[Kidneys in diffuse systemic sclerosis]

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.