PULMONARY-HYPERTENSION ASSOCIATED WITH SYSTEMIC LUPUS-ERYTHEMATOSUS -PREDOMINANTLY THROMBOTIC ARTERIOPATHY ACCOMPANIED BY PLEXIFORM LESIONS

We report an autopsy case of pulmonary hypertension associated with sy stemic lupus erythematosus in a 48-year-old woman. After 8-year follow -up under a definite diagnosis of systemic lupus erythematosus, she ex perienced gradually developing exertional dyspnea with palpitation. He r chest x-ray showed clear lung fields with marked cardiac enlargement . A right cardiac catheterization revealed a pulmonary arterial pressu re of 74/30 mm Hg (mean: 47). She was treated with repeated plasmapher esis, oral corticosteroid, and immunosuppressant without improvement, and she died suddenly, 23 days after admission. Pathological examinati on revealed that small pulmonary arteries and arterioles were diffusel y involved by florid thrombotic lesions, which were characterized by i ntimal eccentric fibrous thickening, luminal occlusion with recanaliza tion, and occasional fresh thrombi. In addition, some arteries showed plexiform lesions coexistent with intimal thrombotic lesions. Concentr ic laminar intimal fibrosis was not seen. No significant parenchymal c hange was seen. Our study not only adds a rare case of thrombotic pulm onary hypertension associated with systemic lupus erythematosus, but a lso suggests that plexiform lesions can occur in association with thro mbotic arteriopathy.