1. Pulmonary Hypertension Associated with Connective Tissue Diseases

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Associated pulmonary arterial hypertension in connective tissue diseases.

In recent years, major advances have been achieved in the understanding of pulmonary arterial hypertension (PAH) patho-physiology. Associated pulmonary arterial hypertension (APAH) can occur in a variety of other conditions and circumstances including a number of systemic autoimmune diseases. As with PAH in general, clinical symptoms of APAH in systemic autoimmune diseases are unspecific. In ad...

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Pulmonary arterial hypertension in connective tissue diseases.

Pulmonary arterial hypertension (PAH) is an entity that is known to complicate connective tissue diseases (CTD). PAH in CTD is a very important diagnosis which greatly affects treatment and prognosis. The most commonly affected CTD is scleroderma, although lupus, inflammatory myopathies such as poly and dermatomyositis, and mixed CTD are also associated with PAH. The manifestations of PAH have ...

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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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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: Nihon Naika Gakkai Zasshi

سال: 2013

ISSN: 0021-5384,1883-2083

DOI: 10.2169/naika.102.2613