Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact

Naranjo, Mario; Hassoun, Paul M.

doi:10.3390/diagnostics11050911

Cited by 23 publications

(26 citation statements)

References 110 publications

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“…Scleroderma-associated PAH (SSc-PAH) was the most common cause of PAH in our cohort due to referral bias, and the second most commonly identified PAH aetiology in most PH registries. It is the leading cause of death in SSc [ 5 , 38 , 39 ]. Dual upfront combination therapy has certainly been a significant therapeutic advance in this disease and offers new hopes for this population [ 7 , 40 ].…”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary arterial hypertension (PAH) remains a disease with significant morbidity and mortality despite a plethora of modern medications which have improved general outcomes [ 1 – 3 ]. It is a complex disorder of the pulmonary vasculature leading to progressive adaptive and maladaptive morphological and functional changes of the right ventricle (RV), with RV failure being the single most important determinant of survival [ 4 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

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Causes and outcomes of ICU hospitalisations in patients with pulmonary arterial hypertension

et al. 2022

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RationalePulmonary arterial hypertension (PAH) is a rare disease characterised by limited survival despite remarkable improvements in therapy. The causes, clinical burden, and outcomes of patients admitted to the intensive care unit (ICU) remain poorly characterised.ObjectivesTo describe patient characteristics, causes of ICU hospitalisation, and risk factors for ICU and 1-year mortality.MethodsData from patients enrolled in the Johns Hopkins Pulmonary Hypertension Registry were analyzed for the period between January 2010 to December 2020. Clinical, functional, hemodynamic, and laboratory data were collected.Measurements and Main Results102 adult patients with 155 consecutive ICU hospitalisations were included. The leading causes for admission were right heart failure (RHF, 53.3%), infection (17.4%), and arrhythmia (11.0%). ICU mortality was 27.1%. Mortality risk factors included Na<136 mEq mL−1 (OR: 3.10, 95% CI: 1.41–6.82), elevated proBNP (OR: 1.75, 95% CI: 1.03–2.98), hyperbilirubinemia (OR: 1.40, 95% CI: 1.09–1.80), hyperlactemia (OR: 1.42, 95% CI: 1.05–1.93), and need for vasopressors/inotropes (OR: 5.29, 95% CI: 2.28–12.28), mechanical ventilation (OR: 3.76, 95% CI: 1.63–8.76), and renal replacement therapy (OR: 5.57, 95% CI: 1.25–24.76). Mortality rates at 3, 6 and 12 months were 17.5%, 27.6%, and 39.0%, respectively. Connective tissue disease-associated PAH has lower 1-year survival compared to idiopathic PAH (51.4% versus 79.8%, log-rank test p=0.019).ConclusionsRHF is the most common cause for ICU admission. In-hospital and 1-year mortality remain exceedingly high despite improved ICU care. Recognising specific risk factors on admission can help identifying patients at risk for poor outcomes.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Causes and outcomes of ICU hospitalisations in patients with pulmonary arterial hypertension

et al. 2022

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“…PAH may occur in SSc patients with or without accompanying interstitial lung disease and/or digital ulcers ( 71 , 72 ). The endothelin system is believed to play a central role in SSc-PAH, and SSc-PAH patients benefit from ERA treatment ( 24 , 71 , 72 ). ERAs are further indicated to treat SSc-related digital ulcers ( 72 ).…”

Section: Discussionmentioning

confidence: 99%

Endothelin B Receptor Immunodynamics in Pulmonary Arterial Hypertension

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et al. 2022

Front. Immunol.

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IntroductionInflammation is a major pathological feature of pulmonary arterial hypertension (PAH), particularly in the context of inflammatory conditions such as systemic sclerosis (SSc). The endothelin system and anti-endothelin A receptor (ETA) autoantibodies have been implicated in the pathogenesis of PAH, and endothelin receptor antagonists are routinely used treatments for PAH. However, immunological functions of the endothelin B receptor (ETB) remain obscure.MethodsSerum levels of anti-ETB receptor autoantibodies were quantified in healthy donors and SSc patients with or without PAH. Age-dependent effects of overexpression of prepro-endothelin-1 or ETB deficiency on pulmonary inflammation and the cardiovascular system were studied in mice. Rescued ETB-deficient mice (ETB-/-) were used to prevent congenital Hirschsprung disease. The effects of pulmonary T-helper type 2 (Th2) inflammation on PAH-associated pathologies were analyzed in ETB-/- mice. Pulmonary vascular hemodynamics were investigated in isolated perfused mouse lungs. Hearts were assessed for right ventricular hypertrophy. Pulmonary inflammation and collagen deposition were assessed via lung microscopy and bronchoalveolar lavage fluid analyses.ResultsAnti-ETB autoantibody levels were elevated in patients with PAH secondary to SSc. Both overexpression of prepro-endothelin-1 and rescued ETB deficiency led to pulmonary hypertension, pulmonary vascular hyperresponsiveness, and right ventricular hypertrophy with accompanying lymphocytic alveolitis. Marked perivascular lymphocytic infiltrates were exclusively found in ETB-/- mice. Following induction of pulmonary Th2 inflammation, PAH-associated pathologies and perivascular collagen deposition were aggravated in ETB-/- mice.ConclusionThis study provides evidence for an anti-inflammatory role of ETB. ETB seems to have protective effects on Th2-evoked pathologies of the cardiovascular system. Anti-ETB autoantibodies may modulate ETB-mediated immune homeostasis.

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“…Right heart catheterization (RHC) is the best technique for PAH diagnosis [10][11][12][13][14][15]. The RHC provides useful information on the degree of hemodynamic damage, determines response to treatments and establishes prognosis of PAH [19,[33][34][35][36][37][38][39][40][41][42][43]. The RCH evaluation is recommended when patients have an intermediate or high risk of developing PH, based on ETT evaluation, defined as a TRV peak >2.8 ms −1 , or a TRV <2.8 ms −1 (or not measurable) with other variables suggestive of PH [38].…”

Section: Right Heart Catheterizationmentioning

confidence: 99%

An Overview of Different Techniques for Improving the Treatment of Pulmonary Hypertension Secondary in Systemic Sclerosis Patients

et al. 2022

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In systemic sclerosis (SSc) mortality is mainly linked to lung involvement which is characterized by interstitial lung disease (ILD) and pulmonary hypertension (PH). In SSc, PH may be due to different etiologies, including ILD, chronic thromboembolic disease, pulmonary veno-occlusive disease, and pulmonary arterial hypertension (PAH). The main tool to screen PAH is transthoracic echocardiography (TTE), which has a sensitivity of 90%, even if definitive diagnosis should be confirmed by right heart catheterization (RHC). The radiological evaluation (i.e., HRTC) plays an important role in defining the possible causes and in monitoring the evolution of lung damage. For PAH, identifying individuals who have borderline elevation of pulmonary arterial pressure needs to be appropriately managed and followed. In the past few years, the strategy for the management of PAH has significantly evolved and new trials are underway to test other therapies. This review provides an overview of the tools to evaluate PAH in SSc patients and on treatment options for these patients.

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Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact

Cited by 23 publications

References 110 publications

Causes and outcomes of ICU hospitalisations in patients with pulmonary arterial hypertension

Causes and outcomes of ICU hospitalisations in patients with pulmonary arterial hypertension

Endothelin B Receptor Immunodynamics in Pulmonary Arterial Hypertension

An Overview of Different Techniques for Improving the Treatment of Pulmonary Hypertension Secondary in Systemic Sclerosis Patients

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