Pulmonary Hypertension

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Video Overview
Primary PH
Secondary PH
Exercised Induced PH
Disease Correlations
Research Registries
Patient Support
Personal Stories


Overview of Pulmonary Hypertension Video
Video: Overview of Pulmonary Hypertension

Presented by Amanda Thorpe
*More Sclero Videos*

Pulmonary Hypertension is not the same as "regular" high blood pressure or hypertension, which can be easily measured with a blood pressure test.

Pulmonary hypertension is a high pressure in the arteries between the heart and lungs or pulmonary arteries. It is very difficult to detect elevation in pressure in these arteries. Pulmonary Hypertension is a type of vascular disease that can be found in scleroderma patients. (Also see Scleroderma Lung Involvement, What is Scleroderma?, Types of Scleroderma, and Systemic Sclerosis)


Causes of Pulmonary Hypertension. There are dozens of causes of pulmonary hypertension, ranging from genetics, medications or street drugs, connective tissue diseases (such as scleroderma), heart and lung diseases, and blood disorders. ISN.

Altitude and Hypoxia
Antiphospholipid Antibodies
Endothelial Dysfunction
Estrogen and Enzyme CYP1B1
Vascular Disease/Scleroderma

Primary Pulmonary Hypertension (PH)

When PH occurs by itself, it is called Primary Pulmonary Hypertension.

Primary pulmonary hypertension occurs by itself and not secondary to another illness, such as scleroderma. Nailfold capillary examination can easily tell whether pulmonary hypertension is primary or secondary. (Also see Nailfold Capillaroscopy)

Differences in Right Ventricular Functional Changes during Treatment between Systemic Sclerosis–associated Pulmonary Arterial Hypertension (SSc–PAH) and Idiopathic Pulmonary Arterial Hypertension (IPAH). In patients with SSc–PAH, echocardiographic RV function does not improve over time compared with that of patients with IPAH, despite institution of pulmonary artery vasodilator therapies. PubMed, Ann Am Thorac Soc, 2017 May;14(5):682-689.

Secondary Pulmonary Hypertension

Treatments and their effectiveness can vary
depending on whether PH is primary or secondary.

Secondary Pulmonary Hypertension. When PH occurs along with other lung, heart, or systemic connective tissue disease (such as scleroderma), it is called Secondary Pulmonary Hypertension. The distinction is important because treatments, and their effectiveness, can vary depending upon whether the pulmonary hypertension is primary or secondary. ISN.

Overview of Secondary PH/PAH
Antiphospholipid Markers
PH Secondary to MCTD
PH Secondary to Pulmonary Fibrosis
PH Secondary to Scleroderma
PH Secondary to Scleroderma: Dyspnea

Exercised Induced

Changes in pulmonary exercise haemodynamics in scleroderma (SSc): a 4–year prospective study. Patients with SSc developed mild deterioration of pulmonary exercise hemodynamics and exercise capacity over a 4–year follow–up period, indicating mild but significant progression of pulmonary vascular disease. PubMed, Eur Respir J, 2017 Jul 13;50(1).

Readdressing the entity of exercise pulmonary arterial hypertension (EPAH). Patients with collagen vascular disease, especially scleroderma, are at risk for EPAH and its presence indicates a relatively poor prognosis. PubMed, Respir Med, 2017 Mar;124:65-71.

Exercise echocardiography for the assessment of pulmonary hypertension in systemic sclerosis: a systematic review. We found very high heterogeneity in the methods, the protocols and the estimated sPAP response to exercise. LV diastolic dysfunction was common and was associated with greater elevation of sPAP on exercise. PubMed, Arthritis Res Ther, 2016 Jul 2;18(1):153.

Correlation with Other Diseases

Prevalence of auto–antibodies associated to pulmonary arterial hypertension in scleroderma – A review. The available evidence points in the direction of a strong association between auto–immune mechanisms and pulmonary hypertension in the setting of scleroderma. PubMed, Autoimmun Rev, 10/11/2018. (Also see Antibodies)

Pulmonary arterial hypertension (PAH) in the setting of scleroderma (SSc) is different than in the setting of lupus: A review. This review highlights the differences between SSc–PAH and Lupus–PAH. PubMed, Respir Med, 2018 Jan;134:42-46.

