GIT involvement in early SSc, and we then determined predictive indicators for severe GIT involvement.
Methods: Three hundred two consecutive Japanese patients with SSc were investigated. The 14 patients with severe GIT involvement (malabsorption syndrome and/or pseudo-obstruction) within 2 years of onset of SSc were referred to as group 1; 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, enzyme-linked immunosorbent assay, and immunoprecipitation.
Results: We found significant differences in clinical features among the three 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) and than in 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) than in 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. In the Japanese individuals, these patients never developed severe ILD, even though they were classified as having diffuse cutaneous SSc.

 Y. Kawaguchi, None; E. Nishimagi, None; A. Tochimoto, None; T. Satoh, None; M. Kuwana, None; T. Sugiura, None; M. Soejima, None; S. Baba, None; N. Kamatani, None; M. Hara, None.