Pulmonary Hypertension Complicating Mixed Connective Tissue Disease

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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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[Mixed connective tissue disease].

Mixed connective tissue disease deserves to be a distinct disease entity due to the persistent citation of this disease in the literature since the original description by Sharp in 1972, in spite of the presence of several criticisms against the independency of this disease. The characteristic features of mixed connective tissue disease are: 1) the presence of anti-U1snRNP antibody with high ti...

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Pulmonary hypertension not a major feature of early mixed connective tissue disease: a prospective clinicoserological study.

BACKGROUND Mixed connective tissue disease (MCTD) has features common to lupus, scleroderma and myositis with high levels of antibodies to U1 ribonucleoprotein (U1 RNP). Identification of a high incidence of pulmonary artery hypertension (PAH) has changed its prospect. We report the largest series from India. SETTINGS AND DESIGN Rheumatology unit of a tertiary care centre in India; prospectiv...

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Recovery from pulmonary hypertension in an adolescent with mixed connective tissue disease.

This paper describes the case of an 11 year old girl who presented with mixed connective tissue disease which was complicated by the development of pulmonary hypertension. This case is unique with respect to the young age of onset, the serial non-invasive method used to follow the disease process, and the favourable response to treatment with vasodilator and anti-inflammatory drugs.

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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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ژورنال

عنوان ژورنال: Internal Medicine

سال: 1998

ISSN: 0918-2918,1349-7235

DOI: 10.2169/internalmedicine.37.347