Korean J Nephrol.  1998 Jul;17(4):629-633.

A Case of Scleroderma Renal Crisis

• Ahn SH
• Lee JH
• Lee MS
• Song JH

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.

Keyword

Scleroderma renal crisis; Hypertension

MeSH Terms

Angiotensin-Converting Enzyme Inhibitors
Arteries
Azotemia
Biopsy
Connective Tissue
Emergency Service, Hospital
Female
Fibrosis
Humans
Hypertension
Hypertension, Malignant
Joints
Middle Aged
Muscle, Skeletal
Oliguria
Peptidyl-Dipeptidase A
Renal Dialysis
Renal Insufficiency
Scleroderma, Systemic
Skin
Angiotensin-Converting Enzyme Inhibitors
Peptidyl-Dipeptidase A