Pulmonary hypertension in systemic lupus erythematosus: evaluation of clinical characteristics and response to immunosuppressive treatment.

To clarify the clinical features of systemic lupus erythematosus (SLE) complicated with pulmonary hypertension (PH) and to evaluate the efficacy of immunosuppressive treatment.Case records were reviewed for 194 patients with SLE who were admitted to Aoyama Hospital of Tokyo Women's Medical University between 1992 and 1999. There were 12 patients with PH [8 SLE and 4 SLE + systemic sclerosis (SSc) overlap syndrome]. These patients were compared with 59 age and sex matched patients with SLE for clinical characteristics and laboratory findings. The efficacy of treatments for PH was also evaluated.In our cohort of 194 patients with SLE, 6.2% had PH. The plasma thrombin-antithrombin III complex and plasma D-dimer levels were significantly higher in patients with PH compared with those without PH. Eight patients with PH (4 SLE and 4 SLE + SSc) were treated with corticosteroids (CS) +/- cyclophosphamide (CYC). Right ventricular systolic pressure (RVSP) was improved in 7 of 8 patients. In 6 of 7 responders to the therapy, the treatment was started as soon as they were diagnosed with PH. PH relapsed in 2 patients treated with oral CS +/- CS pulse therapy, but their RVSP was decreased again by immunosuppressive treatment.CS +/- CYC was effective for PH associated with SLE. Immunosuppressive treatment should be performed during the early stage of PH to improve prognosis.