Genetic Background of Hypertension in Connective Tissue Diseases

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 ...

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...

Treatment of pulmonary hypertension secondary to connective tissue diseases.

Pulmonary hypertension has been identified as one of the well known life threatening complications of connective tissue diseases. In patients with connective tissue diseases pulmonary hypertension occurs with little or no evidence of parenchymal lung disease. As in primary pulmonary hypertension, the pathophysiology is unknown but the clinical course and pathological findings are similar. Scler...

Connective tissue diseases.

Undiagnosed connective tissue diseases

Among different subgroups of pulmonary arterial hypertension (PAH), those associated with connective tissue diseases (CTDs) have distinct hemodynamic and prognostic features; a correct etiologic diagnosis is thus mandatory.To estimate frequency and prognosis of previously undiagnosed CTDs in a suspect idiopathic (i) PAH cohort.Consecutive patients with PAH confirmed by right heart catheterizati...