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A7480 - Primary Sjögren’s Syndrome-Associated Interstitial Lung Disease—A Descriptive Study
Author Block: J. Marco1, N. Gupta2; 1Department of Internal Medicine, University of Cincinnati, Cincinnati, OH, United States, 2Department of Internal Medicine; Division of Pulmonary, Critical Care and Sleep Medicine, University of Cincinnati, Cincinnati, OH, United States.
Rationale
Sjögren’s syndrome (SS) is a chronic autoimmune inflammatory exocrinopathy. It may occur independent of other connective tissue diseases, termed primary SS (pSS), or it may occur in conjunction with other immune disorders, termed secondary SS. Interstitial lung disease (ILD) is present in approximately 16% of patients with pSS. The natural history of pSS-associated ILD (pSS-ILD) is not well defined.
Methods
We queried our medical center’s database using ICD-9 and ICD-10 codes to identify adult patients with pSS and ILD. Demographic data, diagnosis dates, imaging findings, pulmonary function testing (PFTs), clinical symptoms, lung biopsy results, and medical treatments were recorded.
Results
24 patients with pSS-ILD were included in our analysis. Our population was female predominant (88%, 21/24). ILD was the presenting manifestation of pSS in 4 patients, with pSS manifesting an average of 4 years (range 1-6 years) later. In the remaining patients, the diagnosis of ILD was established with mean follow-up period of 8.2 years (range 1-24 years) after the diagnosis of pSS.
Cough and dyspnea were the most common presenting symptoms seen in 83% and 79% of patients, respectively, followed by chest pain (12%) and sputum production (8%). Airway manifestations such as bronchiectasis and bronchiolectasis were the most common CT abnormalities, seen in 63% (15/24) of patients. Other common abnormalities included ground glass opacities (50%, 12/24), cysts (38%, 9/24), honeycomb changes (29%, 7/24), and subpleural fibrosis (25%, 6/24).
Histopathological diagnosis of ILD was established in 11 out of 24 (46%) of patients. Non-specific interstitial pneumonia was the most common ILD identified in 45% (5/11) of the patients, followed by organizing pneumonia (27%, 3/11), usual interstitial pneumonia (18%, 2/11), and lymphocytic interstitial pneumonia (9%, 1/11).
The diagnosis of pSS-ILD led to a change in treatment in 46% (11/24) of patients. The most common medications started were prednisone (64%), hydroxychloroquine (55%), mycophenolate (46%), and azathioprine (46%). At the most recent follow-up, 50% of patients reported improved symptoms compared to the time of diagnosis, and 59% of patients had improved or stable CT findings. Treatment of ILD led to a mean improvement in FEV1 and FVC of 2.14% and 3.14% predicted over an average follow-up period of 2.2 years.
Conclusions
ILD is commonly seen in patients with pSS, and can be the presenting manifestation of pSS. The diagnosis of ILD alters the management of patients with pSS, and immune-modulating treatment can have a beneficial effect on symptoms, CT findings, and PFTs.