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Autoimmune uveitis: clinical, pathogenetic, and therapeutic features

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Abstract

Autoimmune uveitis (AU), an inflammatory non-infectious process of the vascular layer of the eye, can lead to visual impairment and, in the absence of a timely diagnosis and suitable therapy, can even result in total blindness. The majority of AU cases are idiopathic, whereas fewer than 20 % are associated with systemic diseases. The clinical severity of AU depends on whether the anterior, intermediate, or posterior part of the uvea is involved and may range from almost asymptomatic to rapidly sight-threatening forms. Race, genetic background, and environmental factors can also influence the clinical picture. The pathogenetic mechanism of AU is still poorly defined, given its remarkable heterogeneity and the many discrepancies between experimental and human uveitis. Even so, the onset of AU is thought to be related to an aberrant T cell-mediated immune response, triggered by inflammation and directed against retinal or cross-reactive antigens. B cells may also play a role in uveal antigen presentation and in the subsequent activation of T cells. The management of AU remains a challenge for clinicians, especially because of the paucity of randomized clinical trials that have systematically evaluated the effectiveness of different drugs. In addition to topical treatment, several different therapeutic options are available, although a standardized regimen is thus far lacking. Current guidelines recommend corticosteroids as the first-line therapy for patients with active AU. Immunosuppressive drugs may be subsequently required to treat steroid-resistant AU and for steroid-sparing purposes. The recent introduction of biological agents, such as those targeting tumor necrosis factor-α, is expected to remarkably increase the percentages of responders and to prevent irreversible sight impairment. This paper reviews the clinical features of AU and its crucial pathogenetic targets in relation to the current therapeutic perspectives. Also, the largest clinical trials conducted in the last 12 years for the treatment of AU are summarized and critically discussed.

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Acknowledgments

The authors are grateful to Mrs. Laura Di Pietro for her excellent secretarial assistance. This work was supported by grants from the University of Bari and the Italian Association for Cancer Research (AIRC), Milano.

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All authors declare that they have no financial relationships with any organizations that might have an interest in the submitted work.

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  1. Internal Medicine Unit, Department of Biomedical Sciences and Human Oncology (DIMO), University of Bari Medical School, Piazza G. Cesare 11, 70124, Bari, Italy

    Marcella Prete, Maria Celeste Fatone & Vito Racanelli

  2. Department of Otorhinolaryngology and Ophthalmology, University of Bari Medical School, Bari, Italy

    Rosanna Dammacco

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  1. Marcella Prete
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Correspondence to Marcella Prete.

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Prete, M., Dammacco, R., Fatone, M.C. et al. Autoimmune uveitis: clinical, pathogenetic, and therapeutic features. Clin Exp Med 16, 125–136 (2016). https://doi.org/10.1007/s10238-015-0345-6

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