作者
Boyang Zheng,D. Marinescu,Cameron Hague,Néstor L. Müller,Darra Murphy,Andrew Churg,Joanne L. Wright,A. Al-Arnawoot,Ana-Maria Bilawich,P. Bourgouin,Gerard Cox,C. Durand,T. Elliot,Jennifer D. Ellis,J.H. Fisher,Derek Fladeland,Amanda Grant-Orser,G.C. Goobie,Z. Guenther,Ehsan Haider,Nathan Hambly,James Huynh,Kerri A. Johannson,Geoffrey Karjala,Nasreen Khalil,Martin Kolb,Jonathan Leipsic,S.D. Lok,Sarah MacIsaac,Micheal McInnis,H. Manganas,Veronica Marcoux,John R. Mayo,Julie Morisset,Ciaran Scallan,T. Sedlic,Shane Shapera,Kelly Sun,V. Tan,Alyson W. Wong,Christopher J. Ryerson
摘要
Interstitial lung disease (ILD) in connective tissue diseases (CTD) have highly variable morphology. We aimed to identify imaging features and their impact on ILD progression, mortality and immunosuppression response.Patients with CTD-ILD had high-resolution chest computed tomography (HRCT) reviewed by expert radiologists blinded to clinical data for overall imaging pattern (usual interstitial pneumonia [UIP]; non-specific interstitial pneumonia [NSIP]; organizing pneumonia [OP]; fibrotic hypersensitivity pneumonitis [fHP]; and other). Transplant-free survival and change in percent-predicted forced vital capacity (FVC) were compared using Cox and linear mixed effects models adjusted for age, sex, smoking, and baseline FVC. FVC decline after immunosuppression was compared with pre-treatment.Of 645 CTD-ILD patients, the frequent CTDs were systemic sclerosis (n = 215), rheumatoid arthritis (n = 127), and inflammatory myopathies (n = 100). NSIP was the most common pattern (54%), followed by UIP (20%), fHP (9%), and OP (5%). Compared with UIP, FVC decline was slower for NSIP (1.1%/year, 95%CI 0.2, 1.9) and OP (3.5%/year, 95%CI 2.0, 4.9), and mortality was lower for NSIP (HR 0.65, 95%CI 0.45, 0.93) and OP (HR 0.18, 95%CI 0.05, 0.57), but higher in fHP (HR 1.58, 95%CI 1.01, 2.40). The extent of fibrosis also predicted FVC decline and mortality. After immunosuppression, FVC decline was slower compared with pre-treatment in NSIP (by 2.1%/year, 95%CI 1.4, 2.8), with no change for UIP or fHP.Multiple radiologic patterns are possible in CTD-ILD, including a fHP pattern. NSIP and OP were associated with better outcomes and response to immunosuppression, while fHP had worse survival compared with UIP.