Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact

Diagnostics

Mario Naranjo¹  PaulM. Hassoun¹ 

[1] Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University, Baltimore, MD 21287, USA;

关键词: systemic sclerosis;    pulmonary hypertension;    pulmonary arterial hypertension;    pathogenesis;    diagnosis;    management;   

DOI  :  10.3390/diagnostics11050911

来源: DOAJ

【 摘 要 】

Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a catastrophic complication of one of the most common and devastating autoimmune diseases. Once diagnosed, it becomes the leading cause of mortality among this patient population. Screening modalities and risk assessments have been designed and validated by various organizations and societies in order to identify patients early in their disease course and promptly refer them to expert centers for a hemodynamic assessment and formal diagnosis. Moreover, several large multicenter clinical trials have now included patients with SSc-PAH to assess their response to therapy. Despite an improved understanding of the condition and significant advances in supportive and targeted therapy, outcomes have remained far from optimal. Therefore, rigorous phenotyping and search for novel therapies are desperately needed for this devastating condition.

【 授权许可】

Unknown