Yokoi T, Tomita Y, Fukaya M, Ichihara S, Kakudo K, Takahashi Y
Department of Pathology, Wakayama Medical College, Wakayama City, Japan.
Arch Pathol Lab Med. 1998 May;122(5):467-70.
We report an autopsy case of pulmonary hypertension associated with systemic lupus erythematosus in a 48-year-old woman. After 8-year follow-up under a definite diagnosis of systemic lupus erythematosus, she experienced gradually developing exertional dyspnea with palpitation. Her chest x-ray showed clear lung fields with marked cardiac enlargement. A right cardiac catheterization revealed a pulmonary arterial pressure of 74/30 mm Hg (mean: 47). She was treated with repeated plasmapheresis, oral corticosteroid, and immunosuppressant without improvement, and she died suddenly, 23 days after admission. Pathological examination revealed that small pulmonary arteries and arterioles were diffusely involved by florid thrombotic lesions, which were characterized by intimal eccentric fibrous thickening, luminal occlusion with recanalization, and occasional fresh thrombi. In addition, some arteries showed plexiform lesions coexistent with intimal thrombotic lesions. Concentric laminar intimal fibrosis was not seen. No significant parenchymal change was seen. Our study not only adds a rare case of thrombotic pulmonary hypertension associated with systemic lupus erythematosus, but also suggests that plexiform lesions can occur in association with thrombotic arteriopathy.
我们报告一例48岁女性系统性红斑狼疮相关肺动脉高压的尸检病例。在明确诊断为系统性红斑狼疮后经过8年随访，她逐渐出现劳力性呼吸困难伴心悸。胸部X线显示肺野清晰但心脏明显增大。右心导管检查显示肺动脉压为74/30 mmHg（平均：47）。她接受了反复血浆置换、口服糖皮质激素和免疫抑制剂治疗，但病情无改善，入院23天后突然死亡。病理检查显示，小肺动脉和小动脉广泛受累于显著的血栓性病变，其特征为内膜偏心性纤维增厚、管腔闭塞伴再通以及偶尔出现新鲜血栓。此外，一些动脉显示丛状病变与内膜血栓性病变并存。未见同心层状内膜纤维化。未见明显的实质改变。我们的研究不仅增加了一例系统性红斑狼疮相关血栓性肺动脉高压的罕见病例，还提示丛状病变可与血栓性动脉病并存。
