Abstract
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.
Highlights
Systemic sclerosis (SSc) is a chronic multisystem disease characterized by fibrosis, excessive collagen deposition within the skin and internal organs, chronic inflammation, autoimmune dysregulation, and microvascular endothelial dysfunction that leads to multiorgan dysfunction [1,2]
This study identifies important risk factors associated with the development of pulmonary hypertension (PH) and PAH, including diffusing capacity of carbon monoxide (DLCO) 1.6, and/or right ventricular systolic pressure (RVSP)
Data suggest that sclerosis-associated pulmonary arterial hypertension (SSc-PAH) patients who are diagnosed and treated early as a result of screening have improved survival as compared to patients diagnosed following clinical suspicion [47,48]
Summary
Systemic sclerosis (SSc) is a chronic multisystem disease characterized by fibrosis, excessive collagen deposition within the skin and internal organs, chronic inflammation, autoimmune dysregulation, and microvascular endothelial dysfunction that leads to multiorgan dysfunction [1,2]. Limited cutaneous SSc (lcSSc) involving the distal skin of the extremities and the face; and diffuse SSc (dcSSc) involving large areas of skin in the proximal aspect of the extremities and multiple organs [3]. Both forms, are systemic diseases associated with significant morbidity and mortality. Forms of pulmonary hypertension (PH) other than PAH can afflict SSc patients, including PH related to left heart disease, interstitial lung disease (ILD), chronic thromboembolism, and pulmonary venous occlusive disease, which further complicates diagnosis and management [4]. SSc, this review will focus essentially on SSc-associated pulmonary arterial hypertension (SSc-PAH) for which treatment is available.
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