硬皮病肾脏损伤

摘要

A 49-year-old man presented with leg edema,malignant hypertension and skin thicking restricted to distal upper limbs and face.Urinalysis revealed proteinuria and microscopic hematuria.Blood tests demonstrated elevated serum creatinine,microangiopathic hemolytic anemia,thrombocytopenia,positive antinuclear antibody and hypocomplementaemia.Renal biopsy showed a typical histologic picture of thrombotic microangiopathy.Finally,the patient was diagnosed as scleroderma renal crisis.%中年男性,临床表现为多系统损害,肾脏损伤表现恶性高血压、快速进展的肾功能损害,中等量蛋白尿及镜下血尿;皮肤损害表现为手指僵硬,双手、前臂、面部皮肤增厚.实验室检查示溶血性贫血,血小板下降,抗核抗体阳性,补体C3水平下降.肾活检病理表现为典型的血栓性微血管病改变.患者最终诊断为硬皮病肾危象.