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?
Subintimal fibromuscular thickening
Organization of microthrombi
|D.||Cystic medial necrosis|
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.