Eyeworld

54 | EYEWORLD | FALL 2025 C ORNEA References 1. Sjögren's Foundation. sjogrens. org/understanding-sjogrens/di- agnosis. Accessed July 1, 2025. 2. Shiboski CH, et al. 2016 Amer- ican College of Rheumatology/ European League Against Rheu- matism classification criteria for primary Sjögren's syndrome: a consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis. 2017;76:9–16. 3. Tauber J, et al. Lacripep for the treatment of primary Sjögren-as- sociated ocular surface disease: results of the first-in-human study. Cornea. 2023;42:847–857. coming to the doctor and saying, 'I'm super dry; something's wrong,'" she said. Often a patient first comes to a doctor's office with unexplained fatigue, not recognizing (or not having) signs of dryness. Ms. Church was hospitalized for a month with neurological issues (aseptic meningitis) at 40 years old. Nine months later at age 41, she was finally diagnosed with Sjögren's dis- ease. Neurological symptoms are common to Sjogren's patients, though aseptic meningitis is less common. About a year after her diagnosis, Ms. Church said she started feeling eye sensitivities and began using drops. Now, she has severe dry eye that has aggressively progressed despite treatment. Vatinee Y. Bunya, MD, MSCE, said that she uses the 2016 American College of Rheuma- tology-European League Against Rheumatism (ACR-EULAR) classification criteria to diagnose Sjögren's disease. "There are currently no diag- nostic criteria for Sjögren's disease, so clinicians are using these classification criteria to identify Sjögren's patients," she said, including the be- low ACR-EULAR criteria 2 : • Anti-SSA/Ro antibody positivity (3 points) • Positive lip biopsy (focal lymphocytic sialad- enitis with a focus score of ≥1 foci/4 mm 2 ) (3 points) • Abnormal ocular staining score of ≥5 (or a van Bijsterveld score ≥4) (1 point) • Schirmer test without anesthesia ≤5 mm/5 minutes (1 point) • Unstimulated salivary flow rate of ≤0.1 mL/minute (1 point) She said individuals who meet eligibility criteria for Sjögren's have a score of 4 or more. "Of note, the two ocular tests that are included in the criteria include an abnormal ocular stain- ing score or an abnormal Schirmer test without anesthesia," she said. "Therefore, it's important for ophthalmologists to perform these two tests in any dry eye patient who may have Sjögren's disease." Dr. Bunya also said that while there are newer diagnostic tests for dry eye disease, none have been shown to distinguish dry eye that is associated with Sjögren's. "Ophthalmologists and optometrists play a critical role in diagnos- ing Sjögren's patients early on in their disease, as many studies have shown that dry eye is one of the most common symptoms of Sjögren's disease," Dr. Bunya said. "I recommend that the ocular staining score and Schirmer test without anesthesia be performed on all new dry eye patients. Dry eye patients should be referred for systemic workups for Sjögren's disease if they meet any of the ocular criteria, if they have a positive review of systems for symptoms such as joint pain, dry mouth, or fatigue, or if they have dry eye disease that is refractory to treatment." Once a patient has met the eligibility cri- teria for Sjögren's, Dr. Akpek said the patient should have a battery of serology tests that include testing for Sjögren's or another underly- ing autoimmune disease. Dr. Akpek said the gold standard for diag- nosis is a minor salivary gland biopsy from the inner side of the lower lip, but this is invasive and can be difficult to perform accurately. An area of research for a different form of Sjögren's diagnosis is tear film cytokines and chemokines, Dr. Akpek said. She mentioned the research from Sharmila Masli, PhD, at Boston University, whose lab centers around tear film cytokines, chemokines, and different types of mucins to differentiate Sjögren's from non- Sjögren's dry eye. Available treatments and future hopes Dr. Bunya said she has a lower threshold for starting prescription dry eye treatments in patients with Sjögren's than non-Sjögren's dry eye patients, including topical cyclosporine and lifitegrast. "If a patient has concurrent continued from page 52 A patient with Sjögren's disease demonstrating severe lissamine green staining of the conjunctiva Source: Vatinee Y. Bunya, MD

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