I report the case of a woman with systemic lupus erythematosus who had pulmonary hypertension unassociated with chronic interstitial lung disease or pulmonary emboli. She had started taking the contraceptive pill seven months previously.
PIP: This is a case report of pulmonary hypertension in a woman with systemic lupus erythematosus who had taken an oral contraceptive. She was 16 yr old when diagnosed with SLE in July 1984, based on many clinical features and high DNA antibodies, RNP antibodies and CPK, and low complement. She improved slowly with prenisolone. She remained in remission for 7 months except for mild flare-ups involving synovitis, pleuritic chest pain and Raynaud's phenomenon. She began taking oral contraceptives 5 months later (30 mcg ethinyl estradiol and 150 mcg levonorgestrel). 7 months later she was readmitted with the same severe clinical and laboratory findings, but in addition exertional dyspnea. Pulmonary hypertension was evident, by x-ray, EKG, echocardiogram and right heart catheterization. Lupus anticoagulant and anticardiolipin antibodies were negative. She was treated with predniosolone, warfarin and nifedipine and remained stable for 6 months. She died of cardiac arrest after emergency surgery for ovarian cyst, complicated by shock and siezures. The author discussed the relationship between pulmonary hypertension and both oral contraception and SLE, since it is rare in either of these situations.