Systemic Complications Are Common in Sjögren Syndrome

Christopher J. Rapuano, MD


June 02, 2015

Ocular and Systemic Morbidity in a Longitudinal Cohort of Sjögren's Syndrome

Akpek EK, Mathews P, Hahn S, et al
Ophthalmology. 2015;122:56-61

Study Summary

Consecutive patients were evaluated by a rheumatologist and an ophthalmologist at a Sjögren syndrome center between January 2007 and May 2011. Patients with primary Sjögren syndrome were included based on the American-European Consensus Group's 2002 revised criteria. Ocular signs and symptoms, along with systemic diagnoses, were evaluated.

Of 163 patients included with definitively diagnosed Sjögren syndrome, 91% were female with a mean age of 51 years at the time of diagnosis, and 98% had dry eye symptoms based on the Ocular Surface Disease Index questionnaire. The mean duration of dry eye symptoms prior to presentation was 10.4 years (median, 7.9 years). At presentation, only 63% of patients had a diagnosis of Sjögren syndrome, and 25% had ocular findings. Another 12% developed new ocular findings during the follow-up period (mean, 3 years). Vision-threatening complications occurred in 13% of patients, more than half of whom (55%) did not have an established diagnosis of Sjögren syndrome at the time of presentation. In fact, none of the five patients with a sterile corneal melt at presentation had a history of Sjögren syndrome.

In addition, 55% of patients had a systemic autoimmune, inflammatory, or lymphoproliferative disorder. Five patients (3%) had lymphoma. Of the patients with vision-threatening ocular disease, 64% had systemic abnormalities, whereas only 38% of the patients without vision-threatening ocular disease had such abnormalities. Patients with vision-threatening ocular disease were 3.9 times more likely to have systemic manifestations of primary Sjögren syndrome than those without.


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