Multidisciplinary Consensus on Screening and Diagnosis of Interstitial Lung Disease (ILD) Associated with Systemic Autoimmune Rheumatic Diseases (SARDs)
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Abstract
Introduction: Interstitial lung disease (ILD) is a common and high-impact manifestation in patients with systemic autoimmune rheumatic diseases (SARDs). Timely detection requires clinical tools based on evidence and multidisciplinary consensus.
Methods: A multidisciplinary team developed the consensus for the screening and diagnosis of ILD-SARDs through the formulation of PICO clinical questions, a systematic literature review (2017–2023) reported in accordance with the PRISMA statement, evaluation of the evidence using the GRADE system, and the formulation of clinical practice recommendations, which were validated using the Delphi methodology.
Results: Twelve key recommendations were established for the screening and diagnosis of interstitial lung disease (ILD) in patients with Rheumatoid Arthritis (RA), Systemic Sclerosis (SSc), Systemic Lupus Erythematosus (SLE), Sjögren’s Syndrome (SS), Mixed Connective Tissue Disease (MCTD) and Idiopathic Inflammatory Myopathies (IIM). The use of high-resolution computed tomography (HRCT) and pulmonary function tests is highlighted as fundamental diagnostic tools. Respiratory assessment is recommended at each visit, along with active surveillance of fibrotic progression risk in diagnosed patients. Routine lung biopsy is not suggested, except in cases of suspected malignancy or discordant diagnoses.
Conclusion: The implementation of evidence-based recommendations for the screening and diagnosis of ILD-SARDs aims to improve early detection, multidisciplinary intervention and patient prognosis. Their adoption can help standardize clinical practices and optimize care across all levels of the healthcare system.
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García Valdés MG, Soto SM, Armas RR, Hernández GMM. Delphi method for the expert consultation in scientific research. Rev Cubana Salud Pública. 2013;39(2):253-267.
Chiang G, Parimon T. Understanding interstitial lung diseases associated with connective tissue diseases: Genetics, cellular pathophysiology and biologic drivers. Int J Mol Sci 2023;24(3):2405. https://doi.org/10.3390/ijms24032405
Rodríguez-Portal JA, Molina FN, Carmona DP. Recomendaciones SER-SEPAR para el manejo de la EPID en artritis reumatoide. Reumatol Clin 2022;18:443–52. https://doi.org/10.1016/j.reuma.2022.03.005
Joy GM, Wilsher ML, Conlon AC, Kelly CA. Prevalence, imaging patterns and risk factors of ILD in CTD: a systematic review and meta-analysis. Eur Respir Rev 2023;32:220210. https://doi.org/10.1183/16000617.0210-2022
Raghu G, Richeldi L, Ryerson CJ et al. Idiopathic pulmonary fibrosis and progressive pulmonary fibrosis in adults: Clinical practice guideline. Am J Respir Crit Care Med 2022;205(9):e18–e47. https://doi.org/10.1164/rccm.202202-0399ST
Giménez Palleiro A, Franco T. Radiological patterns in ILD. Semin Fund Esp Reumatol 2013;14(4):97–105. https://doi.org/10.1016/j.semreu.2013.05.002
Ruano CA, Gómez MG, Bello A. Multimodality imaging in CTD-ILD. Clin Radiol 2021;76(2):88–95. https://doi.org/10.1016/j.crad.2020.07.035
Hoffmann-Vold AM, Allanore Y, Alves M et al. Identification and management of ILD in systemic sclerosis: European consensus. Lancet Rheumatol 2020;2:e71–e83. https://doi.org/10.1016/S2665-9913(19)30144-4
Panagopoulos P, Goules A, Hoffmann-Vold AM et al. Natural history and screening of ILD in systemic autoimmune diseases. Ther Adv Musculoskelet Dis 2021;13:1–21. https://doi.org/10.1177/1759720X211037519
Guler SA, Scheschkowski T, Renner A et al. Interdisciplinary diagnosis and management of CTD-ILD. Chest 2024. https://doi.org/10.1016/j.chest.2024.02.045
Peredo RA, Mehta V, Beegle S. Interstitial lung disease associated with CTD: overview and diagnosis. Respir Med 2015;109(7):830–8. https://doi.org/10.1007/978-3-030-68748-9_5
Kondoh Y, Taniguchi H, Takeyama S et al. 2020 guide for the diagnosis and treatment of interstitial lung disease associated with connective tissue disease 2021;59(6):709–40. https://doi.org/10.1016/j.resinv.2021.04.011
Lee CT, Semple EF, Min JH et al. CTD-ILD in Sjögren’s, MCTD and SLE. Curr Opin Pulm Med 2022;27(5):388–95. https://doi.org/10.1097/MCP.0000000000000791
Ministerio de Salud Pública. Manual metodológico para adoptar, adaptar y elaborar guías de práctica clínica. Quito: MSP; 2022. [Internet]. [Consultado 3 jul 2025]. Disponible en: http://salud.gob.ec
National Institute for Health and Care Excellence. Developing NICE guidelines: the manual. Manchester: NICE; 2014. [Internet]. [Consultado 3 jul 2025]. Disponible en: https://www.nice.org.uk/process/pmg20
Instituto Aragonés de Ciencias de la Salud. Elaboración de guías de práctica clínica en el Sistema Nacional de Salud. Zaragoza: Guía Salud; 2016. [Internet]. [Consultado 3 jul 2025]. Disponible en: http://portal.guiasalud.es/emanuales/elaboracion_2/?capitulo
Page MJ, McKenzie JE, Bossuyt PM et al. Declaración PRISMA 2020: una guía actualizada para la publicación de revisiones sistemáticas. Rev Esp Cardiol 2021;74(9):790–9. https://doi.org/10.1016/j.recesp.2021.06.016
Schünemann H, Brozek JL, Guyatt GH et al. GRADE para calificar la calidad de la evidencia y la fuerza de la recomendación. 1a ed. en español; Reumatol Clin 2018;14(2):65-67. https://doi.org/10.1016/j.reuma.2017.07.010
Sellarés J, Hernandez-González F, Lucena C et al. Auscultation of velcro crackles is associated with usual interstitial pneumonia. Medicine (Baltimore) 2016;95(21):e2573. https://doi.org/10.1097/MD.0000000000002573
Bernstein EJ, Khanna D, Lederer DJ. Screening HRCT to detect ILD in systemic sclerosis: a global survey. Arthritis Rheumatol 2018;70(6):971–2. https://doi.org/10.1002/art.40441
Abu Qubo A, Khalifah KC. The Role of Radiology in Progressive Fibrosing Interstitial Lung Disease. Front Med (Lausanne) 2022:8:679051. https://doi.org/10.3389/fmed.2021.679051
Coghlan JG, Denton CP, Grünig E et al. Evidence-based detection of pulmonary hypertension in SSc: DETECT study. Ann Rheum Dis 2014;73:1340–9. https://doi.org/10.1136/annrheumdis-2013-203301
Wolff C, Valenzuela O, Lagos V. Rheumatological evaluation of ILD patients. Rev Chil Enferm Respir 2019;35(4):244–51.
