Dębowska Małgorzata, Staszków Monika
Katedra i Klinika Nefrologii, Dializoterapii i Chorób Wewnętrznych WUM, Warszawa, Polska,
Katedra i Klinika Nefrologii, Dializoterapii i Chorób Wewnętrznych WUM, Warszawa, Polska.
Wiad Lek. 2016;69(5):717-720.
Systemic scleroderma is a chronic, autoimmune disease of the connective tissue that involves skin, subcutaneous tissue, muscles and joints, as well as the internal organs: kidneys, lungs, heart. Depending on the extent it can occur as limited or diffuse clinical variant. In 60-80 % of patients with diffuse scleroderma, autopsy studies have shown pathologic changes in the kidneys. About half of the patients with renal involvement the clinical manifestation is limited to a moderate increase in serum creatinine, mild proteinuria, and moderate hypertension. The most serious complication remains sclerodermal renal crisis. It develops in 5-20 % of patients and is characterized by severe hypertension, acute kidney injury with oliguria, proteinuria and erythrocyturia, and microangiopathic hemolytic anemia with thrombocytopenia. In this article pathogenesis, risk factors, symptoms and treatment of scleroderma renal crisis have been reviewed.
系统性硬化症是一种慢性自身免疫性结缔组织疾病,累及皮肤、皮下组织、肌肉和关节,以及内脏器官:肾脏、肺、心脏。根据累及范围,可表现为局限性或弥漫性临床变体。在60%-80%的弥漫性硬化症患者中,尸检研究显示肾脏有病理改变。约一半有肾脏受累的患者,临床表现仅限于血清肌酐中度升高、轻度蛋白尿和中度高血压。最严重的并发症仍是硬皮病肾危象。它发生在5%-20%的患者中,其特征为严重高血压、伴有少尿的急性肾损伤、蛋白尿和红细胞尿,以及伴有血小板减少的微血管病性溶血性贫血。本文对硬皮病肾危象的发病机制、危险因素、症状及治疗进行了综述。