Idiopathic and Systemic Sclerosis-Associated Pulmonary Arterial Hypertension (SSc-PAH): A Comparison of Demographic, Hemodynamic, and MRI 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, 2017 Jul;152(1):92-102.

Hematological disorders and pulmonary hypertension. Recent studies have shown deregulated iron homeostasis in patients with cardiopulmonary diseases including pulmonary arterial hypertension (PAH). PubMed, World J Cardiol, 2016 Dec 26;8(12):703-718. (Also see What is Scleroderma? and Anemia of Chronic Disease)

Impact of pulmonary fibrosis (PF) and elevated pulmonary pressures (PHT) on right ventricular (RV) function in patients with systemic sclerosis (SSc). SSc patients show impaired RV function and both pulmonary fibrosis and PHT are independently associated with RV dysfunction. PubMed, Rheumatology (Oxford), 2016 Mar;55(3):504-12. (Also see Cardiac (Heart) Involvement)

Application of the DETECT algorithm for detection of risk of pulmonary arterial hypertension in systemic sclerosis: data from a Czech tertiary centre. The modified DETECT algorithm detects all patients with PAH diagnosed according to ECS/ERS 2009 guidelines and RHC. Data of the 2–year follow–up indicate a possible positive predictive role for the modified DETECT calculator. PubMed, Rheumatology (Oxford), 2016 Jan;55(1):109-14.

Relationship of main pulmonary artery diameter to pulmonary arterial pressure (PH) in scleroderma patients with and without interstitial fibrosis. In scleroderma patients, an enlarged main pulmonary artery predicts PH even in the presence of mild to moderate fibrosis. PubMed, J Comput Assist Tomogr. (Also see Correlation of Pulmonary Fibrosis and Pulmonary Hypertension)


Diagnosis of Pulmonary Hypertension. Doppler echocardiograms are notoriously inaccurate when the PH is mild or when there is simultaneous presence of lung scarring. PH cannot be diagnosed without right heart catheterization. ISN.

Video Overview
Diagnostic Algorithm
Exercise Echo
Functional Classification
Markers for PH in Scleroderma
P-Wave Amplitude
Right Heart Catheterization
Risk Factors
Six-Minute Walk


Prognosis of Pulmonary Hypertension. Early diagnosis and treatment substantially improves the prognosis for pulmonary hypertension associated with systemic sclerosis (scleroderma). ISN.

Overview of PH Prognosis
Functional Classification of PH
Markers for Prognosis of PH
Personal Stories of Prognosis of PH


Treatments include oxygen therapy, Tracleer (bosentan), Digitalis, diuretics, Flolan (in clinical trials), and, in a few cases, heart-lung or double-lung transplants.

Video Overview
ASK1 inhibitor
Combination Therapies
Endothelin Receptors
Inhaled Prostacyclin (Ventavis, Iloprost)
Heart/Lung Transplants
Flolan (Epoprostenol)
PDE 5 Inhibitors
Sildenafil (Revatio)
Tadalafil (ADCIRCA)
Vardenafil (Levitra)
Stem Cell Transplant
Treprostinil (TYVASO)
Uptravi (Selexipag)
Warfarin (Coumadin)

PH Research Registries

Pulmonary Hypertension Research Registries include PHAROS and CoTherix. SureSteps Patient Support Program by Actelion is for patients on Tracleer or Bosentan. ISN.

PH Research Panel (by WWMR)
PH Registry of Scleroderma (PHAROS)
Scleroderma Research Registries
Pulmonary Support Organizations


Ineffective Therapies


Pulmonary Support Resources ISN.

Patient & Caregiver Stories

Pulmonary Hypertension Patient and Caregiver Stories provide vital support, and let others know they are not alone. ISN.

Archie Bailey: Scleroderma: Sue's Story Time has passed since Sue's death. Only those who have experienced the grieving process can understand the profound agony death presents to friends and loved ones…

Bill Jordan: Surviving Boyfriend of Scleroderma Patient Wendy was heartset on dancing that evening even though she was connected to an oxygen machine…

Brother Alan: Systemic Scleroderma I was diagnosed with Scleroderma three years ago and "too many times ten" doctors later, here I am…

Read more pulmonary hypertension patient and caregiver story listings…

Go to Pulmonary Hypertension Diagnosis

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