Aktuelle Rheumatologie 2020; 45(05): 475-479
DOI: 10.1055/a-1135-8548
Original Article

Clinical Characteristics and Factors Associated with Disease Progression in Chinese Patients with Connective Tissue Disease and Pulmonary Arterial Hypertension

Klinische Merkmale und Einflussfaktoren auf den Krankheitsverlauf bei chinesischen Patienten mit Bindegewebserkrankungen und pulmonal-arterieller Hypertonie
Haichao Zhang
1   Pharmacy, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
,
Xiaoyan Liu xiaoyan
1   Pharmacy, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
› Institutsangaben

Abstract

Objective To clarify the characteristics, survival, and predictors of mortality in Chinese patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD).

Methods This single-cohort study involved 67 CTD-PAH patients who visited Shanghai Renji Hospital between January 2011 and January 2019. Baseline clinical features and hemodynamic parameters were recorded. Logistic regression was used to determine the independent factors associated with mPAP improvement.

Results SLE was the major underlying CTD, affecting 70% of PAH patients, whereas systemic sclerosis was less common (6%). Modern PAH drug use was associated with a favorable outcome and mPAP improvement.

Conclusion The majority of the CTD-PAH population in China suffers from SLE in contrast to the CTD-PAH patients in the USA and Europe. Pulmonary vasodilation treatment has improved the mPAP in these patients.

Zusammenfassung

Zielsetzung Untersuchung der klinischen Merkmale, des Überlebens und der Prädiktoren der Mortalität bei chinesischen Patienten mit pulmonal-arterieller Hypertonie (PAH) in Verbindung mit einer Bindegewebserkrankung (CTD).

Methoden In dieser Einzelkohortenstudie wurden 67 CTD-PAH-Patienten untersucht, die im Zeitraum von Januar 2011 bis Januar 2019 im Shanghai Renji Hospital behandelt wurden. Dabei wurden klinische Ausgangsmerkmale und hämodynamische Parameter erfasst. Mittels logistischer Regression wurden die mit einer Verbesserung des mPAP einhergehenden unabhängigen Faktoren ermittelt.

Ergebnis SLE war die Hauptursache für CTD und betraf 70% der PAH-Patienten, wohingegen die systemische Sklerose seltener auftrat (6%). Die Anwendung einer modernen PAH-Medikation führte zu günstigen Ergebnissen und einer Verbesserung des mPAP.

Schlussfolgerung Im Gegensatz zu den CTD-PAH-Patienten in den USA und Europa leidet die Mehrheit der CTD-PAH-Population in China an SLE. Die pulmonale Vasodilatation führte bei diesen Patienten zu einer Verbesserung des mPAP.



Publikationsverlauf

Artikel online veröffentlicht:
07. April 2020

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