Monitoring and Diagnostic Approaches for Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis

Valenzuela, Antonia; Nandagopal, Saranya; Steen, Virginia D.; Chung, Lorinda

doi:10.1016/j.rdc.2015.04.009

Cited by 12 publications

(13 citation statements)

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“…SSc is a complex clinically heterogeneous disease, the underlying aetiology of which is not fully understood. It is presumed to occur in a genetically susceptible host after exposure to an environmental trigger [ 1 ]. Currently there are no effective disease modifying agents or cure.…”

Section: Introductionmentioning

confidence: 99%

“…SSc-PAH, as diagnosed on right heart catheterisation (mPAP >25 and PAWP <15 mmHg at rest), occurs with a prevalence of 8–12 % and is associated with reduced life expectancy [ 3 ]. The pathogenesis of SSc-PAH is unknown, but thought to be a combination of endothelial cell dysfunction and an imbalance of endothelial derived vasoactive substances [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

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Risk factors for development of pulmonary arterial hypertension in Australian systemic sclerosis patients: results from a large multicenter cohort study

et al. 2016

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BackgroundPulmonary arterial hypertension (PAH) is the leading cause of mortality in patients with systemic sclerosis (SSc). We sought to determine the incidence, prevalence and risk factors for PAH development in a large Australian SSc cohort.MethodsPAH was diagnosed on right heart catheterisation (mPAP >25 and PAWP <15 mmHg at rest). Patients with PH secondary to interstitial lung disease (ILD; defined as abnormal HRCT scan and FVC < 60 %) were excluded. Summary statistics, chi-square tests, univariate and multivariable logistic regression along with post-estimation diagnostics were used to determine the associations of different combinations of risk factors with PAH.ResultsAmong 1579 SSc patients, 8.4 % (132 patients) were diagnosed with PAH over a mean (±SD) follow-up of 3.2 (±2.5) years. The incidence of PAH in this cohort was 0.7 % per annum. Of these, 68.9 % had limited disease subtype (lcSSc). In multivariable regression analysis, the presence of anti-centromere antibody (ACA) (OR 1.6, 95 % CI 1.1–2.5, p = 0.03), oesphageal stricture (OR 2.0, 95 % CI 1.2–3.3, p = 0.006), calcinosis (OR 1.9, 95 % CI 1.2–2.9, p = 0.003), sicca symptoms (OR 1.6, 95 % CI 1.1–2.5, p = 0.03), mild ILD (OR 2.3, 95 % CI 1.5–3.7, p < 0.001) and digital ulcers (OR 1.6, 95 % CI 1.0–2.4, p = 0.03) were predictive of PAH. This model had an area under the curve of 0.7 and concordance of 91.8 %. When analysed by disease subtype, the presence of calcinosis (OR 2.2, 95 % CI 1.4–3.7, p = 0.01), sicca symptoms (OR 2.6, 95 % CI 1.5–4.6, p = 0.001), mild ILD (OR 2.3, 95 % CI 1.4–3.8, p = 0.001) and digital ulcers (OR 1.9, 95 % CI 1.2–3.7, p = 0.01) were predictive of PAH in lcSSc; and oesophageal stricture (OR 4.4, 95 % CI 1.9–10.5, p = 0.001), mild ILD (OR 2.8, 95 % CI 1.2–6.8, p = 0.02) and ACA (OR 5.2, 95 % CI 1.8–14.8, p = 0.002) were predictive of PAH in dcSSc.ConclusionsThe incidence and prevalence of PAH in this cohort are 0.7 % per annum and 8.4 %, respectively. The clinical-serologic risk factors for PAH differ based on disease subtype. In both subtypes, mild ILD is associated with PAH, suggesting the possibility of common pathogenic mechanisms underlying both of these disease manifestations. This model identifies a subset of patients at an appreciably higher risk of developing PAH, who should be screened and would in future, benefit from preventative therapies.Electronic supplementary materialThe online version of this article (doi:10.1186/s12890-016-0296-z) contains supplementary material, which is available to authorized users.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Risk factors for development of pulmonary arterial hypertension in Australian systemic sclerosis patients: results from a large multicenter cohort study

et al. 2016

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“…This is addition to the vague nature of symptoms related to PH and the absence of systematic screening of PAH in SSc patients despite the presence of validated screening tools for PAH in SSc patients. [614] For example, the detect instrument, which incorporates 6MWD and BNP level, has been shown to increase the identification of PAH patients compared to clinical assessment alone. [14151617]…”

