Background/Purpose: Primary Sjögren’s syndrome (pSS) is a chronic autoimmune exocrinopathy that features interstitial lung disease (ILD) in up to 16% of patients. The clinical characteristics and natural history of pSS-associated ILD (pSS-ILD) are not well defined.

Methods: Patients with pSS-ILD were identified at two academic institutions: University of Cincinnati and University of Washington. Clinical data including demographics, diagnosis dates, serologies, imaging findings, histopathology, pulmonary function testing results, and treatments were abstracted from chart review. ILD patterns were assigned on the basis of histopathology (when available) and radiology characteristics on chest high-resolution CT scans. Categorical variables are summarized as frequencies and percentages, and continuous variables are summarized as median and range. Survival analysis was conducted using Kaplan-Meier method.

Results: There were a total of 81 patients with pSS-ILD in our cohort. The majority of the patients (86%, 70/81) were women. Sjögren’s-related antibodies were common. Positive SSA alone was observed in 49%, positive SSB alone was observed in 3%, and both SSA and SSB were observed in 40%. ILD was the presenting manifestation of pSS in 17 (21%) patients. ILD and pSS were diagnosed concurrently in 34 (43%) patients. In the remaining patients (36%, 28/79), the diagnosis of ILD was established after a median of 6 years (range 1-31 years) following the diagnosis of pSS.

The most common ILD pattern in our cohort was usual interstitial pneumonia (UIP) (32%, 25/78), followed by nonspecific interstitial pneumonia (NSIP) (28%, 22/78), lymphocytic interstitial pneumonia (LIP) (21%, 16/78), and organizing pneumonia (OP) (10%, 8/78). Over a median follow-up of 4 years, 70% (46/66) had a stable or improving functional vital capacity, and 75% (47/63) had a stable or improving diffusion capacity for carbon monoxide.

The diagnosis of pSS-ILD led to a new immunomodulatory therapy in 54 (67%) patients. The most common steroid-sparing immunomodulatory medications utilized were mycophenolate (49%), hydroxychloroquine (44%), azathioprine (25%), and rituximab (7%). 21 patients (26%) with pSS-ILD died during follow-up. The median survival time after ILD diagnosis was 11 years (95% confidence interval: 6-12 years).

Conclusion: ILD is frequently seen and can be the presenting manifestation in patients with pSS. UIP and NSIP are the most common ILD patterns in patients with pSS-ILD.  Although most patients with pSS-ILD have stable to improving pulmonary function tests over time, approximately one-fourth may continue to decline over time.  Close serial monitoring is warranted to identify the subset of patients that are declining and institute timely interventions. The diagnosis of ILD has a substantial impact on pharmacological therapy and overall patient survival.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-characteristics-and-outcomes-in-patients-with-primary-sjogrens-syndrome-associated-interstitial-lung-disease/