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Abstract

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Article in Japanese

Topics Series Pulmonary circulation and lung injury

Pulmonary hypertension as one of vascular complications in patients with systemic sclerosis

Masataka Kuwana 

Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine

ABSTRACT

Pulmonary hypertension (PH) is caused by various underlying pathogenesis. PH associated with systemic sclerosis (SSc) remains a unique subgroup with poor prognosis even in the modern treatment era. SSc-PH often has complex pathophysiologies consisting of pulmonary arterial hypertension, pulmonary veno-occlusive disease, PH left heart disease, and PH because of lung diseases and/or hypoxia. Current pulmonary vasodilators have been confirmed to be effective for pulmonary arterial hypertension alone, and they often unmask or worsen other forms of PH. Since it is difficult to fully assess mechanisms underlying SSc-PH before introduction of the treatment, in clinical practice it is important to monitor clinical and hemodynamic parameters frequently and to modify treatment regimens based on the underlying pathogenic process.

KEYWORDS

Systemic sclerosis  Pulmonary hypertension  Myocardial diastolic dysfunction  Interstitial lung disease 

AJRS, 3(4): 498-504, 2014

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