The Korean journal of internal medicine

The Korean Association of Internal Medicine (대한내과학회)
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Treatment of connective tissue disease-associated interstitial lung disease: the pulmonologist's point of view

  • Koo, So-My (Division of Allergy and Respiratory Medicine, Department of Internal Medicine, Soon Chun Hyang University Seoul Hospital) ;
  • Uh, Soo-Taek (Division of Allergy and Respiratory Medicine, Department of Internal Medicine, Soon Chun Hyang University Seoul Hospital)
  • Received : 2016.07.31
  • Accepted : 2017.06.16
  • Published : 2017.07.01
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Abstract

Interstitial lung disease (ILD) occurs in 15% of patients with collagen vascular disease (CVD), referred to as connective tissue disease (CTD). Despite advances in management strategies, ILD continues to be a significant cause of mortality in patients with CVD-associated ILD (CTD-ILD). There is a lack of randomized, clinical trials assessing pharmacological agents for CTD-ILD, except in cases of ILD-associated systemic sclerosis (SSc). This may be due to the lack of CTD cases available, the difficulty of histological confirmation of ILD, and the various types of CTD and ILD. As a result, evidence-based pharmacological treatment of CTD-ILD is not yet well established. CTD-ILD presents with varying degrees of histology, from inflammation to fibrosis, and a wide spectrum of clinical manifestations, from minimal symptoms to respiratory failure. This renders it difficult for clinicians to make decisions regarding treatment options, observational strategies, optimal timing for interventions, and the appropriateness of pharmacological agents for treatment. There is no specific treatment for reversing fibrosis-like idiopathic pulmonary fibrosis in a clinical setting. This review describes pharmacological interventions for SSc-ILD described in randomized control trials, and presents an overview of recent advances of CTD-ILD-dependent treatments based on the types of CTD.

Keywords

Acknowledgement

Supported by : Soonchunhyang University

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