Successful treatment of pulmonary hypertension preceding the diagnosis of systemic lupus erythematosus with corticosteroid and with plasma exchange: A case report

Abstract

A 28 year old woman was admitted to our hospital because of exertional dyspnea. She was diagnosed by cardiac catheterization as primary pulmonary hypertension (PPH). Some laboratory findings such as positive anti-nuclear antibody, leucopenia and hypergam-maglobulinemia suggested that she had connective tissue disease (CTD), but any diagnostic criteria for CTD were not fulfilled. On month after the diagnosis of PPH, she developed butterfly rash and high titer of anti-dsDNA antibody and fulfilled the criteria for systemic lupus erythematosus (SLE).
Corticosteroid therapy and plasma exchange decreased the pulmonary artery pressure as well as the disease activity. She was a rare case who developed pulmonary hypertension prior to the diagnosis of SLE. PPH may be successfully treated by these therapy if immunological disorders are involved in its pathogenesis.