Sjögren's syndrome (SS) is a common autoimmune disease affecting about four million Americans. Although approximately 1 in 10 patients with clinically-significant aqueous deficient dry eye has underlying SS, widespread underappreciation of SS leads to significant underdiagnosis, delays in diagnosis, and consequent morbidity and mortality. The purpose of this article is to illustrate that in addition to dry eye, SS can cause serious, vision-threatening extraglandular ocular manifestations.
We conducted a narrative review of studies that have examined the dry eye and extraglandular ocular complications of SS.
SS-related dry eye is a progressive condition with major negative impact on the quality-of-life of afflicted patients, not only due to symptoms of ocular discomfort but also visual dysfunction. In addition, SS can lead to corneal melt/perforation, uveitis, scleritis, retinal vasculitis, and optic neuritis. A major problem with currently-available SS disease activity measurement instruments is the lack of domains evaluating dry eye-related visual dysfunction. For example, one of the most commonly-used instruments for assessing patient symptoms in SS (the EULAR Sjögren's Syndrome Patient Reported Index [ESSPRI]) only includes one item (out of three) that addresses the severity of overall dryness, without mention of dry eye symptoms or vision-related quality-of-life. Similarly, no extraglandular ocular complications are included in currently-available SS disease activity instruments (e.g., the EULAR Sjögren's Syndrome Disease Activity Index [ESSDAI]).
There needs to be a paradigm shift in which eye care providers and rheumatologists become more familiar with various SS-related extraglandular ocular manifestations. Existing disease activity measurement instruments must incorporate dry eye symptoms, particularly those related to visual dysfunction. An evidence-based screening algorithm for determining which patients with dry eye should be tested for underlying SS may be particularly helpful in preventing delays in diagnosis.
*The Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, MD;
†Scheie Eye Institute, University of Pennsylvania, Philadelphia, PA; and
‡Department of Health Services, Policy, and Practice, Center for Evidence Synthesis in Health, Brown University School of Public Health, Providence, RI.
Correspondence: Esen K. Akpek, MD, Ocular Surface Disease and Dry Eye Clinic, The Wilmer Eye Institute, Johns Hopkins University School of Medicine, 600 North Wolfe St, Maumenee #317, Baltimore, MD 21287-9238 (e-mail: firstname.lastname@example.org).
The authors have no funding to disclose.
E. K. Akpek is an uncompensated member of the Board of Directors of the Sjögren's Syndrome Foundation, Bethesda, MD. The other authors have no conflicts of interest to disclose.
Received October 31, 2018
Received in revised form November 30, 2018
Accepted December 02, 2018