Scleroderma Renal Crisis in a Normotensive Patient

INTRODUCTION S ystemic sclerosis (scleroderma) is an autoimmune disorder of unclear etiology that leads to fibrosis and vascular injury of the skin and multiple organs, including the lungs, heart, gastrointestinal tract, kidneys, and the musculoskeletal system. It is classified as either diffuse or limited cutaneous based on the extent of skin thickening. In the diffuse form of systemic sclerosis, skin thickening is extensive and usually occurs within 1 year, compared with the limited cutaneous form, in which skin thickening is often confined to the face, fingers, and hands. Diffuse systemic sclerosis is also characterized by Raynaud’s phenomenon and significant visceral involvement. Renal complications are common in patients with systemic sclerosis. The most serious renal manifestation is scleroderma renal crisis, which occurs in up to 10% of patients with diffuse cutaneous systemic sclerosis, and only rarely (1%–2%) in limited cutaneous systemic sclerosis. It is a relatively early complication of systemic sclerosis, typically occurring within 5 years of diagnosis, and may be the initial presentation of systemic sclerosis. Scleroderma renal crisis is usually characterized by abrupt onset of marked hypertension, rapidly progressive renal failure due to thrombotic microangiopathy (TMA), vasospasm, and tissue ischemia. However, up to 10% of patients with scleroderma renal crisis are normotensive, although their blood pressure may be increased from baseline values.