Interstitial lung disease in connective tissue diseases: evolving concepts of pathogenesis and management

Arthritis Res Ther. 2010;12(4):213. doi: 10.1186/ar3097. Epub 2010 Aug 23.


Interstitial lung disease (ILD) is a challenging clinical entity associated with multiple connective tissue diseases, and is a significant cause of morbidity and mortality. Effective therapies for connective tissue disease-associated interstitial lung disease (CTD-ILD) are still lacking. Multidisciplinary clinics dedicated to the early diagnosis and improved management of patients with CTD-ILD are now being established. There is rapid progress in understanding and identifying the effector cells, the proinflammatory and profibrotic mediators, and the pathways involved in the pathogenesis of CTD-ILD. Serum biomarkers may provide new insights as risk factors for pulmonary fibrosis and as measures of disease progression. Despite these recent advances, the management of patients with CTD-ILD remains suboptimal. Further studies are therefore urgently needed to better understand these conditions, and to develop effective therapeutic interventions.

Publication types

  • Review

MeSH terms

  • Biomarkers*
  • Connective Tissue Diseases* / etiology
  • Connective Tissue Diseases* / mortality
  • Connective Tissue Diseases* / therapy
  • Humans
  • Lung Diseases, Interstitial* / etiology
  • Lung Diseases, Interstitial* / mortality
  • Lung Diseases, Interstitial* / therapy
  • Morbidity
  • Risk Factors


  • Biomarkers