Pulmonary hypertension in systemic sclerosis: different phenotypes

Launay, David; Sobanski, Vincent; Hachulla, É.; Humbert, Marc

doi:10.1183/16000617.0056-2017

Cited by 111 publications

(105 citation statements)

References 39 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In clinical practice, it may be challenging to classify patients with SSc in a discrete PH group because of complex and frequently overlapping clinical features. Indeed, in the setting of pre-capillary PH, SSc patients may present with no, limited, or extensive ILD and a wide range of hemodynamic severity [ 9 – 11 ]. However, classification of these patients remains meaningful, as medical therapies are dependent on it.…”

Section: Introductionmentioning

confidence: 99%

Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis

et al. 2018

Self Cite

View full text Add to dashboard Cite

Pre-capillary pulmonary hypertension (PH) in systemic sclerosis (SSc) is a heterogeneous condition with an overall bad prognosis. The objective of this study was to identify and characterize homogeneous phenotypes by a cluster analysis in SSc patients with PH. Patients were identified from two prospective cohorts from the US and France. Clinical, pulmonary function, high-resolution chest tomography, hemodynamic and survival data were extracted. We performed cluster analysis using the k-means method and compared survival between clusters using Cox regression analysis. Cluster analysis of 200 patients identified four homogenous phenotypes. Cluster C1 included patients with mild to moderate risk pulmonary arterial hypertension (PAH) with limited or no interstitial lung disease (ILD) and low DLCO with a 3-year survival of 81.5% (95% CI: 71.4–88.2). C2 had pre-capillary PH due to extensive ILD and worse 3-year survival compared to C1 (adjusted hazard ratio [HR] 3.14; 95% CI 1.66–5.94; p = 0.0004). C3 had severe PAH and a trend towards worse survival (HR 2.53; 95% CI 0.99–6.49; p = 0.052). Cluster C4 and C1 were similar with no difference in survival (HR 0.65; 95% CI 0.19–2.27, p = 0.507) but with a higher DLCO in C4. PH in SSc can be characterized into distinct clusters that differ in prognosis.

show abstract

Section: Introductionmentioning

confidence: 99%

Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis

et al. 2018

Self Cite

View full text Add to dashboard Cite

show abstract

“…It is suggested that PH associated with the extensive form of SSc-ILD (>20% fibrosis on HRCT or forced vital capacity (FVC) <70% in indeterminate HRCT) could be classified as group 3. 15 Although the etiology of PH is associated with extent of lung disease, the mPAP does not seem to correlate with extent of fibrosis on imaging or the forced vital capacity. 22,24 In patients with SSc-ILD, a diffusion capacity (DLCO) <40% of predicted or a reduction in DLCO out of proportion to FVC (FVC/ DLCO ratio >1.6) also suggests the presence of pulmonary vascular disease.…”

Section: Types Of Pulmonary Hypertension In Systemic Sclerosismentioning

confidence: 98%

“…Due to the systemic nature of SSc, an overlap between more than one type of PH is common (Table 1 and Figure 1), making the identification of the predominant type of PH not always straightforward, 15 and complicating the management of these patients.…”

Section: Types Of Pulmonary Hypertension In Systemic Sclerosismentioning

confidence: 99%

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

Almaaitah¹,

Highland²,

Tonelli³

2020

IBPC

View full text Add to dashboard Cite

Systemic sclerosis (SSc) is a rare and complex immune-mediated connective tissue disease characterized by multi-organ fibrosis and dysfunction. Systemic sclerosisassociated pulmonary arterial hypertension (SSc-PAH) is a leading cause of death in this population. Pulmonary arterial hypertension (PAH) can coexist with other forms of pulmonary hypertension in SSc, including pulmonary hypertension related to left heart disease, interstitial lung disease, chronic thromboembolism and pulmonary venous occlusive disease, which further complicates diagnosis and management. Available pulmonary arterial hypertension therapies target the nitric oxide, endothelin and prostacyclin pathways. These therapies have been studied in SSc-PAH in addition to idiopathic PAH, often with different treatment responses. In this article, we discuss the management as well as the treatment options for patients with SSc-PAH.

show abstract

“…Chronic lung diseases including pulmonary fibrosis lead to the elevation of pulmonary artery pressure and right heart failure [6]. CTD-associated PH is known to originate, by different pathogenesis, from PH due to lung disease in which hypoxia induces vascular change [7]. In CTD-associated PH, autoimmune response mediates vascular hypertrophy and fibrosis in pulmonary artery and it advances to a status without hypoxia.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary Hypertension in Connective Tissue Disease is Associated with the New York Heart Association Functional Class and Forced Vital Capacity, But Not with Interstitial Lung Disease

et al. 2018

View full text Add to dashboard Cite

Objective. Pulmonary hypertension (PH) develops frequently in connective tissue diseases (CTD) and is an important prognostic factor. The aim of this study was to assess the prevalence of PH in patients with CTD by non-invasive echocardiography and analyze the potential biomarkers for helping to detect PH. Methods. All Korean patients with CTD who had dyspnea on exertion or interstitial lung disease (ILD) were screened for PH with echocardiography and clinical data were collected from four hospitals. Results. Among 196 patients with CTD, 108 (55.1%) had ILD and 21 had PH defined as ＞40 mmHg. Of the 21 patients with PH, 10, 4, and 3 patients had systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease, respectively. There was no difference in the incidence of PH according to the presence of ILD; 12 patients (11.1%) with ILD had PH and 9 patients (10.2%) without ILD had PH. The results of the pulmonary function test, total cholesterol, red cell volume distribution width, alkaline phosphatase, and the New York Heart Association (NYHA) functional class III or IV differed significantly according to the presence of PH. In multiple regression analysis, NYHA functional class III or IV (odd ratio

show abstract

Pulmonary hypertension in systemic sclerosis: different phenotypes

Abstract: @ERSpublicationsPulmonary hypertension in systemic sclerosis is heterogeneous with various possible mechanisms

Cited by 111 publications

References 39 publications

Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis

Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis

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

Pulmonary Hypertension in Connective Tissue Disease is Associated with the New York Heart Association Functional Class and Forced Vital Capacity, But Not with Interstitial Lung Disease

Contact Info

Product

Resources

About