Diagnostic Delays Linked to Greater Disease Activity, Worse Quality of Life in Sjögren Disease

Patients with Sjögren disease (SjD) who were diagnosed more than a year after symptom onset had 2.3 times the odds of moderate or greater disease activity compared with those diagnosed within 3 months, according to a real-world US survey published in ACR Open Rheumatology.¹

“This analysis suggests an unmet treatment need for patients with SjD,” the authors concluded. “Although clinical guidelines exist to help manage treatment decisions, treatment of SjD remains based on the symptomatic treatment of sicca manifestations and immunosuppression for systemic disease.”

Real-World Data From Rheumatology Practices

Researchers used data from the Adelphi Primary Sjögren’s Syndrome Disease Specific Programme, a cross-sectional survey conducted in the US in 2018. In it, rheumatologists reported on their next 6 consecutive eligible patients with primary SjD using medical records, and patients could also complete their own questionnaires.

Overall, 84 rheumatologists provided data on 511 patients, whose mean age was 53.3 years; 88.5% were female. Based on proxy scores from the Clinical EULAR Sjögren’s Syndrome Disease Activity Index (ClinESSDAI), 44.2% of patients had mild disease, 30.9% had moderate disease, and 24.9% had severe disease. Among 297 patients with available data, 33.7% had a EULAR Sjögren’s Syndrome Patient Reported Index (ESSPRI) proxy score of 5 or higher. The authors define a patient-acceptable symptom state as an ESSPRI score below 5.

The descriptive trends in scores on the EQ-5D-3L, the EuroQoL visual analog scale (EQ-VAS), the Functional Assessment of Chronic Illness Therapy–Fatigue (FACIT-F) scale, and the Work Productivity and Activity Impairment questionnaire suggested worsening impairment as disease severity increased.

How Patients With Sjögren Disease Reach a Diagnosis

After symptoms began, patients often first consulted a rheumatologist (38.0%) or a primary care physician (31.5%), and a rheumatologist ultimately made the diagnosis for 81.4% of patients. Physicians most often cited awaiting test results, specialist referral, and patients’ inability to consult sooner as reasons for delayed diagnosis. The most used treatments were over-the-counter therapies such as eye drops (56%), antimalarials (52%), and conventional synthetic disease-modifying antirheumatic drugs (38%), while 12% of patients received biologics.

Time to Diagnosis and Sjögren Disease Activity

The mean time from symptom onset to diagnosis was 2.3 years (SD, 5.6). Among 276 patients with available data, 29.3% were diagnosed within 3 months and 32.6% after more than 12 months. In unadjusted analyses, EQ-5D-3L utility (P = .042), EQ-VAS (P = .026), FACIT-F (P = .007), and ESSPRI proxy scores (P = .001) differed significantly by length of delay. The quality-of-life and fatigue scores were numerically lower, and ESSPRI scores were numerically higher, with longer delays.

Researchers also ran models adjusted for time since diagnosis, age, sex, body mass index, and ethnicity. In those models, patients diagnosed more than 12 months after onset had 2.3 times the odds of a ClinESSDAI proxy score of 5 or higher (95% CI, 1.16-4.72; P = .018) compared with those diagnosed within 3 months. They also had significantly lower EQ-5D-3L utility (P = .013) and EQ-VAS scores (P = .004). The odds of an ESSPRI proxy score of 5 or higher were 2.1 times greater in this group, but the difference was not statistically significant (95% CI, 0.88-5.06; P = .096). FACIT-F and work impairment scores did not differ significantly by length of delay in adjusted models.

The authors concluded that greater disease activity, longer diagnostic delays, and inefficient patient referrals were associated with worsened symptom management and poorer outcomes, and that patients with moderate or severe disease who do not receive timely advanced treatment “may experience greater disease burden, highlighting an unmet treatment need.”

“Delays in diagnosis may be attenuated by SjD management based in centers of expertise and a multidisciplinary approach, with a principal role for specialists in autoimmune diseases and involvement of PCPs and other health care professionals,” the authors wrote.

Sjögren Disease Costs and Emerging Treatments

The Sjögren’s Foundation estimates that about 2.5 million Americans with the disease are undiagnosed and that the average time to diagnosis is 3 years. A MarketScan analysis presented at ISPOR 2026, whose authors include Amgen employees, found that commercially insured patients with SjD incurred mean annual all-cause health care costs of $27,147, compared with $9736 for matched controls, and were more likely to visit an emergency department (27% vs 19%).²

There is currently no FDA-approved disease-modifying therapy for SjD, and management is largely symptomatic. Monthly ianalumab (Novartis) met its primary end points in the phase 3 NEPTUNUS-1 and NEPTUNUS-2 trials, although a quarterly dosing arm in NEPTUNUS-2 did not significantly differ from placebo. The drug received FDA breakthrough therapy designation in January 2026. In September 2026, Amgen reported that dazodalibep met its primary end point in the phase 3 OASIZ 301 trial.

Study Limitations

The authors noted that rheumatologists were not required to apply standardized diagnostic criteria, so some patients may have been misdiagnosed, and patient selection was not formally verified. The sample may overrepresent patients who see their rheumatologist frequently and are more severely affected, and few patients with severe disease completed patient-reported measures. The findings relied on retrospective reporting, including the estimated time from symptom onset to diagnosis, which the authors said may underestimate the true delay. Disease activity and symptom scores were derived from proxy measures. Additionally, the cross-sectional design does not allow causal conclusions.¹

References

1. Lalla A, Egana A, Milligan J, Massey N, Haughton J. Patient journey, disease burden, treatment patterns, and the impact of delayed diagnosis among patients with Sjögren’s disease: a real-world US survey. ACR Open Rheumatol. 2026;8(10):e90143. doi:10.1002/acr2.90143
2. Joszt L. Pipeline advances in Sjögren disease bring targeted therapies closer. AJMC. October 2, 2026. Accessed October 8, 2026. https://www.ajmc.com/view/pipeline-advances-in-sj-gren-disease-bring-targeted-therapies-closer