MCQ – 87798

A 45-year-old woman complains that her fingers feel stiff. On physical examination the skin of the patient’s face appears tense and radial furrows are evident around the mouth. Laboratory studies establish a diagnosis of scleroderma. Several years later, the patient subsequently develops renal insufficiency. A renal biopsy is shown in the image. Which of the following

best describes the pathogenesis of renal vascular involvement in this patient with progressive systemic sclerosis?

Correct Answer � B Explanation

Answer : B Subintimal fibromuscular thickening

Scleroderma is characterized by vasculopathy and excessive collagen deposition in the skin and a variety of internal organs.

• The disease occurs four times as often in women as in men, mostly in persons between 25 and 50 years of age.

• Lesions in the arteries, arterioles, and capillaries are typical, and in some cases may be the first demonstrable pathological finding in the disease.

• The kidneys are involved in more than half of patients with scleroderma. They show marked vascular changes, often with focal hemorrhage and cortical infarcts.

• Among the most severely affected vessels are the interlobular arteries and afferent arterioles. 

• Early fibromuscular thickening of the subintima causes luminal narrowing, followed by fibrosis. 

• The other pathologic lesions may occur, but they are not the principal cause of vasculopathy in patients with scleroderma.