Uriuhara A, Morita S, Yamada K, Masugi J, Yamada H, Hasegawa Y, Yokono K, Kasuga M
Second Department of Internal Medicine, Kobe University School of Medicine.
Ryumachi. 1997 Jun;37(3):483-6.
A 37-year-old female, who had been suffering from progressive systemic sclerosis (PSS) with long-term renal failure, was diagnosed as MPO-ANCA positive PSS in active stage. She had systemic scleroderma, normotensive renal failure and pulmonary fibrosis. Respiratory management and steroid therapy under the control of CVVH were done for her treatment. She developed hemolytic anemia and thrombocytopenia during the treatment. She was diagnosed as microangiopathic hemolytic anemia, and was treated with plasma exchange treatment. About eight weeks after her admission, she developed status epileptics. Cerebral computed tomography and lumbar puncture did not reveal any abnormal sign. The case reported herein is a MPO-ANCA positive PSS with normotensive renal failure. Generally, PSS rarely reveals neurological sign. This case suggests possibility of association between MPO-ANCA and systemic vasculitis in PSS.
一名37岁女性，患有进行性系统性硬化症（PSS）并伴有长期肾衰竭，被诊断为活动期MPO - ANCA阳性PSS。她患有系统性硬皮病、血压正常的肾衰竭和肺纤维化。针对其治疗进行了呼吸管理以及在连续性静脉 - 静脉血液滤过（CVVH）控制下的类固醇治疗。治疗期间她出现了溶血性贫血和血小板减少症。她被诊断为微血管病性溶血性贫血，并接受了血浆置换治疗。入院约八周后，她出现癫痫持续状态。脑部计算机断层扫描和腰椎穿刺未发现任何异常迹象。本文报道的病例是一名血压正常的肾衰竭的MPO - ANCA阳性PSS。一般来说，PSS很少出现神经学体征。该病例提示了MPO - ANCA与PSS中系统性血管炎之间关联的可能性。
