What is the role of elastin in the pathogenesis of giant cell arteritis (GCA) (temporal arteritis)?

Updated: Sep 03, 2020
  • Author: Mythili Seetharaman, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

A cellular immune reaction to elastin has been implicated in the pathogenesis of GCA. [18] In support of the hypothesis that elastin is the inciting antigen, disease severity has been shown to correlate with the amount of elastic tissue within the vessels. [19] For example, intracranial arteries lack an internal elastic lamina, and GCA does not cause a widespread intracranial cerebral vasculitis.

This hypothesis also is supported by histopathologic findings of a disrupted, fragmented internal elastic lamina in affected vessels and the presence of characteristic giant cells, which may contain elastic fiber fragments, close to the internal elastic lamina. Along with elevated serum levels of neutrophil elastase, deposition of elastase along the damaged internal elastic lamina has been described.


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