Prognosis of Pulmonary Hypertension in Systemic Sclerosis

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview of PH Prognosis
Functional Classification of PH
Markers for Prognosis of PH
Personal Stories of Prognosis of PH


Early diagnosis and treatment substantially improves the prognosis for pulmonary hypertension associated with systemic sclerosis (scleroderma). (Also see Types of Scleroderma, PH Diagnosis, PH Treatments and Types of Scleroderma: Prognosis and Mortality)

Validation of the REVEAL Prognostic Equation and Risk Score Calculator in Incident Systemic Sclerosis–Associated Pulmonary Arterial Hypertension. In predicting one–year survival in newly diagnosed SSc–PAH, the REVEAL prognostic equation and risk score provide very good discrimination but poor calibration. PubMed, Arthritis Rheumatol, 05/08/2019.

Functional Classification of Pulmonary Hypertension

Functional Classification of Severe Pulmonary Hypertension. The higher the class the more severe the disease and the greater the urgency for the patient to be accurately diagnosed and started on therapy for pulmonary hypertension. Pulmonary Hypertension RN.

Haemodynamic phenotypes and survival in patients with systemic sclerosis: the impact of the new definition of pulmonary arterial hypertension (PAH). The data of this study show that a pulmonary vascular resistance threshold ≽3 WU is too high to enable an early diagnosis of PAH. PubMed, Ann Rheum Dis, 12/09/2019.

Markers for Prognosis of Pulmonary Hypertension

Identifying early pulmonary arterial hypertension (PAH) biomarkers in systemic sclerosis (SSc): Machine learning on proteomics from the DETECT cohort. This novel 8-protein biomarker panel has the potential to improve early detection of PAH in SSc patients and may provide novel insights into the pathogenesis of PAH in the context of SSc. PubMed, Eur Respir J, 2020 Dec 17;2002591.

Whole blood viscosity in systemic sclerosis: a potential biomarker of pulmonary hypertension (PAH)? Higher whole blood viscosity levels in scleroderma patients with PAH was an independent indicator for PAH development. PubMed, Clin Rheumatol, 05/26/2019.

Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis (SSc) Patients At Risk for Pulmonary Hypertension (PH), in the PHAROS Registry. Risk factors for poor outcome in patients with SSc who are at risk for PH were similar to others with SSc–PH and SSc–pulmonary arterial hypertension. PubMed, J Rheumatol, 10/01/2018.

Long–term Outcomes in Systemic Sclerosis Associated Pulmonary Arterial Hypertension PAH) from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry (PHAROS). PAH accounted for more than half of deaths and primarily within the first few years after PAH diagnosis. PubMed, Chest, 05/16/2018.

High acute phase protein levels correlate with pulmonary and skin involvement in patients with diffuse systemic sclerosis. High serum amyloid A (SAA) and C-reactive protein (CRP) levels could serve as biomarkers for pulmonary involvement. PubMed, J Int Med Res, 2018 Apr;46(4):1634-1639. (Also see Prognosis and Mortality)

Red blood cell distribution width (RDW) as a related factor of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc). Besides the recognized risk factors, high RDW was an independent predictor of PAH in patients with SSc and may be a related factor for identifying the pulmonary arterial hypertension in SSc patients. PubMed, Clin Rheumatol, 2018 Apr;37(4):979-985.

Patients with systemic sclerosis–associated pulmonary arterial hypertension express a genomic signature distinct from patients with interstitial lung disease. Many gene expression changes occur early in the disease course, potentially allowing early detection. Journal of Scleroderma and Related Disorders, 03/22/2018.

Stories of Prognosis of Pulmonary Hypertension

Syl: Systemic Scleroderma/CREST/Pulmonary Hypertension/Sjögren's The physician's assistant noticed that my hands went really blue…

Tony B: Systemic Scleroderma with Pulmonary Fibrosis, Pulmonary Hypertension and Raynaud's It has taken me some time to come to terms with this disease…

Go to Pulmonary Hypertension Research Registries

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