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[Idiopathic lung fibrosis].

A Schmid-Zumstein1, R Bernheim, T C Medici

  • 1Departement für Innere Medizin, Universitätsspital Zürich.

Schweizerische Medizinische Wochenschrift
|June 25, 1988
PubMed
Summary
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Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with cellular and fibrotic forms. Treatment aims to suppress inflammation, but only 12-30% of patients respond.

Area of Science:

  • Pulmonology
  • Interstitial Lung Diseases

Context:

  • Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease of unknown origin.
  • Two forms exist: cellular (desquamative interstitial pneumonia) and hypocellular (usual interstitial pneumonia), potentially representing different disease stages.

Purpose:

  • To describe the clinical presentation, diagnostic methods, and treatment strategies for Idiopathic Pulmonary Fibrosis.
  • To outline the varying prognoses associated with different IPF forms.

Summary:

  • Clinical findings include dyspnea, cough, clubbing, and rales, with pulmonary function tests showing restriction and reduced diffusion capacity.
  • Diagnosis requires bronchoalveolar lavage and lung biopsy to assess inflammation and impairment.
  • Treatment involves corticosteroids and immunosuppressants to manage alveolitis and fibrosis, with regular follow-up.

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Impact:

  • Prognosis varies significantly, with median survival over 10 years for the cellular form and 3.2–5.6 years for the hypocellular form without treatment.
  • Anti-inflammatory treatment efficacy is limited, with only 12-30% of patients showing a response.