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DOI: 10.1055/s-0042-104742
Non-CF-Bronchiektasen: Aktuelle therapeutische Entwicklungen
New and Current Concepts of Therapy in Non-CF BronchiectasisPublikationsverlauf
Publikationsdatum:
13. Mai 2016 (online)
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Zusammenfassung
Non-CF (NCF)-Bronchiektasen führen durch chronische Inflammation zu progredienter Zerstörung von Lungenstruktur. Die Erkrankung wird durch verbesserte Diagnostik (HR-CT) zunehmend wahrgenommen, die Prävalenz in Deutschland liegt bei 67/100 000.
Die Qualität der Therapie ist entscheidend von präziser Diagnostik bestimmt. Genetische (Mukoviszidose, Alpha-1-AT-Mangel, Rheumaerkrankungen) und immunologische Ursachen (Immundefekte, allergische bronchopulmonale Aspergillose) sind von idiopathischen NCF-Bronchiektasen zu differenzieren.
Ziel einer Therapie ist die Durchbrechung der entzündlichen Kaskade. Kombiniert werden kontinuierliche Sekretmobilisation durch Physiotherapie, immunmodulierende Verfahren und die bedarfsgerechte antibiotische Therapie. Operative Therapien bleiben speziellen Problemen vorbehalten.
Makrolide vermitteln eine Vielzahl immunmodulierender Wirkungen. Sie reduzieren Symptome und Exazerbationen bei guter Verträglichkeit, sofern kardiale Risiken ausgeschlossen werden. Die Wirkung von Statinen auf NCF-Bronchiektasen ist nicht abschließend untersucht.
Antibiotika werden resistenzgerecht bei Exazerbationen und Erstnachweis von Leitkeimen (Pseudomonas aeruginosa, MRSA) angewendet. Inhalative Antibiotikatherapien werden zunehmende Bedeutung erfahren, derzeit erfolgt die Anwendung nur in begründeten Einzelfällen.
Abstract
Non-CF (NCF)-bronchiectasis is a syndrome of chronic inflammation leading to dilatation of airways and structural lung damage. Improvements of diagnostic procedures increase its perceived frequency. In Germany, recent data suggest a prevalence of 67/100 000.
The outcome of therapeutic interventions is critically related to thorough diagnostic procedures. Genetical or immunological disorders (cystic fibrosis, alpha-1-AT deficiency, immune deficiency syndromes) require treatment options different from idiopathic NCF-bronchiectasis.
Therapy is aimed at suppression of chronic inflammation and includes continuous mobilisation of secretions, immunomodulatory strategies and antibiotic therapy, whenever required. Surgical procedures are limited to specific complications (e. g. destroyed lung after airway obstruction, uncontrolled hemorrhage)
Macrolides show a variety of immunological properties with favourable results in reduction of symptoms and frequency of exacerbations. Longtime tolerance is good, if individual risk factors are excluded.
Antibiotics are given according to resistance patterns in acute exacerbations and in first-time detection of Pseudomonas aeruginosa and MRSA. Inhaled antibiotics for NCF-bronchiectasis will gain importance, depending on future studies. Currently, they are only used in individualized concepts of therapy.
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