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.

Survival and quality of life in incident systemic sclerosis–related pulmonary arterial hypertension (SSc-PAH). Despite the significant benefits conferred by advanced PAH therapies suggested in this study, the median survival in SSc PAH remains short at only 4 years. BioMed Central, Arthritis Research & Therapy, 06/02/2017.

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

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.

Assessment of Life Expectancy in Scleroderma–Associated Pulmonary Arterial Hypertension (SSc–PAH). Patients with SSc–PAH have a slightly higher mortality risk than patients with SSc–PAH with concomitant interstitial lung disease and significant differences exist between the 2 patient populations with regard to key cardiovascular markers. Rheumatology Advisor, 11/08/2017.

Factors associated with disease progression in early–diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort. More than 40% of early–diagnosed patients with SSc–PAH had disease progression during a short follow–up time, which suggests that even mild PAH should be considered a high–risk complication of SSc. PubMed, Ann Rheum Dis, 10/23/2017.

Idiopathic and Systemic Sclerosis (SSc) associated Pulmonary Arterial Hypertension (PAH): A Comparison of Demographic, Haemodynamic and Magnetic Resonance Imaging Characteristics and Outcomes. The reasons for poorer outcomes in SSc–PAH are likely to be multifactorial including, but not limited to, older age and reduced gas transfer. PubMed, Chest, 02/18/2017.

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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