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Behr, Jürgen (2021): Interstitielle Lungenerkrankungen. In: Pneumologe, Bd. 18, Nr. 2: S. 129-137

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Abstract

Interstitial lung diseases represent a heterogeneous group of inflammatory and/or fibrosing diseases, which are primarily manifested in peripheral bronchioalveolar structures and lead to a mostly progressive impairment of pulmonary gas exchange and loss of lung volume. In addition to the basic clinical diagnostic procedures, making a diagnosis usually necessitates comprehensive lung function diagnostic tests, high-resolution thin layer computed tomography (native) of the lungs and a transbronchial lung (cryo)biopsy. Depending on the underlying disease and the dominant pathogenesis, immunomodulatory drugs or antifibrotic drugs are used for treatment. Progressive fibrosing interstitial lung disease (PF-ILD) is now newly described as a uniform phenotype, which benefits from an antifibrotic treatment, independent of the original underlying disease.

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