Ocular microvascular damage in autoimmune rheumatic diseases: The pathophysiological role of the immune system

Elvis Hysa, Carlo Alberto Cutolo, Emanuele Gotelli, Sabrina Paolino, Marco Amedeo Cimmino, Greta Pacini, Carmen Pizzorni, Alberto Sulli, Vanessa Smith, Maurizio Cutolo

Research output: Contribution to journalReview articlepeer-review


Pathological eye involvement represents a quite common finding in a broad spectrum of autoimmune rheumatic diseases (ARDs). Ocular signs, often occur as early manifestations in ARDs, ranging from symptoms related to the mild dry eye disease to sight-threatening pathologies, linked to the immune response against retinal and choroidal vessels. Retinovascular damage driven by markedly inflammatory reactivity need a prompt diagnosis and treatment. Immune-complexes formation, complement activation and antibody-mediated endothelial damage seem to play a key role, particularly, in microvascular damage and ocular symptoms, occurring in systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and Sjögren's syndrome (SS). Conversely, early alterations of retinal and choroidal vessels in the asymptomatic patient, often detectable coincidentally, might be indicators of widespread vascular injury in other connective tissue diseases. Particularly, endothelin-induced hypoperfusion and pathological peri-choroidal extracellular matrix deposition, might be responsible for the micro-architectural alterations and loss of capillaries detected in systemic sclerosis (SSc). Instead, interferon alpha-mediated microvascular rarefaction, combined with endothelial lesions caused by specific autoantibodies and immune-complexes, appear to play a significant role in retinal vasculopathy associated to inflammatory idiopathic myopathies (IIM). The immuno-pathophysiological mechanisms of ocular microcirculatory damage associated with the major ARDs will be discussed under the light of the most recent achievements.

Original languageEnglish
Article number102796
JournalAutoimmunity Reviews
Issue number5
Publication statusPublished - May 2021


  • Inflammatory idiopathic myopathies
  • Ocular microcirculation
  • Rheumatoid arthritis
  • Sjögren's syndrome
  • Systemic lupus erythematosus
  • Systemic sclerosis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology


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