AUTOIMMUNE ASPECTS OF PRIMARY PULMONARY-HYPERTENSION

Although primary pulmonary hypertension (PPH) is considered to be an i diopathic condition, it has been postulated that autoimmunity may play a role in the pathogenesis of the disease. This argument has been bas ed on frequent coexisting clinical and serological rheumatic findings. Moreover, approximately in a third of the patients with PPH, and anti nuclear factor can be detected. Pulmonary hypertension may appear as a secondary complication to various autoimmune conditions. In light of these findings we examined sera derived from 40 patients diagnosed as having PPH for the presence of 18 different autoantibodies by the ELIS A and immunofluorescent techniques. Of the 40 patients, 62.4% had circ ulating autoantibodies and 47.5% presented with multiantibody response s. Autoantibodies most commonly observed were antinuclear (42.5%), ant i-ssDNA (25%) and antithyroglobulin (30%) antibodies. These results ma y imply that in a subgroup of patients with PPH the disease may be asc ribed to an immune dysregulation or alternatively that autoantibodies accompany the disease progression as an epiphenomenon.