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


METHODS
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.


METHODS
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.


CONCLUSIONS
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.
Beckh
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Medizinische Klinik 3, Klinikum Nürnberg.
Iversen
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Loose
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Bölcskei
P L
PL
D000208:Acute Disease; D001999:Bronchoscopy; D003131:Combined Modality Therapy; D015150:Echocardiography, Doppler; D004562:Electrocardiography; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology / Q000628:therapy; D008168:Lung / Q000000981:diagnostic imaging; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D008875:Middle Aged; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000473:pathology; D011859:Radiography; D011877:Radionuclide Imaging
Schwere pulmonale Hypertonie bei systemischem Lupus erythematodes. Erfolgreiche Therapie bei einer ungewöhnlichen Manifestation.