Successful treatment of severe pulmonary hypertension in systemic lupus erythematodes with an unusual presentation

Objective and clinical findings: A 48-year-old woman was hospitalized becau se of haemoptysis. Until shortly before admission she had been on phenproco umon after pulmonary embolism sustained 18 months previously. Six months be fore admission systemic lupus erythematodes (SLE) had been diagnosed and tr eatment with cortisone initiated. Physical examination revealed jugular ven ous congestion, tachycardia, dyspnoea on even minimal physical activity and pretibial edema. Investigations: Lung scintigraphy showed a perfusion deficiency in the righ t lung, unchanged since a test 18 month before. Doppler echocardiography re corded an estimated pulmonary artery systolic pressure of 110 mm Hg. Angiog raphy showed a fully patent superior vena cava and nearly complete occlusio n of the main right pulmonary artery by a thrombus. Diagnosis, treatment and course: The haemoptyses ceased after 5 days of tre atment with methylprednisolone, 130 mg daily for 5 days, reduced after 5 da ys to 80 mg i.v. every other day, plus cyclophosphamide, 50 mg daily by mou th. The pulmonary hypertension remained unchanged so that pulmonary thrombe ndarterectomy was indicated. Surgery revealed extensive mediastinal fibrosi s and almost complete occlusion of the thick-walled right pulmonary artery by thrombus adherent to the wall. Histology showed vasculitis of the pulmon ary 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 immunosuppr essive treatment. The patient has remained free of symptoms and is able to be physically active. Conclusion: Pulmonary hypertension is a serious complication of SLE. Echoca rdiography is recommended for both the original diagnosis and serial follow -up, complemented by other imaging methods if indicated.