[Severe pulmonary hypertension in systemic lupus erythematosus. The successful therapy of an unusual manifestation].

UNLABELLED: OBJECTIVE AND CLINICAL FINDINGS: A 48-year-old woman was hospitalized because of haemoptysis. Until shortly before admission she had been on phenprocoumon after pulmonary embolism sustained 18 months previously. Six months before admission systemic lupus erythematodes (SLE) had been diagnosed and treatment with cortisone initiated. Physical examination revealed jugular venous congestion, tachycardia, dyspnoea on even minimal physical activity and pretibial oedema.

INVESTIGATIONS: Lung scintigraphy showed a perfusion deficiency in the right lung, unchanged since a test 18 month before. Doppler echocardiography recorded an estimated pulmonary artery systolic pressure of 110 mm Hg. Angiography showed a fully patent superior vena cava and nearly complete occlusion of the main right pulmonary artery by a thrombus.

DIAGNOSIS, TREATMENT AND COURSE: The haemoptyses ceased after 5 days of treatment with methylprednisolone, 130 mg daily for 5 days, reduced after 5 days to 80 mg i.v. every other day, plus cyclophosphamide, 50 mg daily by mouth. The pulmonary hypertension remained unchanged so that pulmonary thrombendarterectomy was indicated. Surgery revealed extensive mediastinal fibrosis and almost complete occlusion of the thick-walled right pulmonary artery by thrombus adherent to the wall. Histology showed vasculitis of the pulmonary arterial intima and of the small pulmonary vessels. After thrombectomy the pulmonary arterial systolic pressure fell to an remained at below 40 mm Hg. Phenprocoumon was continued (at an INR of 2.5-3.5) as was immunosuppressive treatment. The patient has remained free of symptoms and is able to be physically active.

CONCLUSION: Pulmonary hypertension is a serious complication of SLE. Echocardiography is recommended for both the original diagnosis and serial follow-up, complemented by other imaging methods if indicated.