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Interstitielle Lungenerkrankungen

Differentialdiagnose und Therapie

Interstitial lung diseases

Differential diagnostics and therapy

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Zusammenfassung

Interstitielle Lungenerkrankungen (ILE) sind eine ätiologische heterogene Gruppe von Erkrankungen, denen ein Befall peripherer bronchoalveolärer Strukturen gemeinsam ist. Hierbei kommt es zu entzündlichen, granulomatösen und fibrosierenden Prozessen in variabler Ausprägung. Klinisch dominieren Dyspnoe und trockener Husten sowie häufig inspiratorisches Knisterrasseln in der Auskultation. Funktionelle Einschränkungen umfassen eine Restriktion und Gasaustauschstörung sowie verminderte Compliance und Hypoxämie unter Belastung. Die Diagnosestellung beruht neben den klinischen Befunden auf dem Nachweis radiologischer und/oder histologischer Veränderungen des Lungenparenchyms. Die Pharmakotherapie der ILE umfasst, je nach Grunderkrankung, antiinflammatorische, antioxidative und antifibrotische Behandlungsansätze. Ergänzend sind die Langzeitsauerstofftherapie, die pulmonale Rehabilitation sowie die Behandlung von Komplikationen und Begleiterkrankungen von Bedeutung. In progredienten und therapierefraktären Fällen stellt die Lungentransplantation eine Behandlungsoption dar.

Abstract

Interstitial lung diseases (ILD) are a heterogeneous group of diseases which have in common manifestations of the disease process in the distal airways and alveolar-capillary membrane. Clinical presentation comprises dyspnea, dry cough and inspiratory crackles on auscultation. Lung physiology is characterized by restrictive impairment and disturbed gas exchange as well as decreased compliance and hypoxemia. Besides clinical findings the diagnosis is based on radiological and/or histological evidence of parenchymal lung disease. Pharmacotherapy of ILD utilizes anti-inflammatory, anti-oxidative and anti-fibrotic approaches, depending on the underlying disease. In addition, long-term oxygen therapy and pulmonary rehabilitation as well as treatment of complications and co-morbidities are relevant and necessary treatment measures. In progressive refractory cases lung transplantation is a treatment option.

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehung hin: Honorare für Vorträge und/oder Beratertätigkeit von Actelion, Almirall, Bayer, Boehringer, Gilead, GSK, InterMune, Lilly, Novartis, Nycomed, Pfizer, Takeda. Forschungsförderung von Actelion.

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  1. Asklepios Fachkliniken München-Gauting und Medizinische Klinik und Poliklinik V , Klinikum der Universität München, Robert-Koch-Allee 2, 82131, Gauting, Deutschland

    J. Behr

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Behr, J. Interstitielle Lungenerkrankungen. Pneumologe 10, 45–58 (2013). https://doi.org/10.1007/s10405-012-0613-z

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  • DOI: https://doi.org/10.1007/s10405-012-0613-z

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