2020 guide for the diagnosis and treatment of interstitial lung disease associated with connective tissue disease

Respir Investig. 2021 Nov;59(6):709-740. doi: 10.1016/j.resinv.2021.04.011. Epub 2021 Oct 1.


The prognosis of patients with connective tissue disease (CTD) has improved significantly in recent years, but interstitial lung disease (ILD) associated with connective tissue disease (CTD-ILD) remains a refractory condition, which is a leading cause of mortality. Because it is an important prognostic factor, many observational and interventional studies have been conducted to date. However, CTD is a heterogeneous group of conditions, which makes the clinical course, treatment responses, and prognosis of CTD-ILD extremely diverse. To summarize the current understanding and unsolved questions, the Japanese Respiratory Society and the Japan College of Rheumatology collaborated to publish the world's first guide focusing on CTD-ILD, based on the evidence and expert consensus of pulmonologists and rheumatologists, along with radiologists, pathologists, and dermatologists. The task force members proposed a total of 27 items, including 7 for general topics, 9 for disease-specific topics, 3 for complications, 4 for pharmacologic treatments, and 4 for non-pharmacologic therapies, with teams of 2-4 authors and reviewers for each item to prepare a consensus statement based on a systematic literature review. Subsequently, public opinions were collected from members of both societies, and a critical review was conducted by external reviewers. Finally, the task force finalized the guide upon discussion and consensus generation. This guide is expected to contribute to the standardization of CTD-ILD medical care and is also useful as a tool for promoting future research by clarifying unresolved issues.

Keywords: Antifibrotic agent; Immunosuppressant; Myositis; Rheumatoid arthritis; Systemic sclerosis.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Connective Tissue Diseases* / complications
  • Connective Tissue Diseases* / diagnosis
  • Connective Tissue Diseases* / therapy
  • Humans
  • Japan / epidemiology
  • Lung Diseases, Interstitial* / diagnosis
  • Lung Diseases, Interstitial* / etiology
  • Lung Diseases, Interstitial* / therapy
  • Prognosis
  • Pulmonologists