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Pathological change of SSc
The early pathological changes of SSc include swelling
and homogenization of collagen fibers, and perivascular and collagen fiber
infiltration by lymphocytes dominantly, followed by increase in dense collagen
fibers in reticular dermis, thinning of the epidermis, disappearance of Rete
ridges, atrophy of sebaceous glands and decrease in sweat glands. Besides, thickening
of the vascular wall and stenosis or even occlusion of the lumen can also be seen.
The damage to internal organs is mainly caused by the hyperplasia and sclerosis
of the mesenchyma and the collagen fibers in vascular wall. Esophageal
involvements include atrophy of the smooth muscle, while fibrosis happens in
submucosa and lamina propria, and mucosa shows varying degrees of thinning and
erosion. Fibrosis change also shows in the nutrient vessels of esophagus. Barrett
metaplasia can happen in 1/3 of the SSc patients. As for the lungs, Pulmonary
fibrosis and pulmonary vascular lesions often coexist. Pulmonary fibrosis is
often severer in Scl-70 positive diffuse sclerosis patients, and What can be
seen in patients with pulmonary hypertension are pulmonary interstitial and
peribronchial fibrosis, and medial hyperplasia of pulmonary arterioles. Autopsy
showed that hyperplasia of intima in small pulmonary arteries occurs in about
29%-47% of the patients.