Identification and management of connective tissue disease-associated interstitial lung disease: evidence-based Japanese consensus statements. Issue 1 (2nd January 2023)

• Record Type: Journal Article
• Authors: Kuwana, Masataka; Bando, Masashi; Kawahito, Yutaka; Sato, Shinji; Suda, Takafumi; Kondoh, Yasuhiro
• Is Part Of: Expert review of respiratory medicine. Volume 17:Issue 1(2023)
• Page Start: 71
• Page End: 80
• Publication Date: 2023-01-02
• Subjects: Connective tissue diseases -- Japan -- lung diseases -- interstitial; Respiratory organs -- Diseases -- Periodicals; Respiratory organs -- Diseases -- Treatment -- Periodicals; 616.2005
• DOI: 10.1080/17476348.2023.2176303
• Languages: English
• ISSNs: 1747-6348

Abstract

ABSTRACT: Background: Interstitial lung disease (ILD) is a common complication of connective tissue diseases (CTD), but there are few clinical trials to guide disease management. We aimed to develop expert consensus statements and an algorithm for CTD-ILD management. Research design and methods: Based on a targeted literature review, we developed 109 statements on managing CTD-ILD across six domains. We used a modified Delphi process to survey 22 physicians in Japan involved in managing CTD-ILD (specialists in pulmonology, rheumatology, pathology, and radiology). These panelists participated in two rounds of web-based survey to establish consensus statements, which were used to define an algorithm. Consensus was defined as a mean value ≥70 on a scale of 0 (strong disagreement) to 100 (strong agreement). Results: Between May–August 2022, consensus was reached on 93 statements on CTD-ILD management. The most important consensus statements included screening CTD patients for ILD (typically with high-resolution computed tomography), using imaging, pulmonary function testing and serum biomarkers for diagnosis and severity assessment, regularly following up patients, and multidisciplinary management of CTD-ILD. Consensus statements were interpreted into an algorithm for clinical guidance. Conclusions: Using the Delphi process, we have developed consensus statements and an algorithm to guide clinical decision-making for CTD-ILD.