Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact.
Mario Naranjo-TovarPaul M HassounPublished in: Diagnostics (Basel, Switzerland) (2021)
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.
Keyphrases
- systemic sclerosis
- pulmonary arterial hypertension
- pulmonary hypertension
- interstitial lung disease
- pulmonary artery
- clinical trial
- end stage renal disease
- ejection fraction
- chronic kidney disease
- newly diagnosed
- peritoneal dialysis
- randomized controlled trial
- cross sectional
- high throughput
- polycyclic aromatic hydrocarbons
- cardiovascular events
- case report
- cardiovascular disease
- risk factors
- adipose tissue
- clinical practice
- cell therapy
- mesenchymal stem cells
- insulin resistance
- coronary artery disease