Associated factors of early-onset pulmonary hypertension and clinical difference between early- and late-onset pulmonary hypertension in Thai systemic sclerosis

Krikeerati, Thanachit; Pussadhamma, Burabha; Mahakkanukrauh, Ajanee; Suwannaroj, Siraphop; Nanagara, Ratanavadee; Foocharoen, Chingching

doi:10.1080/14397595.2020.1823067

Cited by 3 publications

(3 citation statements)

References 45 publications

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“…Moreover, early onset of SSc-PAH is associated with more severe vascular disease than late onset SSc-PAH. 73 , 74 …”

Section: Risk Stratificationmentioning

confidence: 99%

“…Moreover, early onset of SSc-PAH is associated with more severe vascular disease than late onset SSc-PAH. 73,74 Valid algorithms exist to help select patients for evaluation for PAH using a right heart catheterization. 75 These algorithms include the measurement of proBNP, presence of telangiectasias on physical examination, measurement of serum urate, presence of right axis deviation on ECG and the detection of anticentromere antibodies.…”

Section: Pulmonary Hypertensionmentioning

confidence: 99%

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Predicting the Progression of Very Early Systemic Sclerosis: Current Insights

Bellocchi¹,

Chung²,

Volkmann³

2022

OARRR

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Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease with distinct pathological hallmarks (ie, inflammation, vasculopathy, fibrosis) that may predominate at different stages in the disease course with varying severity. Initial efforts to classify patients with SSc identified a subset of patients with very early SSc. These patients possessed signs of SSc (eg, Raynaud phenomenon, SSc specific autoantibodies and/or nailfold capillary abnormalities) without fulfilling complete SSc classification criteria. Recognizing the inherent value in early diagnosis and intervention in SSc, researchers have endeavored to identify risk factors for progression from very early SSc to definite SSc. The present review summarizes the clinical phenotype of patients with very early and early SSc. Through a scoping review of recent literature, this review also describes risk factors for progression to definite SSc with a focus on the specific clinical features that arise early in the SSc disease course (eg, diffuse cutaneous sclerosis, interstitial lung disease, esophageal dysfunction, renal crisis, cardiac involvement). In addition to clinical risk factors, this review provides evidence for how biological data (ie, serological, genomic, proteomic profiles, skin bioengineering methods) can be integrated into risk assessment models in the future. Furthering our understanding of biological features of very early SSc will undoubtedly provide novel insights into SSc pathogenesis and may illuminate new therapeutic targets to prevent progression of SSc.

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“…Moreover, early onset of SSc-PAH is associated with more severe vascular disease than late onset SSc-PAH. 73 , 74 …”

Section: Risk Stratificationmentioning

confidence: 99%

Section: Pulmonary Hypertensionmentioning

confidence: 99%

Predicting the Progression of Very Early Systemic Sclerosis: Current Insights

Bellocchi¹,

Chung²,

Volkmann³

2022

OARRR

View full text Add to dashboard Cite

show abstract

“…Of connective tissue diseases, systemic sclerosis (SSc) is of particularly clinical significance as an underlying disease of PAH, as the outcome of SSc-PAH is less favourable than that of other PAH (1,2). There are several potential explanations for the poor outcome of SSc-PAH (3)(4)(5). First, pulmonary vasculopathy of SSc-PAH does not always share the histopathological aspects with that of other PAH.…”

Section: Introductionmentioning

confidence: 99%

The assessment of left heart disease in patients with systemic sclerosis and pulmonary hypertension

Ninagawa

Kato

Ohira

et al. 2021

Clinical and Experimental Rheumatology

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Objective. Systemic sclerosis associated pulmonary arterial hypertension (SSc-PAH) is of clinical significance owing to its poor outcome. One of the explanations for the outcome is the copresence of left heart disease (LHD). The aim of this study is to assess LHD phenotype in patients with SSc and pulmonary hypertension (PH).Methods. This study included consecutive patients with SSc who underwent right heart catheterisation to diagnose PAH. Heart failure with preserved ejection fraction (HFpEF) was evaluated according to the recommendation of 6 th WSPH and to the Framingham criteria. Results. In total, 76 patients were enrolled in this study. Of them, 42 had PH (mPAP >20 mmHg) with a normal left ventricle ejection fraction (≥50%). Among the 42 patients, four and three patients were classified "HFpEF not excluded" and "HFpEF confirmed" whereas 10 had a clinical diagnosis of HFpEF according to 6 th WSPH and Framingham criteria, respectively. These differences were due mainly to relatively low PAWP (<13 mmHg). By a combination of ROC curve and logistic regression analyses, left atrial dimension and left ventricular end-diastolic volume index assessed with echocardiography and cardiac MRI, respectively, had significantly higher predictive values for detecting the complication of HFpEF rather than PAWP. Conclusion. Morphological evaluation using echocardiography and cardiac MRI, compared with haemodynamic evaluation by PAWP, may better reflect the copresence of LHD phenotype in patients with SSc and PH. Our data would also indicate a limited elevation of PAWP in patients with SSc, PH and HFpEF.

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Increased risk of mortality in systemic sclerosis-associated pulmonary hypertension: a systemic review and meta-analysis

et al. 2022

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Background Pulmonary hypertension (PH) is a frequent complication of systemic sclerosis (SSc) and is currently one of the primary causes of death in patients with this disease. We conducted a systematic review and meta-analysis to assess the association between PH and mortality in patients with SSc to verify trends in mortality in patients with SSc-associated PH. Methods We searched the PubMed and Embase databases for published studies on SSc-associated PH from inception to May 2021. All cohort studies in which mortality and/or survival for SSc-associated PH were reported were included in the analysis. The outcome parameters were pooled and analyzed using a random-effects model via generic inverse-variance weighting in conventional and cumulative meta-analysis. Results The literature search identified 1161 citations, and the full texts of 54 studies were examined. Sixteen articles, with a total of 7857 patients with SSc and 1140 patients with SSc-associated PH, were included in the meta-analysis. Patients with SSc-associated PH had a higher pooled risk of mortality than patients with SSc without PH (risk ratio = 3.12; 95% confidence interval: [2.44, 3.98]). Conclusions This meta-analysis revealed a higher mortality in patients with SSc-associated PH. PH was a significant predictor of death in patients with SSc. Thus, early diagnosis and treatment of PH are important in patients with SSc.

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Associated factors of early-onset pulmonary hypertension and clinical difference between early- and late-onset pulmonary hypertension in Thai systemic sclerosis

Cited by 3 publications

References 45 publications

Predicting the Progression of Very Early Systemic Sclerosis: Current Insights

Predicting the Progression of Very Early Systemic Sclerosis: Current Insights

The assessment of left heart disease in patients with systemic sclerosis and pulmonary hypertension

Increased risk of mortality in systemic sclerosis-associated pulmonary hypertension: a systemic review and meta-analysis

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