Pulmonary hypertension in systemic lupus erythematosus: clinical association and survival in 18 patients

Abstract

Objective: To examine the clinical and serologic features in patients with systemic lupus erythematosus (SLE) with pulmonary hypertension (PHT).

Methods: Case records were reviewed for 419 Chinese patients with SLE from a single center in Hong Kong between January 1985 and June 1997. Patients with PHT were compared with SLE controls. The diagnosis of PHT was made when the pulmonary artery systolic pressure (PASP), measured by 2 dimensional echocardiogram and Doppler studies at rest, was >30 mm Hg, or at postmortem. Clinical features, serologic profile, and outcome of those with PHT were compared with those without PHT. Thirteen patients had repeat echocardiogram to determine subsequent PASP.

Results: Eighteen of 419 patients, all female, were identified as having PHT. The clinical and serologic features of patients with SLE with PHT were similar to those without PHT except for serositis and Raynaud's phenomenon, which occurred more frequently in patients with PHT (p = 0.01, p = 0.03, respectively). The age at diagnosis of PHT was 30.5+/-10.2 (mean +/- SD) years. The duration of followup from time of diagnosis of PHT was 23.6+/-20.1 months. PASP was 52+/-23.4 mm Hg. There were 4 deaths. Survival of PHT patients as a group was 40.2+/-5.18 (95% CI 30.1-50.4) months. Repeat echocardiogram in 13 patients revealed a mean reduction in PASP of 21.3% over a median interval of 43 (range 4-52) months.

Conclusion: Raynaud's phenomenon is associated in patients with SLE with PHT. The prognosis of these patients is variable, with the majority of patients having mild to moderate disease showing little change over 2 years. Nearly a quarter died and a few improved markedly.