Caron M, Khanna D, Allanore Y et al. Pulmonary function tests as outcomes for SSc-ILD. Eur Respir Rev 2018;27(148):170102. https://doi.org/10.1183/16000617.0102-2017
Orlandi M, Matucci-Cerinic M, Guiducci S. Bronchoalveolar lavage in SSc-ILD: Systematic review. J Clin Med 2022;15(12):4172. https://doi.org/10.3390/ph15121584
Tomassetti S, Ravaglia C, Puglisi S et al. Impact of lung biopsy information on ILD treatment strategy. Ann Am Thorac Soc 2022;19(5):737–45. https://doi.org/10.1513/AnnalsATS.202104-466OC
Hetzel J, Maldonado F, Ravaglia C et al. Transbronchial cryobiopsy in DPLD: safety and standardization. Respiration 2018;95:188–200. https://doi.org/10.1159/000484055
Kheir F, Uribe Becerra JP, Bissell B et al. Transbronchial lung cryobiopsy in patients with interstitial lung disease: a systematic review. Ann Am Thorac Soc 2022;19:1193-202. https://doi.org/10.1513/AnnalsATS.202102-198OC
Ghuman A, Kolb M, Fraser L et al. Prognostic markers in SSc-ILD from trial and cohort. Rheumatology 2023;1–10. https://doi.org/10.3389/fimmu.2020.01990
Qiu M, Xie J, Niu X et al. Factors associated with mortality in rheumatoid arthritis-associated interstitial lung disease: a systematic review and meta-analysis. Respir Res 2021;22:264. https://doi.org/10.1186/s12931-021-01856-z
Juge PA, Borie R, Dieude M et al. A risk score to detect subclinical rheumatoid arthritis-associated ILD. Arthritis Rheumatol 2022;74(11):1755–65. https://doi.org/10.1002/art.42162
Doyle TJ, Smith S, Bhattacharya B et al. Detection of rheumatoid arthritis–ILD is enhanced by serum biomarkers. Am J Respir Crit Care Med 2015;191(12):1403–12. https://doi.org/10.1164/rccm.201411-1950OC
Makol A, Nagaraja V, Crona DE. Recent innovations in the screening and diagnosis of systemic sclerosis-associated interstitial lung disease. Expert Rev Clin Immunol 2023;19(6):571–83. https://doi.org/10.1080/1744666X.2023.2198212
Rahaghi FF, Distler O, Khanna D et al. Expert consensus on management of SSc-ILD. Respir Res 2023;24:6. https://doi.org/10.1186/s12931-022-02292-3
Radić M, Dogas H, Gelemanović A et al. Pulmonary ultrasonography in SSc-ILD: Systematic review. Diagnostics 2023;13(7):1450. https://doi.org/10.3390/diagnostics13081429
Huang Y, Song TL, Hao S et al. Screening value of lung ultrasound in connective tissue disease related interstitial lung disease. Heart Lung 2023;57:110–6. https://doi.org/10.1016/j.hrtlng.2022.09.011
Fields A, Cheadle K, Price R et al. Mediators of SSc-ILD: Systematic review and meta-analysis. Interstitial Lung Dis 2023;78:799–807. https://doi.org/10.1136/thorax-2022-219226
Paulin F, Moreau T, Funck-Brentano C et al. Development of a risk indicator score for the identification of interstitial lung disease in patients with rheumatoid arthritis. Reumatol Clin 2021;17(4):207–11. https://doi.org/10.1016/j.reuma.2019.05.007
Kuwana M, Asanuma Y, Garofalo A. Role of autoantibodies in the diagnosis and prognosis of interstitial lung disease in autoimmune rheumatic disorders. Ther Adv Musculoskelet Dis 2021;13:1–17. https://doi.org/10.1177/1759720X211032457
Tezcan D, Yildirim S, Korkmaz M et al. Evaluation of serum interleukin-6 (IL-6), IL-13, and IL-17 levels and computed tomography finding in interstitial lung disease associated with connective tissue disease patients. Clin Rheumatol 2021;40:4713–24. https://doi.org/10.1007/s10067-021-05773-w
Berardicurti O, Lazzaroni MG, Gatto M et al. Interstitial Lung Disease and Pulmonary Damage in Primary Sjögren's Syndrome: A Systematic Review and Meta-Analysis. J Clin Med 2023;12(7):2586. https://doi.org/10.3390/jcm12072586