Background: Primary Sjögren’s syndrome (pSjS) is a chronic and complex systemic autoimmune disease, primarily characterised by inflammation and progressive destruction of the exocrine glands (ie, autoimmune epithelitis) [1].

Objectives: We evaluated disease progression, treatment patterns, mortality, and healthcare resource utilization (HCRU) of pSjS patients in the United States (US) to understand the real-world experiences of patients with pSjS.

Methods: This retrospective cohort study utilised data from US Optum Clinformatics claims between 01 May 2000 and 31 December 2020. The study included pSjS patient cohort and general population cohort, matched (1:1) on age, sex, and index date. Baseline period of 365 days prior to the index date was used for assessment of baseline comorbidities. Descriptive statistics were used to describe baseline characteristics, HCRU, and treatment pattern while multivariable models were used to assess hazard ratios (HRs) and risk factors.

Results: Overall, 23,168 patients with pSjS (ICD-9 710.2 Sicca syndrome and ICD-10 M 35.0 Sjogren Syndrome and excluding patients with Rheumatoid arthritis [RA], Systemic sclerosis [SS] and Systemic lupus erythematosus [SLE] diagnoses codes) were included in the cohort (mean [SD] age: 61.5 [15.3] years; females: 85%). At baseline, 79.4% and 8.2% of pSjS patients had systemic complications and organ-specific autoimmune comorbidities, respectively. In a sub-set of the cohort including patients with at least 5 years of follow-up, by the end of 5 years 96.8% and 16.5% of patients developed systemic complications and organ-specific autoimmune comorbidities, respectively ( Table 1 ). The most frequently occurring organ-specific autoimmune comorbidities over the 5-year follow-up included Graves disease (5.4%), Hashimoto disease (3.7%), and discoid/subacute cutaneous lupus erythematosus (3.5%). Mortality was reported in 7.4% of the patients during 5-year follow-up. Risk factors associated with higher mortality included systemic complications in renal (HR [95% CI]: 2.29 [2.09–2.52]), cardiovascular (HR [95% CI]: 2.42 [2.19–2.67]), lungs (HR [95% CI]: 3.73 [3.41–4.09]) and haematological domains (HR [95% CI]: 2.83 [2.56–3.13]), non-Hodgkin’s lymphoma (HR [95% CI]: 2.58 [2.12–3.14]), and primary biliary cirrhosis (HR [95% CI]: 2.17 [1.60–2.96]). Corticosteroids (28.2%), hydroxychloroquine (15.7%), and cyclosporine (10.9%) were most frequently used medications. During the year following the first pSjS diagnosis, defined as the first claim with the Sjogren Syndrome ICD code (index date), the mean all-cause healthcare costs have increased by 27% from $21,634 to $27,526 per patient per year.

Conclusion: These results provide additional evidence that pSjS is associated with substantial morbidity and clinical burden supporting the need for safe and efficacious disease modifying treatment options in this patient population.

REFERENCES:

[1]Mariette X, Criswell LA. N Engl J Med . 2018;378(10): 931-939.

Acknowledgements: Medical writing and editorial assistance were provided by Sanjeev Kallapari and Chiranjit Ghosh, PhD of Sanofi. This study was funded by Sanofi.

Disclosure of Interests: LAMA KALOUCHE-KHALIL Shareholder of: May hold stock/stock options in Sanofi., Employee of: Employee of Sanofi., Roopali Gandhi Shareholder of: May hold stock/stock options in Sanofi., Employee of: Employee of Sanofi., Lichen Hao Shareholder of: May hold stock/stock options in Sanofi., Employee of: Employee of Sanofi., Ekaterina Smolkina Shareholder of: May hold stock/stock options in Sanofi., Employee of: Employee of Sanofi., Fabienne Schumacher Shareholder of: May hold stock/stock options in Sanofi., Employee of: Employee of Sanofi.