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)

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.

Survival protection by bodyweight in isolated scleroderma–related pulmonary artery hypertension. The results suggest body mass index decline should be contrasted, lung diffusion is useful for screening and with stroke volume index and mixed venous saturation for assessing prognosis and treatment. PubMed, Intern Emerg Med, 04/06/2016.

Sex disparities in systemic sclerosis (SSc)–associated pulmonary arterial hypertension (PAH): a cohort study. Sex disparities appear to exist in the frequency of PAH, time to PAH diagnosis, PAH disease duration and SSc disease burden. PubMed, Arthritis Res Ther, 2016 Jan 27;18(1):30.

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.

Markers for Prognosis of Pulmonary Hypertension

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.

Aberrant immune response with consequent vascular and connective tissue remodeling – causal to scleroderma and associated syndromes? Therapeutic helminth infection or treatment with parasite–derived response modifiers could be promising new management tools for autoimmune connective tissue diseases. PubMed, Curr Opin Rheumatol, 2016 Nov;28(6):571-6.

The role of asymmetric dimethylarginine (ADMA) alone and in combination with N–terminal pro–B–type natriuretic peptide as a screening biomarker for systemic sclerosis–related pulmonary arterial hypertension (SSc–PAH): A case control study. In this small study, use of ADMA in combination with NT–proBNP produced excellent sensitivity and specificity for the non–invasive identification of SSc–PAH. PubMed, Clin Exp Rheumatol, 05/23/2016.

Prospects for improving outcomes in systemic sclerosis-related pulmonary hypertension (PAH). With the advent of evidence-based therapies, including newer agents such as macitentan, riociguat and selexipag, the establishment of centres with expertise in PAH and the focus on early detection, there has been considerable improvement in survival. PubMed, Intern Med J, 2015 Mar;45(3):248-54. (Also see Prognosis and Mortality)

Pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc) in patients with functional class II dyspnoea: mild symptoms but severe outcome. A majority of patients with mildly symptomatic SSc-PAH in New York Heart Association (NYHA) functional class (FC) II at diagnosis have a severe disease with poor prognosis. Eric Hachulla. Rheumatology. February 8, 2010.

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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