SUMMARYIn order to determine the prevalence and clinical significance of¯2-GPI-dependent anticardiolipin antibodies (¯2-GPI/aCL) in patients with systemic sclerosis (SSc), serum samples from 80 patients with SSc, 20 patients with systemic lupus erythematosus (SLE), and 120 healthy control subjects were examined by ELISA using purified¯2-GPI. IgG isotype¯2-GPI/aCL was present in eight of 80 patients with SSc (10%), and the presence of¯2-GPI/aCL IgG was significantly correlated with the presence of isolated pulmonary hypertension (PH). Furthermore, levels of¯2-GPI/aCL IgG were significantly correlated with levels of mean pulmonary arterial pressure. These data suggest that IgG isotype¯2-GPI/ aCL might be a serological indicator of the severity of PH in patients with SSc.