Pulmonary hypertension and systemic lupus erythematosus.

Various pleuropulmonary manifestations are reported to occur in the course of systemic lupus erythematosus (SLE). Among these, pulmonary hypertension is one of the most uncommonly observed. The natural course of this involvement can be independent of the primary disease, and resembles primitive pulmonary hypertension. Various pathogenetic mechanisms might be responsible for this picture. In the case here reported, the pathological data were strongly suggestive of pulmonary vasculitis, emphasizing that SLE, like other connective tissue diseases, can be responsible for fatal pulmonary hypertension.