Reference Work Entry

Encyclopedia of Molecular Mechanisms of Disease

pp 1840-1842

Restrictive Lung Disease

  • Alfredo ChettaAffiliated withInstitute of Physiology, University of TuebingenDepartment of Clinical Sciences, Respiratory Disease Section, University of Parma
  • , Dario OlivieriAffiliated withInstitute of Physiology, University of TuebingenDepartment of Clinical Sciences, Respiratory Disease Section, University of Parma

Synonyms

Diffuse parenchymal lung diseases; Idiopathic pulmonary fibrosis; Interstitial lung disease; Sarcoidosis; Kyphoscoliosis; Neuromuscular disorders; RLD

Definition and Characteristics

Restrictive lung disease (RLD) comprises a heterogeneous group of chronic disorders characterized by a decrease in the ability to expand the lungs, as a consequence of either inflammation and/or scarring of the lung parenchyma (diffuse parenchymal disease) [1] or disease of the components of the respiratory pump, such as the pleura, chest wall, and neuromuscular apparatus (extraparenchymal disease) (Fig. 1). There is no unique molecular pathogenetic mechanism in RLD, given the heterogeneity of the disease. Furthermore, the molecular pathophysiology of the different diseases comprised in RLD is not yet well known. This chapter deals with RLD due to diffuse parenchymal diseases.

Restrictive Lung Disease. Figure 1

Diagrammatic representation of diffuse parenchymal lung ...

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