Pulmonary Hypertension Associated with Connective Tissue Disease

作者: Stephen C. Mathai , Laura K. Hummers

DOI: 10.1007/978-1-4939-0770-0_11

关键词: Obstructive lung diseasePulmonary hypertensionRespiratory diseasePopulationInterstitial lung diseaseInternal medicineCardiologyMedicineLung transplantationPulmonary wedge pressureConnective tissue disease

摘要: Pulmonary hypertension (PH) commonly complicates connective tissue disease and is associated with increased morbidity mortality in this population. The incidence of PH varies widely between diseases; patients systemic sclerosis are most likely to develop PH. Several different types can present CTD, including related left heart disease, respiratory such as interstitial lung or chronic obstructive thromboembolic renal disease. Importantly, CTD at risk for developing pulmonary arterial (PAH), which without any the aforementioned co-morbidities. However, despite development PH, PAH may benefit from specific vasodilator therapy thus, an important group identify. Unfortunately, response general less robust survival worse CTD-associated than other patient populations PAH. Still, early diagnosis treatment if indicated, allow institution appropriate referral transplantation.

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