Section: Discussionmentioning

confidence: 99%

Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality

et al. 2019

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BACKGROUND:Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in patients with systemic sclerosis (SSc). The objective of this study is to describe the clinical characteristics, mortality, and predictors of SSc-PAH in Saudi patients.METHODS:Retrospective chart review study of SSc patients who were followed for at least 1 year in three tertiary care centers in Saudi Arabia was conducted. Clinical information, echocardiographic findings, and right heart catheterization (RHC) results were collected. Descriptive statistics were used for demographic and disease characteristics.RESULTS:Fifty-seven patients with SSc were reviewed. PAH was confirmed by RHC in 40 patients (87.5%, females). Their mean age was 45.43 ± 13.48 years. The mean pulmonary artery pressure was 42.9 ± 12.7 mmHg, the pulmonary vascular resistance index was 19.4 ± 7.7 woods unit, and cardiac index was 2.43 ± 0.68 min/m2. The median time from symptoms to first assessment was 42.8 ± 115.62 months. Most patients (77.5%) presented with functional Class III or IV and more than half (22.55%) were on dual combination therapy. Ten patients (25%) SSc PAH died over a follow up period of 37 ± 7 months. Compared to SSc patients without PAH, SSc-PAH patients had shorter 6-min walk distance (6MWD) (296.1 ± 116.5 vs. 399.59 ± 40.60 m, P < 0.0001), higher pro-brain natriuretic peptide (1755.8 ± 2123.4 vs. 69.8 ± 44.3 pg/ml P = 0.004), and more frequent Raynaud's phenomenon (RP) (90% vs. 35%, P < 0.0001). Logistic regression showed RP (odds ratio [OR] =48.58, 95% confidence interval [CI]; 3.73–633.10) and 6MWD (OR 1.02: 95% CI; 1.01–1.03) were associated with the development of PAH.CONCLUSION:Our cohort of Saudi SSc-PAH patients has a younger disease onset and a lower mortality than what is described worldwide despite late presentation and requirement of combination therapy. The presence of RP and lower were associated with the development of SSc-PAH.

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“…The early identification of PAH in its 'active' phase remains challenging as systemic markers of vasculopathic manifestations of SSc are lacking. 39 Defining activity is complicated by the lack of definitions of organ involvement for particular SSc-affected systems, for example, cardiac disease. There is no expert agreement as to the definition of primary cardiac involvement; therefore, measures of the pathological processes causing ultimate cardiac dysfunction remain elusive.…”

Section: Measuring Activity In Sscmentioning

confidence: 99%

“…The early identification of PAH in its ‘active’ phase remains challenging as systemic markers of vasculopathic manifestations of SSc are lacking. 39…”

Section: Measuring Activity In Sscmentioning

confidence: 99%

The challenges and controversies of measuring disease activity in systemic sclerosis

Ross

Baron

Nikpour

2018

Journal of Scleroderma and Related Disorders

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Major alteration of the natural history of systemic sclerosis is limited with current treatments, and the development of novel therapies has been hampered, in part, by the lack of fully validated multi-system outcome measures. There remains a lack of consensus as to the very definition of systemic sclerosis disease activity, complicating efforts to measure activity in clinical trials. Previously published multi-system measures of disease status are yet to be fully validated according to the Outcome Measures in Rheumatology (OMERACT) filter. There is currently significant research interest in developing new systemic sclerosis-specific measures to better describe and compare patient cohorts and measure therapeutic responses in clinical trials. An accurate measure of disease activity in systemic sclerosis will facilitate the enrichment of clinical trials with patients who have active disease, targeting a group of patients most likely to benefit from therapeutic intervention. In addition, following on from successes in other rheumatic conditions, a state of low disease activity, measured by an activity index, may become a clinical trial end point and therapeutic target. The Scleroderma Clinical Trials Consortium has undertaken to develop a definition of disease activity and fully validate a new systemic sclerosis activity index. The Scleroderma Clinical Trials Consortium Activity Index will be developed using consensus and datadriven methods and is envisaged to be widely used in research and clinical settings.

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Monitoring and Diagnostic Approaches for Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis

Cited by 12 publications

References 94 publications

Risk factors for development of pulmonary arterial hypertension in Australian systemic sclerosis patients: results from a large multicenter cohort study

Risk factors for development of pulmonary arterial hypertension in Australian systemic sclerosis patients: results from a large multicenter cohort study

Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality

The challenges and controversies of measuring disease activity in systemic sclerosis

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