Differenzialdiagnostik der pulmonalen Hypertonie am Beispiel der Kollagenose assoziierten PAH im Kontext chronischer Lungen- und Linksherzerkrankungen

Zusammenfassung

Die pulmonale Hypertonie (PH) ist eine regelmäßige Komplikation von Kollagenosen und wird zudem im Zusammenhang mit Lungen- und Linksherzerkrankungen diagnostiziert.

Die korrekte Differenzierung und Klassifikation der PH ist essenziell, um Therapien möglichst zielgerichtet einsetzen zu können und ein Vorenthalten von wichtigen Therapien ebenso zu vermeiden wie eine Übertherapie.

Bei den Kollagenosen umfasst die Differenzialdiagnostik und Klassifikation die Differenzierung innerhalb der assoziierten pulmonalarteriellen Hypertonie (APAH) Gruppe 1, aber auch, Klassifikationsgruppen übergreifend, die Gruppe 2 (mit führender linkskardialer Beteiligung), Gruppe 3 (mit führender parenchymatöser Lungenerkrankung), Gruppe 4 (chronisch thromboembolische PH) und letztlich die Gruppe 5 (z. B. bei terminaler Niereninsuffizienz und Dialysepflicht).

Ähnliches trifft auch auf die zunehmend älteren Patienten zu, die mit einer PAH diagnostiziert werden und Komorbiditäten aufweisen, die entweder selbst eine PH hervorrufen können oder als Komorbiditäten einer echten PAH auftreten.

Die abnehmende Trennschärfe zwischen den einzelnen PH-Gruppen erschwert die korrekte Klassifikation und stellt eine große klinische Herausforderung dar.

Auf dem Boden unterschiedlicher klinischer Szenarien wird im vorliegenden Artikel die Differenzialdiagnostik der PH sowohl bei Kollagenosen als auch bei chronischen Linksherz- und Lungenerkrankungen aufgearbeitet und objektive Kriterien vorgestellt, die diese Differenzierung erleichtern.

Abstract

Pulmonary hypertension (PH) can be diagnosed in the context of connective tissue diseases (CTD) as well as in elderly patients with multiple comorbidities. A correct clinical differential diagnosis and classification is essential before adequate therapeutic decisions can be made. Differential diagnosis of PH in CTD comprises associated pulmonary arterial hypertension (APAH), group 2 or 3 PH (PH arising from left heart or chronic lung disease), chronic thromboembolic PH (PH) and group 5 (e. g. in the context of terminal renal insufficiency). This is also true of elderly patients in whom the decision has to be made if the increasing number of coincident diseases lead to PH or have to be interpreted as comorbidities. In this manuscript, the differential diagnosis of PH is elucidated, focusing on CTD, in the context of left heart disease and chronic lung disease. Furthermore, criteria are presented facilitating an objective approach in this context.

Publication History

Received: 17 June 2020

Accepted: 21 June 2021

Article published online:
12 February 2021

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