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Akut-auf-chronisches respiratorisches Versagen bei interstitiellen Pneumonien

Acute on chronic respiratory failure in interstitial pneumonias

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Zusammenfassung

Hintergrund

Die akute Exazerbation einer idiopathischen interstitiellen Pneumonie wie auch von interstitiellen Pneumonien im Rahmen von Systemerkrankungen ist mit einer hohen Mortalität verbunden.

Diagnostik

Differenzialdiagnostisch gilt es diese von respiratorischen Verschlechterungen einer interstitiellen Pneumonie als Folge von Infektionen, Lungenembolien, Linksherzerkrankungen und Medikamentennebenwirkungen abzugrenzen, da letztere kausal therapiert werden können. Der Umfang und die Invasivität der Diagnostik müssen sich am klinischen Zustand des Patienten orientieren. Eine rasche Diagnostik ist wichtig, damit eine Therapie (Antibiose, Antikoagulation, Immunsuppresion) im Fall behandelbarer Exazerbationsursachen zeitnah eingeleitet werden kann.

Stellenwert der Beatmung

Die Prognose für invasiv beatmete Patienten mit akuter Exazerbation einer interstitiellen Pneumonie ist schlecht. Für die primäre akute Exazerbation einer idiopathischen pulmonalen Fibrose (IPF) kann keine generelle Empfehlung zur invasiven oder nichtinvasiven Beatmung ausgesprochen werden. Bei akuter Exazerbation einer anderen Form der interstitiellen Pneumonie, sei sie idiopathisch oder im Rahmen einer Systemerkrankung, oder bei Vorliegen einer sekundären Exazerbation einer idiopathischen interstitiellen Pneumonie mit behandelbarer Ursache ist der Stellenwert der Beatmung differenzierter zu betrachten.

Lungentransplantation

Insbesondere bei jüngeren Patienten mit prognostisch ungünstigen interstitiellen Lungenerkrankungen sollte frühzeitig und noch vor einer Exazerbation eine Entscheidung über die Indikation zur Lungentransplantation getroffen werden. Bei akuter Exazerbation während der Wartezeit auf eine Lungentransplantation sind mit dem Transplantationszentrum extrakorporale Verfahren als Bridging-to-transplant-Maßnahm zu klären. Bei infauster Prognose sind palliative Therapiekonzepte einzusetzen.

Abstract

Background

Acute exacerbations of idiopathic interstitial pneumonias and exacerbations of pleuropulmonary disease in connective tissue diseases are associated with a high mortality.

Diagnostic procedures

They have to be differentiated from respiratory failure in patients with interstitial lung disease as a result of infections, pulmonary embolism, cardiac failure and drug toxicity, because the latter can be treated causally. The extent and the invasivity of diagnostic procedures have to be adopted to the patient’s situation. A rapid diagnosis is important in order to initiate therapies (antibiotics, anticoagulation, immunosuppression) in treatable causes of exacerbations.

Importance of ventilation

The prognosis for patients who are under invasive mechanical ventilation in acute exacerbations of interstitial lung disease is poor. Especially in acute exacerbations of idiopathic pulmonary fibrosis (IPF), there is no general recommendation for either invasive or for noninvasive ventilation. In acute exacerbations of other subtypes of interstitial pneumonia, either idiopathic or as a pulmonary manifestation of connective tissue disease, and in all secondary exacerbations of idiopathic interstitial pneumonias which offer further treatment options, the decision about mechanical ventilation requires a differentiated assessment.

Lung transplantation

In younger patients with interstitial lung disease and a progressive disease, indication for lung transplantation should be made early and before an exacerbation. If patients listed for lung transplantation experience an acute exacerbation, bridging-to-transplant has to be discussed with the transplant unit. In cases without further causal treatment options palliative care must be initiated.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. M. Westhoff gibt an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Klinik für Pneumologie, Schlaf- und Beatmungsmedizin, Lungenklinik Hemer – Zentrum für Pneumologie und Thoraxchirurgie, Theo-Funccius-Str. 1, 58675, Hemer, Deutschland

    M. Westhoff

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Westhoff, M. Akut-auf-chronisches respiratorisches Versagen bei interstitiellen Pneumonien. Med Klin Intensivmed Notfmed 110, 188–196 (2015). https://doi.org/10.1007/s00063-014-0388-6

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