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A1096 - Prevalence of Small Airways Diseases in Systemic Sclerosis
Author Block: J. Yun1, J. Kim2, A. Podolanczuk2, E. J. Bernstein1, N. Patel2; 1Rheumatology, Columbia University, New York, NY, United States, 2Medicine - Division of Pulmonary, Allergy, and Critical Care, Columbia University, New York, NY, United States.
Rationale: Pulmonary manifestations of systemic sclerosis (SSc) are a major cause of morbidity and mortality. While a restrictive ventilatory pattern with interstitial lung disease (ILD) is a well-known and prominent feature of SSc, the prevalence and significance of small airway diseases (SAD) has not been thoroughly investigated despite prior observations from autopsies. The aims of this study are to determine the prevalence of SAD in SSc patients using all available methods, including lung biopsy, pulmonary function tests (PFTs), and HRCT in a large tertiary academic medical center.
Methods: We retrospectively analyzed all SSc patients, as diagnosed by ACR/EULAR 2013 criteria, evaluated at Columbia University Medical Center from November 2000 to November 2015. Patients with history of lung transplantation were excluded. Small airways disease was defined as the presence of one or more of the following: airway centered fibrosis on surgical lung biopsy, FEF 25-75% ≤ 50% on PFTs, and/or HRCT findings of bronchiolitis, mosaic attenuation, or air trapping on expiratory views.
Results: There were 136 patients with SSc. 118 were women (87%) with mean age of 58 years (range 26-90). 64 had diagnosis of ILD (47%), 44 were ever-smokers (32%), and 7 had history of COPD or asthma (5%). Of 136, 55 (40%) had evidence of SAD based on lung biopsy (n=6/12, 50%), PFTs (n= 33/52, 63%), and/or HRCT (n=26/47, 55%). Among patients with SAD, 25 (42%) patients had evidence of esophageal dysmotility or gastro-esophageal reflux disease (GERD) compared to only 22% in those without SAD. 30 (55%) patients with SAD did not have any traditional risk factors for SAD. Risk factors examined were: ever-smokers (n=22, 40%), history of overlap syndrome including RA (n=3, 5%), history of COPD/asthma (n=7, 13%), history of inflammatory bowel disease, sarcoidosis, tuberculosis or hypersensitivity pneumonitis (n=3, 5%).
Conclusion: Small airways diseases, based on HRCT, lung biopsy or PFTs findings, is highly prevalent among SSc patients in this retrospective cohort study at a large tertiary academic center. To our knowledge this is the largest cohort study that has examined SAD in SSc patients and one of the first to incorporate HRCT findings into the definition of SAD. Of those with SAD, a higher number of patients had evidence of esophageal dysmotility or GERD, and more than 50% did not have any traditional risk factors for SAD. This suggests that there may be SSc-specific pathophysiology in the development of SAD and warrants further investigation.