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Connective tissue disease-associated interstitial lung disease.

Karin Mueller Storrer1, Carolina de Souza Müller1, Maxwell Cássio de Albuquerque Pessoa1

  • 1Universidade Federal do Paraná - UFPR - Curitiba (PR) Brasil.

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|March 27, 2024
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Summary
This summary is machine-generated.

Connective tissue disease-associated interstitial lung disease (CTD-ILD) affects 15% of patients with autoimmune disorders, impacting morbidity and mortality. This review covers CTD-ILD diagnosis, treatment, and recent therapeutic advances.

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Area of Science:

  • Rheumatology and Pulmonology
  • Immunology
  • Internal Medicine

Background:

  • Connective tissue disease-associated interstitial lung disease (CTD-ILD) involves immune-mediated lung damage in systemic autoimmune disorders.
  • Commonly associated CTDs include systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myositis, and Sjögren's syndrome.
  • CTD-ILD impacts 15% of CTD patients, ranging from asymptomatic findings to severe respiratory failure.

Approach:

  • This review summarizes diagnostic strategies for CTD-ILD.
  • It outlines current pharmacological treatments, primarily immunosuppression with corticosteroids and cytotoxic agents.
  • Recent therapeutic advancements for various CTD-ILD forms are highlighted.

Key Points:

  • Determining which CTD patients require ILD screening remains a challenge.
  • Disease presentation varies widely, influencing prognosis and management.
  • Progressive CTD-ILD often necessitates aggressive immunosuppressive therapy.

Conclusions:

  • CTD-ILD is a significant complication of systemic autoimmune diseases.
  • Effective diagnosis and management are crucial for improving patient outcomes.
  • Ongoing research into novel therapeutics promises better treatment options for CTD-ILD.