Abstrakt

The idiopathic pulmonary fibrosis (IPF) is a special form of chronic and progressively trending fibrosing interstitial pneumonia with unknown etiology. Tobacco smoke, contact with metal, wood dust and birds, activities in agriculture, in the stone working industry, in hairdressing, as well as viral infections are associated with the formation of IPF. In Western countries, the incidence of IPF is between 6.8 and 17.4 per 100,000 per year and the prevalence between 2 up to 42.7. It occurs mainly in men at the age between 60 and 80. For diagnosis the chest HRCT is considered as the gold standard for detecting symmetrically existing subpleural and basal honeycomb structures. The average survival rate is 2 – 3 years. Therapeutically, the previously applied corticosteroids in combination with azathioprine and N-acetylcysteine and anticoagulants are no longer recommended. In 2011, the European Medicines Agency approved pirfenidone, an antifibrotic pyridone compound, for the treatment of patients with mild-to-moderate IPF. Long-term oxygen therapy with proven resting hypoxemia is recommended. The indication for a lung transplant should be considered at an early stage.

Autoreninformation

Autoren

• H. Schweisfurth

Abteilungen

• Pulmologisches Forschungsinstitut – IPR, Cottbus

Adresse

Prof. Dr. med. H. Schweisfurth
Pulmologisches Forschungsinstitut – IPR
Walther-Rathenau-Straße 11
D–03044 Cottbus
Email: [email protected]

Citation