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Connective Tissue Disease Associated Interstitial Lung Disease.

Scott M Matson1, M Kristen Demoruelle2

  • 1Division of Pulmonary, Critical Care and Sleep Medicine, University of Kansas School of Medicine, 3901 Rainbow boulevard, Mailstop 3007, Kansas City, KS 66160, USA.

Immunology and Allergy Clinics of North America
|April 13, 2023
PubMed
Summary

Connective tissue disease associated interstitial lung disease (CTD-ILD) requires more research. Randomized controlled trials are needed to evaluate immunosuppression and antifibrotic treatments for fibrotic CTD-ILD and subclinical cases.

Keywords:
AntifibroticAutoimmunityConnective tissue diseaseImmunosuppressionInterstitial lung diseaseSubclinical interstitial lung disease

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Area of Science:

  • Pulmonology
  • Rheumatology
  • Immunology

Background:

  • Connective tissue disease associated interstitial lung disease (CTD-ILD) presents a heterogeneous spectrum of interstitial lung disease (ILD) manifestations.
  • Current treatment guidelines for lung-directed immunosuppression in CTD-ILD are based on limited evidence, primarily from scleroderma patients and observational studies in other autoimmune conditions.

Purpose of the Study:

  • To highlight the urgent need for rigorous clinical trials in CTD-ILD.
  • To advocate for the investigation of immunosuppression and antifibrotic agents in fibrotic CTD-ILD.
  • To emphasize the importance of studying interventions for subclinical CTD-ILD.

Main Methods:

  • Review of existing randomized, placebo-controlled trials (RCTs) in scleroderma-associated ILD.
  • Analysis of observational, retrospective studies in other autoimmune conditions.
  • Identification of research gaps in fibrotic and subclinical CTD-ILD.

Main Results:

  • Evidence supporting immunosuppression in CTD-ILD is limited and primarily derived from specific subgroups.
  • The potential harm of immunosuppression in idiopathic pulmonary fibrosis underscores the need for caution and targeted research in CTD-ILD.

Conclusions:

  • There is a critical need for well-designed RCTs to evaluate immunosuppressive and antifibrotic therapies in patients with fibrotic CTD-ILD.
  • Further research is essential to explore interventions for individuals with subclinical CTD-ILD to prevent disease progression.