PMID- 31977850 OWN - NLM STAT- MEDLINE DCOM- 20200210 LR - 20221005 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 99 IP - 4 DP - 2020 Jan TI - Impact of a systematic evaluation of connective tissue disease on diagnosis approach in patients with interstitial lung diseases. PG - e18589 LID - 10.1097/MD.0000000000018589 [doi] LID - e18589 AB - To 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. FAU - Hernandez-Gonzalez, Fernanda AU - Hernandez-Gonzalez F AD - Servei de Pneumologia, Hospital Clinic, IDIBAPS, Universitat de Barcelona. FAU - Prieto-Gonzalez, Sergio AU - Prieto-Gonzalez S AD - Servei de Malalties Autoimmunes, Hospital Clinic. FAU - Brito-Zeron, Pilar AU - Brito-Zeron P AD - Servei de Malalties Autoimmunes, Hospital Clinic. FAU - Cuerpo, Sandra AU - Cuerpo S AD - Servei de Pneumologia, Hospital Clinic, IDIBAPS, Universitat de Barcelona. FAU - Sanchez, Marcelo AU - Sanchez M AD - Servicio de Radiodiagnostico, Hospital Clinic. FAU - Ramirez, Jose AU - Ramirez J AD - Servicio de Anatomia Patologica, Hospital Clinic. FAU - Agusti, Carlos AU - Agusti C AD - Servei de Pneumologia, Hospital Clinic, IDIBAPS, Universitat de Barcelona. FAU - Lucena, Carmen Maria AU - Lucena CM AD - Servei de Pneumologia, Hospital Clinic, IDIBAPS, Universitat de Barcelona. FAU - Paradela, Marina AU - Paradela M AD - Servei de Cirurgia Toracica, Hospital Clinic, IDIBAPS, Universitat de Barcelona, Barcelona. FAU - Grafia, Ignacio AU - Grafia I AD - Servei de Malalties Autoimmunes, Hospital Clinic. FAU - Espinosa, Gerard AU - Espinosa G AD - Servei de Malalties Autoimmunes, Hospital Clinic. FAU - Sellares, Jacobo AU - Sellares J AD - Servei de Pneumologia, Hospital Clinic, IDIBAPS, Universitat de Barcelona. AD - Centro de Investigacion Biomedica En Red-Enfermedades Respiratorias (CibeRes, CB06/06/0028), Barcelona, Spain. LA - eng PT - Journal Article PT - Observational Study PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Autoantibodies) SB - IM MH - Aged MH - Aged, 80 and over MH - Autoantibodies/immunology MH - Biopsy MH - Connective Tissue Diseases/*complications/*diagnosis/pathology MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Lung Diseases, Interstitial/*complications/*diagnosis/pathology MH - Male MH - Middle Aged MH - Tomography, X-Ray Computed PMC - PMC7004576 COIS- This work has been financed with the grant SLT008/18/00176 and the support of the Department of Health of Generalitat de Catalunya, in the call for grants 2019-2021, under a competitive regime, for the financing of different programs and instrumental actions included in the Strategic Research and Innovation Plan in Health 2016-2020. It has also been financed by SEPAR, SOCAP, FUCAP and with the PhD4MD Programme of the Institute for Research in Biomedicine (IRB) Barcelona, Hospital Clinic of Barcelona and the Institut d'Investigacions Biomediques August Pi i Sunyer (IDIBAPS). The authors have no conflicts of interest to disclose. EDAT- 2020/01/25 06:00 MHDA- 2020/02/11 06:00 PMCR- 2020/01/24 CRDT- 2020/01/25 06:00 PHST- 2020/01/25 06:00 [entrez] PHST- 2020/01/25 06:00 [pubmed] PHST- 2020/02/11 06:00 [medline] PHST- 2020/01/24 00:00 [pmc-release] AID - 00005792-202001240-00009 [pii] AID - MD-D-19-07317 [pii] AID - 10.1097/MD.0000000000018589 [doi] PST - ppublish SO - Medicine (Baltimore). 2020 Jan;99(4):e18589. doi: 10.1097/MD.0000000000018589.