CASE REPORT Successful treatment with immunosuppression, anticoagulation and vasodilator therapy of pulmonary hypertension in SLE associated with secondary antiphospholipid syndrome L-S Tam and EK Li Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong Severe pulmonary hypertension (PHT) occurring in patients with systemic lupus erythematosus (SLE) is uncommon. Different modalities have been tried in the treatment for this condition but have not been effective because of progressive increase of pulmonary resistance over time. Our patient with SLE and PHT with antiphospholipid syndrome (APS), a condition which has previously never been described, responded rapidly to combination treatment with immuno- suppression, anticoagulation and vasodilator therapy. Keywords: pulmonary hypertension; SLE; antiphospholipid syndrome Introduction The association of pulmonary hypertension (PHT) and antiphospholipid antibodies (aPL) was ®rst described in 1983 when ®ve out of six patients with PHT and SLE were positive for the lupus anticoagulant. 1 Despite a prevalence of 68% of aPL in a group of SLE patients with PHT, antiphospholipid syndrome (APS) related features are distinctly uncommon. 2 The prevalence of PHT in SLE is estimated between 9± 14%, with most being mild, subclinical cases. 3 Nonetheless, mild cases of PHT have been shown to progress with increased pulmonary vascular resistance over time. 4 Severe, symptomatic PHT in SLE is rare and carries a grave prognosis because a fatal outcome can occur within months, usually from end-stage circulatory collapse in the majority of patients. 2 We describe a patient with SLE with severe PHT and secondary APS who responded favourably to prednisone, intravenous cyclophosphamide (IVCY), warfarin and diltiazam. Case history A 34-year-old woman with SLE ®rst presented to us in June 1997 because of progressive shortness of breath over the past 12 months with exercise tolerance reduced to one ¯ight of stairs. In 1982 she was diagnosed, at the age of 18 as having SLE with polyarthritis, malar rash and digital vasculitis and had been on prednisone 5 mg daily since. The patient denied symptoms such as chest pain, cough, or features suggestive of deep vein thrombosis, cerebral vascular accident or previous lung infections. Past health was unremarkable apart from a history of recurrent second trimester abortions with foetal death in-utero at approximately 16 weeks in 1989 and 1991. On both occasions, she had uncontrolled hypertension that required hospitalisations for two out of four weeks. On examination, the patient was not in respiratory distress. Her blood pressure was 130=80 mm Hg, the heart rate was 75 bpm, and the respiratory rate 14=min. The rest of the examination was essentially normal. The erythrocyte sedimentation rate (ESR) was 28 mm in 1 h, the haemoglobin concentration was 11.6 g=dl, the leukocyte count was 4100=ml 3 and the platelet count was 204000=ml 3 . Serum electrolytes, urea and creatinine Correspondence: Dr. Edmund K. Li, Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong. Received 15 April 1998; accepted 28 May 1998 Lupus (1998) 7, 495±497 ß 1998 Stockton Press. All rights reserved 0961-2033/98 $12.00 http://www.stockton-press.co.uk/lup