A Case of Pulmonary Hypertension Associated with Systemic Lupus Erythematosus without Anti-ribonucleoprotein Antibodies

We encountered a 32-year-old woman presenting with high-grade fever and swollen lymph nodes. Echocardiography showed a pericardial effusion and progressive pulmonary hypertension (PH). Although the criteria for systemic lupus erythematosus (SLE) were met, typical laboratory values and symptoms of SLE were not present. Anti-ribonucleaoprotein antibodies were negative. Computed tomography (CT) showed a dilatation of peripheral arteries and veins and a thickening of the interlobular septa. The patient was given anticoagulants, but these were not effective. Steroids, however, improved her condition dramatically. The clinical course of this patient indicates the importance of steroid therapy for PH even when the patient does not show the typical clinical features of SLE.