FRI0254 SERUM IL-17 AND IL-21 AFFECT THE HEMODYNAMICS IN CONNECTIVE TISSUE DISEASE ASSOCIATED-PULMONARY HYPERTENSION

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Connective tissue disease-associated pulmonary arterial hypertension

Although rare in its idiopathic form, pulmonary arterial hypertension (PAH) is not uncommon in association with various associated medical conditions, most notably connective tissue disease (CTD). In particular, it develops in approximately 10% of patients with systemic sclerosis and so these patients are increasingly screened to enable early detection. The response of patients with systemic sc...

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Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients.

We sought to investigate the characteristics, survival and risk factors for mortality in Chinese patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (APAH) in modern therapy era. 129 consecutive adult patients who visited one of three referral centres in China with a diagnosis of CTD-APAH confirmed by right heart catheterisation during the previous 5 years w...

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Connective tissue disease-associated pulmonary arterial hypertension: "Beijing style".

Patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (APAH) experience poorer outcomes than those with idiopathic PAH (IPAH) [1–6]. In the USA, patients with systemic sclerosis (SSc) are most commonly affected by CTD-APAH and have the worst survival [1]. Similarly, 74% of a large cohort with CTD-APAH in the UK had SSc, and these patients had poorer survival t...

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Sildenafil for pulmonary arterial hypertension associated with connective tissue disease.

OBJECTIVE Pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD) is difficult to manage, and has a poor prognosis. The phosphodiesterase-5 inhibitor sildenafil citrate enhances vasodilatation, has antiproliferative effects, and is effective in the treatment of PAH. We examined the efficacy and safety of oral sildenafil in patients with PAH-CTD. METHODS In a 12-wee...

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[Treatment of pulmonary hypertension associated with connective tissue disease].

Pulmonary hypertension is the most ominous complication of connective tissue diseases and its treatment has been a big challenge to clinicians. Vasodilators including epoprostenol (prostacyclin), bosentan (endothelin-receptor antagonist), and a sildenafil (phosphodiesterase type 5 inhibitor) have recently become available in Japan. These vasodilators may improve exercise tolerance, hemodynamics...

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ژورنال

عنوان ژورنال: Annals of the Rheumatic Diseases

سال: 2020

ISSN: 0003-4967,1468-2060

DOI: 10.1136/annrheumdis-2020-eular.5537