Which pathologic changes are characteristic of scleroderma?

Updated: Nov 13, 2018
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Answer

The pathologic changes in systemic sclerosis encompass a spectrum reflecting variable stages of development and progression of the three major processes occurring in the affected tissues, as follows:

  • Severe tissue fibrosis with exaggerated deposition of collagen and other connective tissue components in the extracellular matrix

  • Chronic inflammation, occurring predominantly in the early stages of disease and characterized by infiltration with mononuclear cells, mostly of the macrophage and T-cell lineages

  • Microvascular disease, characterized by intimal proliferation, concentric subendothelial deposition of collagen and mucinous material, and narrowing and thrombosis of the vessel lumen


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