Background/Purpose: The reported frequency of pulmonary involvement in primary Sjögren’s Syndrome (pSS) varies widely ranging from 8 to 75% depending on the detection method employed and consists of various forms of airways disease. There is little information regarding interstitial lung disease (ILD) patterns other than lymphocytic interstitial pneumonia occurring in pSS in terms of frequency and risk factors. We aimed to assess incidence, and mortality of ILD in a well-characterized, population-based cohort of patients with pSS.
Methods: We examined a population-based incidence cohort of patients diagnosed with pSS in 1976-2005. All subjects were followed longitudinally through their complete community medical records, until death, migration or August 2011. ILD was defined using strict, validated composite criteria developed by an expert panel including pulmonologists and rheumatologists. These criteria included physician diagnosis, radiologic data, pulmonary function parameters, lung biopsy and autopsy findings. Cumulative incidence adjusted for the competing risk of death was estimated. A Cox model with a time-dependent covariate for development of ILD was used to examine the impact of ILD on survival in patients with pSS.
Results: 105 patients with pSS were identified (mean age 58.1 years; range 23-95; 91% female). Lung disease was present prior to diagnosis of pSS in 12 patients and developed after diagnosis of pSS in 35 patients with a median follow-up time of 9.2 years (1205 total person-years). Among pSS patients without prior ILD, the cumulative incidence of ILD in patients with pSS was 10% (± 3%) at 1 year after diagnosis of pSS and increased to 20% (± 4%) by 5 years after pSS. The development of lung disease in pSS was associated with poor survival with an hazard ratio of 2.16 (95%CI: 0.99, 4.74) adjusted for age, sex, and calendar year. ILD was identified as the most frequent type of lung disease detected at or after pSS diagnosis (53%) followed by emphysema (13%)
Conclusion: Our findings emphasize the high incidence of ILD and the adverse effect on survival in patients with pSS. Patients with pSS should be carefully assessed for diagnosis and treatment of ILD in order to improve the detrimental survival experience.
Disclosure:
C. Nannini,
None;
A. Jebakumar,
None;
J. H. Ryu,
None;
C. S. Crowson,
None;
E. L. Matteson,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/interstitial-lung-disease-in-sjogren-syndrome-a-population-based-study/