A Case of Scleroderma Renal Crisis / 대한신장학회잡지

ABSTRACT

Systemic sclerosis is a generalized connective tissue disorder. It is characterized by fibrosis, degenerative changes and vascular lesions affecting the skin, joints, skeletal muscles and multiple internal organs. Clinical renal disease in systemic sclerosis ranges from scleroderma renal crisis to more subtle abnormalities. Scleroderma renal crisis is characterized by malignant hypertension and rapidly progressive renal failure. It can be successfully treated with angiotensin converting enzyme inhibitors, but remains the most feared complication of systemic sclerosis. We have experienced a 57 year old female patient who visited emergency room with a sudden onset of mental deterioration, azotemia and oliguria. She was under symptomatic treatment for Raynaud's phenomenon for 2 years. Renal biopsy of the patient showed onion-skin configuration in the interlobular arteries which is characteristic of scleroderma renal crisis. She proceded to maintenance hemodialysis despite a angiotensin converting enzyme inhibitor treatment. We report a case of SRC with a brief review of literature.