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Zeitschrift für Rheumatologie

, Volume 77, Issue 3, pp 219–230 | Cite as

Pulmonale Hypertonie bei Kollagenosen

  • E. I. Schwarz
  • S. Ulrich
CME

Zusammenfassung

Die pulmonal-arterielle Hypertonie (PAH) ist eine schwerwiegende Folge der systemischen Sklerose (SSc), und Screening auf PAH ist maßgebend, um eine frühzeitige spezifische Therapie zu initiieren und damit die Krankheitsprogression zu verlangsamen oder gar aufzuhalten. Patienten, die in frühen Krankheitsstadien diagnostiziert werden, haben eine bessere Prognose, und daher ist eine frühzeitige Therapieeinleitung höchstwahrscheinlich prognostisch günstig. Die PAH kann in allen Stadien und Subtypen der SSc vorkommen. Ein regelmäßiges Screening (Echokardiographie, Diffusionskapazität, BNP [„brain natriuretic peptide“] und 6‑min-Gehtest) wird empfohlen, da PAH-Symptome unspezifisch sind, häufig spät auftreten und effiziente Therapieoptionen zur Verfügung stehen. Bei SSc-Patienten mit Verdacht auf PAH sollte die Diagnose mittels Rechtsherzkatheter bestätigt werden. Die Betreuung von Patienten mit Kollagenose-assoziierter PAH oder dem Risiko für die Entwicklung einer solchen sollte interdisziplinär zwischen Rheumatologen und auf pulmonale Hypertonie spezialisierten Pneumologen und Kardiologen erfolgen.

Schlüsselwörter

Rechtsherzkatheter Pulmonale Hypertonie Lungenfibrose Systemische Sklerose Screening 

Pulmonary hypertension in connective tissue disease

Abstract

Pulmonary arterial hypertension (PAH) is a major complication of systemic sclerosis (SSc) and screening is recommended for a timely initiation of disease-targeted drug therapy to modify disease progression. Patients with SSc-PAH have a better prognosis when detected and treated early. The PAH can occur in all disease stages and subsets of SSc. Regular screening tests, which are indicative for PAH, e.g. echocardiography, diffusion capacity, brain natriuretic protein (BNP) and a 6-min walking test, are recommended to enhance the suspicion, since clinical symptoms are unspecific and occur late in the course of PAH. In patients with suspected PAH, the diagnosis should be confirmed by right heart catheterization. A multidisciplinary approach in expert centres including rheumatologists and respiratory physicians and cardiologists specialized in pulmonary hypertension is mandatory for management of patients with SSc at risk for or with manifest pulmonary arterial hypertension.

Keywords

Right heart catheterization Pulmonary hypertension Pulmonary fibrosis Systemic sclerosis Screening 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

E.I. Schwarz und S. Ulrich geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren. Alle Patienten, die über Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts zu identifizieren sind, haben hierzu ihre schriftliche Einwilligung gegeben. Im Falle von nicht mündigen Patienten liegt die Einwilligung eines Erziehungsberechtigten oder des gesetzlich bestellten Betreuers vor.

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Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2018

Authors and Affiliations

  1. 1.Klinik für Pneumologie und Sprechstunde für Pulmonale HypertonieUniversitätsspital ZürichZürichSchweiz
  2. 2.Zürcher Zentrum für Integrative Humanphysiologie (ZIHP)Universität ZürichZürichSchweiz

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