Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis is scarring or thickening of the lungs without a known cause.

Theories of Etiology:

There are genetic markers of susceptibility for the genetics and pathogenesis of familial IPF.

  • A central repository of DNA samples and lung biopsies of patients with familial IPF would be useful and may provide the genetic material for defining this important subset of patients with IPF.

Environmental factors contributing to the pathogenesis of IPF.

  • Case control studies with matched, well-defined cases of IPF to define environmental associations would be informative.

DNA viruses in the pathogenesis of IPF.

Pathogenesis:

Inflammatory and parenchymal cells affect fibroblasts. Fibroblasts also alter inflammatory and parenchymal cells. Stimulation of fibroblasts causes fibrosis of the lung tissue.

“In the normal lung, the interstitium of the alveolar wall is thin and the number of fibroblasts is limited. Most fibroblasts and collagen fibers occur along blood vessels and conducting airways. The balance of fibrogenic and antifibrogenic factors must be toward suppression of fibroblast proliferation and matrix production. However, we know little about the antifibrogenic factors that are present in the normal lung.”

Symptoms:

  • chest pain
  • cough, usually dry
  • Decreased exercise tolerance
  • dyspnea, upon exertion or in progressive cases also at rest)Diagnostics:
    • broncoscopy with transbronchial lung biopsy
    • chest CT
    • chest x-ray
    • SpO2, blood oxygen level
    • pulmonary function tests
  • Lung tissue biopsy

Treatment and management:

  • There is no cure.
  • corticosteroids and cytotoxic drugs may help reduce swelling (inflammation) in some cases
  • supplemental oxygen
  • lung physiotherapy to maintain exercise tolerance
  • lung transplant in severe advanced cases

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001134/

http://www.nhlbi.nih.gov/meetings/workshops/ipf-sum.htm

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