&lt;p&gt;Management of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis&lt;/p&gt;

Almaaitah, Saja; Highland, Kristin B.; Tonelli, Adriano R.

doi:10.2147/ibpc.s232038

Cited by 30 publications

(22 citation statements)

References 108 publications

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“…Data suggest that 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]. The following annual strategies have demonstrated similar sensitivity, specificity, positive and negative predictive values [49]. The DETECT algorithm combines two steps.…”

Section: Screening For Ssc-pahmentioning

confidence: 99%

“…A decrease in DLCO <60% or >20% in one year in the absence of significant lung volume abnormalities, or an FVC/DLCO percent >1.6 suggests PH [12]. York et al, reported that a DLCO/alveolar volume (Va) of <70% predicted suggested an 18-fold higher risk for developing SSc-PAH within 2.5 years compared with a DLCO/Va >70% [16,49,63].…”

Section: Pulmonary Function Testmentioning

confidence: 99%

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Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact

Naranjo

Hassoun

2021

Diagnostics

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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.

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Section: Screening For Ssc-pahmentioning

confidence: 99%

Section: Pulmonary Function Testmentioning

confidence: 99%

Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact

Naranjo

Hassoun

2021

Diagnostics

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“…The endothelin pathway can be blocked by endothelin receptor antagonists. Ambrisentan, bosentan and macitentan are the three endothelin receptor antagonists (ERA) approved for the treatment of SSc–PAH [ 55 ]. Ambrisentan, the only endothelin-1 selective antagonist, was able to improve 6MWD in connective tissue disease (CTD)-associated PAH patients, even though survival was higher in idiopathic PAH patients, in the Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy Study (ARIES) 1 and 2 trials [ 56 , 57 ].…”

Section: Molecular Pathways Targeted By Pah-specific Therapiesmentioning

confidence: 99%

Pulmonary Hypertension Phenotypes in Systemic Sclerosis: The Right Diagnosis for the Right Treatment

Attanasio

Cuomo

Pirozzi

et al. 2020

IJMS

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Systemic sclerosis is an auto-immune disease characterized by skin involvement that often affects multiple organ systems. Pulmonary hypertension is a common finding that can significantly impact prognosis. Molecular pathophysiological mechanisms underlying pulmonary hypertension in systemic sclerosis can be extremely heterogeneous, leading to distinct clinical phenotypes. In addition, different causes of pulmonary hypertension may overlap within the same patient. Since pulmonary hypertension treatment is very different for each phenotype, it is fundamental to perform an adequate diagnostic work-up to properly and promptly identify the prevalent mechanism underlying pulmonary hypertension in order to start the right therapies. When pulmonary hypertension is caused by a primary vasculopathy of the small pulmonary arteries, treatment with pulmonary vasodilators, often in an initial double-combination regimen, is indicated, aimed at reducing the mortality risk profile. In this review, we describe the different clinical phenotypes of pulmonary hypertension in the scleroderma population and discuss the utility of clinical tools to identify the presence of pulmonary vascular disease. Furthermore, we focus on systemic sclerosis-associated pulmonary arterial hypertension, highlighting the advances in the knowledge of right ventricular dysfunction in this setting and the latest updates in terms of treatment with pulmonary vasodilator drugs.

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“…PAH is a leading cause of mortality in SSc, with a death rate of around 50% at 3 years (4,5). In the last decades, we experienced important advances in the treatment of SSc associated PAH (SSc-PAH); however, despite the availability of effective treatments, mortality remains high and survival curves are worse than in idiopathic PAH (6).…”

Section: Introductionmentioning

confidence: 99%

Cutaneous Iontophoresis of Vasoactive Medications in Patients with Scleroderma- Associated Pulmonary Arterial Hypertension

Ampnti

Almoushref

Naal

et al. 2021

Preprint

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Background:It remains unknown whether the cutaneous microvascular responses are different between patients with scleroderma-associated pulmonary arterial hypertension (SSc-PAH) and SSc without pulmonary hypertension (PH).Methods:We included 59 patients with SSc between March 2013 and September 2019. We divided patients into 4 groups: a) no PH by right heart catheterization (RHC) (n=8), b) no PH by noninvasive screening tests (n=16), c) treatment naïve PAH (n=16) and d) PAH under treatment (n=19). Microvascular studies using laser Doppler flowmetry (LDF) were done immediately after RHC or at the time of an outpatient clinic visit (group b). Results:The median (IQR) age was 59 (54-68) years, and 90% were females. The responses to thermal and post occlusive reactive hyperemia, acetylcholine, and sodium nitroprusside iontophoresis were similar among groups. The microvascular response to treprostinil was more pronounced in SSc patients without PH by screening tests (% change: 340 (214-781)) compared with SSc-PAH (naïve + treatment) (Perfusion Units (PU) % change: 153 (94-255) % [p=0.01]). The response to A-350619 (a sGC activator) was significantly higher in patients with SSc without PH by screening tests (PU % change: 168 (46-1,296)) than those with SSc-PAH (PU % change: 22 (15-57) % [p=0.006]). The % change in PU with A350619 was directly associated with cardiac index and stroke volume index (R: 0.36, p=0.03 and 0.39, p=0.02, respectively). Conclusions:Patients with SSc-PAH have a lower cutaneous microvascular response to a prostacyclin analog treprostinil and the sGC activator A-350619 when compared with patients with SSc and no evidence of PH on screening tests.

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<p>Management of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis</p>

Cited by 30 publications

References 108 publications

Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact

Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact

Pulmonary Hypertension Phenotypes in Systemic Sclerosis: The Right Diagnosis for the Right Treatment

Cutaneous Iontophoresis of Vasoactive Medications in Patients with Scleroderma- Associated Pulmonary Arterial Hypertension

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