Hernández González, FernandaPrieto González, SergioBrito Zerón, María del PilarCuerpo Cardeñosa, SandraSánchez, MarceloRamírez Ruz, J. (José)Agustí García-Navarro, CarlesLucena, Carmen M.Paradela, MarinaGrafia, IgnacioEspinosa Garriga, GerardSellarés Torres, Jacobo2021-02-152021-02-152020-01-010025-7974https://hdl.handle.net/2445/173968To date, there is no clear agreement regarding which is the best method to detect a connective tissue disease (CTD) during the initial diagnosis of interstitial lung diseases (ILD). The aim of our study was to explore the impact of a systematic diagnostic strategy to detect CTD-associated ILD (CTD-ILD) in clinical practice, and to clarify the significance of interstitial pneumonia with autoimmune features (IPAF) diagnosis in ILD patients. Consecutive patients evaluated in an ILD Diagnostic Program were divided in 3 groups: IPAF, CTD-ILD, and other ILD forms. Clinical characteristics, exhaustive serologic testing, high resolution computed tomography (HRCT) images, lung biopsy specimens, and follow-up were prospectively collected and analyzed. Among 139 patients with ILD, CTD was present in 21 (15.1%), 24 (17.3%) fulfilled IPAF criteria, and 94 (67.6%) were classified as other ILD forms. Specific systemic autoimmune symptoms such as Raynaud phenomenon (19%), inflammatory arthropathy (66.7%), and skin manifestations (38.1%) were more frequent in CTD-ILD patients than in the other groups (all P< .001). Among autoantibodies, antinuclear antibody was the most frequently found in IPAF (42%), and CTD-ILD (40%) (P= .04). Nonspecific interstitial pneumonia, detected by HRCT scan, was the most frequently seen pattern in patients with IPAF (63.5%), or CTD-ILD (57.1%) (P< .001). In multivariate analysis, a suggestive radiological pattern by HRCT scan (odds ratio [OR] 15.1, 95% confidence interval [CI] 4.7-48.3, P< .001) was the strongest independent predictor of CTD-ILD or IPAF, followed by the presence of clinical features (OR 14.6, 95% CI 4.3-49.5, P< .001), and serological features (OR 12.4, 95% CI 3.5-44.0, P< .001). This systematic diagnostic strategy was useful in discriminating an underlying CTD in patients with ILD. The defined criteria for IPAF are fulfilled by a considerable proportion of patients referred for ILD.9 p.application/pdfengcc-by (c) Hernández González, Fernanda et al., 2020http://creativecommons.org/licenses/by/3.0/esMalalties del pulmóDiagnòsticMalalties autoimmunitàriesPulmonary diseasesDiagnosisAutoimmune diseasesinfo:eu-repo/semantics/article7070252021-02-15info:eu-repo/semantics/openAccess31977850