Pulmonary manifestations of collagen diseases

Arch Bronconeumol. 2013 Jun;49(6):249-60. doi: 10.1016/j.arbres.2012.11.005. Epub 2013 Feb 4.
[Article in English, Spanish]


Collagen diseases are a large group of systemic inflammatory diseases of autoimmune etiology. The etiopathogenesis of collagen diseases is multifactorial. There is genetic susceptibility, as many connective tissue disorders show family history, and environmental factors may trigger the disease. Collagen diseases can affect almost all the organs of the body. The respiratory system is one of the most frequently affected, although the prevalence of pulmonary disease is not precisely known for the different collagen disorders. Any structure of the respiratory tract can be affected, but perhaps the most frequent is pulmonary parenchymal disease in the form of pneumonitis, which can be produced in any of the idiopathic interstitial pneumonitis patterns. The pleura, pulmonary vessels, airways and respiratory muscles may also be affected. The frequency of lung disease associated with collagen diseases is on the rise. This due in part to the better diagnostic methods that are available to us today (such as high-resolution computed tomography) and also to the appearance of new forms of pneumonitis associated with the new treatments that are currently used. The objective of this article is to offer a global vision of how collagen diseases can affect the lungs according to the latest scientific evidence.

Publication types

  • Review

MeSH terms

  • Bronchiectasis / etiology
  • Bronchiolitis Obliterans / etiology
  • Caplan Syndrome / etiology
  • Collagen Diseases / complications*
  • Collagen Diseases / physiopathology
  • Humans
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / physiopathology
  • Lung Diseases / etiology*
  • Lung Diseases / physiopathology
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / physiopathology
  • Lymphoproliferative Disorders / etiology
  • Pleural Effusion / etiology
  • Pneumonia / etiology
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / physiopathology