Tuberc Respir Dis > Accepted Articles
DOI: https://doi.org/10.4046/trd.2024.0148    [Accepted]
Published online January 10, 2025.
Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Connective Tissue Disease Associated Interstitial Lung Disease
Ju Hyun Oh1, Jae Ha Lee2, Sung Jun Chung3, Young Seok Lee4, Tae-Hyeong Kim5, Tae-Jung Kim6, Joo Hun Park7, on behalf of Korean Interstitial Lung Diseases Study Group
1Department of Pulmonology and Critical Care Medicine, Sanggye Paik Hospital, Inje University College of Medicine, Seoul, Korea
2Division of Pulmonology and Critical Care Medicine, Department of Internal Medicine, Haeundae Paik Hospital, Inje University College of Medicine, Busan, Korea
3Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea
4Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Internal Medicine, Korea University Guro Hospital, Korea University College of Medicine, Seoul, Korea
5Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Hanyang University Guri Hospital, Hanyang University College of Medicine, Seoul, Korea
6Department of Hospital Pathology, College of Medicine, The Catholic University of Korea, Seoul, Korea
7Department of Pulmonary and Critical Care Medicine, Ajou University School of Medicine, Suwon, Korea
Correspondence:  Joo Hun Park, Tel: 82-31-219-5116, Fax: 82-31-219-5124, 
Email: jhpamc@naver.com
Received: 1 October 2024   • Revised: 5 December 2024   • Accepted: 6 January 2025
Abstract
Connective tissue disease (CTD) comprising a various range of autoimmune disorders is often accompanied by lung involvement, which can lead to life-threatening complications. The main types of CTDs that can present as interstitial lung disease (ILD) include rheumatoid arthritis, systemic sclerosis, Sjögren's syndrome, mixed connective tissue disease, idiopathic inflammatory myopathies, and systemic lupus erythematosus. CTD-ILD poses a significant challenge in clinical diagnosis and management due to its heterogeneous nature and variable prognosis. Early diagnosis by clinical, serological, and radiographic assessments is crucial to differentiate CTD-ILD from idiopathic forms and to adopt appropriate therapeutic strategies. Therefore, we have reviewed the numerous clinical manifestations and diagnostic approaches for each type of CTD-ILD, considering the diversity and complexity of CTD-ILD. The significance of a multidisciplinary approach in optimizing the management of CTD-ILD has been underscored by recent therapeutic advancements, which include the use of immunosuppressive agents, antifibrotic therapies, and newer biological agents targeting specific pathways implicated in the pathogenesis. Therapeutic strategies should be tailored to each condition, considering the type of CTD, the extent of lung involvement, and the presence of extrapulmonary manifestations. Furthermore, we aimed to provide clinical guidance including therapeutic recommendations for the effective management of CTD-ILD, based on PICO analysis.


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