The Sclera pp 33-58 | Cite as

Immunological Considerations of the Sclera

  • C. Stephen Foster
  • Maite Sainz de la Maza


Scleritis remains an enigmatic disease. Attempts to demonstrate a specific antigen associated with scleral vessel and tissue damage, or to reproduce suggested underlying pathogenic mechanisms in experimental animal models, have not yielded consistent abnormalities. The prevailing consensual view, however, based on the available evidence, is that disordered immune responses leading to vessel and tissue damage are central to the pathogenesis of scleritis. The histopathological and immunofluorescence detection of immune complex inflammatory microangiopathy in affected scleral biopsy specimens,1 the frequent association of scleritis with systemic autoimmune diseases associated with circulating immune complexes (rheumatoid arthritis, systemic lupus erythematosus, or polyarteritis nodosa),1–3 the favorable response of scleritis to immunosuppressive agents,2,3 and the absence of vascular perfusion in severe types of scleritis, as determined by anterior segment fluorescein angiography,4 all suggest that scleritis represents an autoimmune process mediated by a localized immune complex inflammatory microangiopathy or type III hypersensitivity reaction. The histopathological finding of a chronic granulomatous inflammation characterized predominantly by macrophages and T lymphocytes in scleritis biopsy specimens suggests that a cellular immunity dysfunction or type IV hypersensitivity reaction also may play a role.1


Rheumatoid Arthritis Mast Cell Major Histocompatibility Complex Immune Complex Major Histocompatibility Complex Gene 
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Copyright information

© Springer Science+Business Media New York 1994

Authors and Affiliations

  • C. Stephen Foster
    • 1
  • Maite Sainz de la Maza
    • 2
  1. 1.Harvard Medical School, Immunology and Uveitis ServiceMassachusetts Eye and Ear InfirmaryBostonUSA
  2. 2.Central University of BarcelonaBarcelonaSpain

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