["Pulmonary hypertension" of the gastrointestinal tract in patients with systemic sclerosis: thoughts on a not (yet) existing disease entity].

Vascular changes are often found in numerous organs of patients with systemic sclerosis (SSc). It has been shown that, e.g. Raynaud's syndrome, ulcers of the digits and pulmonary arterial hypertension have similar patterns of endothelial dysfunction. There is evidence of gastrointestinal complications in almost all patients with SSc, most often caused by abnormal motility and fibrosis. Immunohistological studies have shown that gastric and intestinal mucosae have not only activation of classical activation markers and adhesion molecules, but also of growth factors demonstrated in pulmonary hypertension as well as overexpression of endothelin-1. The possible common pathogenesis of he vasculopathies in the lung and gastrointestinal tract suggests that explorative studies should be undertaken in which endothelin-receptor antagonists are administered to patients with abnormal gastrointestinal motility and SSc.