ESSDAI in Practice and Missed Sjögren’s Diagnoses

New targeted therapies for Sjögren’s disease will reach patients only if clinicians can document systemic activity and recognize the many patients who go undiagnosed.

In this Special Report series, Nehad Soloman, MD, FACR, of Arizona Arthritis and Rheumatology Associates, spoke with Teja Kapoor, MD, director of the Columbia Sjögren’s Center, about practical barriers to identifying candidates.

Payers often anchor coverage to trial entry criteria, and the NEPTUNUS trials required an ESSDAI¹ score above 5. The index demands formal training, including tape measurement of glands rather than ultrasound and careful grading of pulmonary and neurologic features.

Scoring by hand can take several minutes per visit, making routine use difficult in busy clinics. Some practices are turning to digital and AI-assisted tools to calculate the score from patient-reported data and chart documentation.

Underdiagnosis compounds the problem, with diagnosis often delayed by years.² An estimated 50% of patients are undiagnosed or misdiagnosed. The 2016 ACR/EULAR classification criteria serve research purposes and miss many patients, so they should not function as diagnostic criteria.³

Younger patients may report no dryness, or may have adapted to it over years. Extraglandular clues include tubulointerstitial nephritis, renal tubular acidosis, small fiber neuropathy often mistaken for fibromyalgia, inflammatory eye disease, pleural effusions, and interstitial lung disease.

Diagnostic guidance expected over the next year may help advanced practice providers and generalists screen more effectively. Broader recognition of extraglandular disease will determine how many eligible patients new agents ultimately reach.

References

1. Seror R, Ravaud P, Bowman SJ, et al. EULAR Sjogren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjogren’s syndrome. Ann Rheum Dis. 2010;69(6):1103-1109. doi:10.1136/ard.2009.110619

2. Aung T. Bridging the gap in Sjögren’s disease: a comprehensive review of unmet needs, diagnostic challenges, and emerging therapeutic strategies. Cureus. 2026;18(7):e113186. doi:10.7759/cureus.113186

3. Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatol. 2017;69(1):35-45. doi:10.1002/art.39859