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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)

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

Response to exercise in patients with pulmonary arterial hypertension (PAH) treated with combination therapy. We investigated pulmonary haemodynamic response to exercise, and the relationship to ventilatory efficiency and hypoxaemia in patients with PAH treated with combination therapy. PubMed, ERJ Open Res, 2021 Jan 25;7(1):00725-2020.

Exercise Pulmonary Hypertension (PH) Predicts Clinical Outcomes in Patients With Dyspnea on Effort. These findings suggest incremental value of exercise hemodynamic assessment to resting measurements alone in characterizing the burden of PH in individuals with dyspnea. PubMed, J Am Coll Cardiol, 2020 Jan 7;75(1):17-26. (Also see Secondary Pulmonary Hypertension)

Correlation with Other Diseases

The incidence rate of pulmonary arterial hypertension (PAH) and scleroderma renal crisis (SRC) in systemic sclerosis patients with digital ulcers on endothelin antagonist receptors (ERAs) and phosphodiesterase-5 inhibitors (PDE5i). Treatment with ERAs and/or PDE5i appeared to delay the occurrence of SRC. PubMed, Rheumatology (Oxford), 2020 Aug 26;keaa401. (Also see Kidney (Renal) Involvement and Research on Digital Ulcers)

Complement alternative pathway activation associated with pulmonary hypertension in lupus nephritis patients. Activation of the complement alternative pathway may be involved in the pathogenesis of pulmonary hypertension in lupus nephritis. PubMed, Lupus, 2019 Jul 11:961203319860192. (Also see Symptoms and Complications of Lupus)

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.

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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