Pulmonary Hypertension Diagnosis in Systemic Sclerosis (Scleroderma)

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Video Overview
Diagnostic Algorithm
Exercise Echo
Functional Classification
Markers for PH in Scleroderma
P-Wave Amplitude
Right Heart Catheterization
Risk Factors
Six-Minute Walk

Video Overview by Amanda Thorpe

All scleroderma patients should be screened for PAH and PH on a regular basis.
Diagnosis of Pulmonary Hypertension in Systemic Scleroderma Video
Video: Diagnosis of Pulmonary Hypertension in Systemic Scleroderma

Presented by Amanda Thorpe
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Diagnosing pulmonary hypertension in systemic scleroderma, especially in the early stages, can be difficult.

Systemic scleroderma can affect the same organs as pulmonary hypertension and symptoms such as fatigue and shortness of breath, the early symptoms of pulmonary hypertension, can also be caused by systemic scleroderma.

Systemic scleroderma symptoms can make it difficult to evaluate the progression and severity of pulmonary hypertension. The 6 minute walk test, used to assess pulmonary hypertension can be hindered or rendered impossible by fatigue, muscle weakness, contractures, musculoskeletal pain, heart and lung complications caused by systemic scleroderma.

For any queries about pulmonary hypertension secondary to systemic scleroderma please see a registered scleroderma expert. (Also see What is Scleroderma?, What is Pulmonary Hypertension?, and Scleroderma Experts)

Diagnosing and managing scleroderma–related pulmonary arterial hypertension. This article describes scleroderma–related PAH and its diagnosis and management. PubMed, JAAPA, 2017 Sep;30(9):11-18.

Expert consensus for performing right heart catheterisation (RHC) for suspected pulmonary arterial hypertension in systemic sclerosis (SSc): a Delphi consensus study with cluster analysis. Among experts in pulmonary arterial hypertension-SSc, a core set of criteria for clinical practice to refer SSc patients for RHC has been defined by Delphi consensus methods. PubMed, Ann Rheum Dis, 2014 Jan 1;73(1):191-7. (Also see Pulmonary Hypertension)

How is Pulmonary Arterial Hypertension diagnosed? Diagnosis can be delayed for months or even years while other possible causes are investigated and as a result, Pulmonary Arterial Hypertension (PAH) is frequently only identified when it is already quite advanced. Actelion Pharmaceuticals.

Symptoms of Pulmonary Hypertension

Initially there may be no symptoms at all of pulmonary hypertension. Later, symptoms include shortness of breath, weakness, and fatigue with exertion. As it progresses, patients become very tired after only slight activity. Eventually, patients experience right-sided heart failure and death. However, the course of mild to moderate PH in scleroderma patients is still unknown, and its possible that it may persist unchanged for long periods of time.

Shortness of Breath This worrisome symptom has many acute and chronic causes. Follow this flowchart for more information about the diseases in which shortness of breath occurs.

What are common symptoms of Pulmonary Arterial Hypertension? Common early symptoms include breathlessness or shortness of breath (particularly on exertion), chronic fatigue, dizziness (especially when walking upstairs or when standing up), fainting, swollen ankles and legs, and chest pain (especially during physical activity). Actelion Pharmaceuticals.

Diagnostic Algorithm and Clinical Characteristics for Pulmonary Hypertension with Scleroderma

Pulmonary arterial hypertension (PAH) in scleroderma (SSc): care gaps in screening. Screening for PAH in SSc allows for earlier detection and treatment that prolongs survival and improves symptoms. BioMed Central, Arthritis Research & Therapy, 201719:128.

Combined pulmonary fibrosis and emphysema (CPFE) in scleroderma lung disease (SSc-ILD) has a major confounding effect on lung physiology and screening for pulmonary hypertension (PH). The confounding effect of CPFE on measures of gas exchange has major implications for the construction of screening algorithms for PH in SSc-ILD. PubMed, Arthritis Rheumatol, 12/04/2015.

A comparison of the predictive accuracy of three screening models for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc). Ultimately, the choice of SSc-PAH screening algorithm will also depend on cost and ease of application. PubMed, Arthritis Res Ther, 2015 Jan 18;17(1):7.

Development of pulmonary hypertension (PH) in a high risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study. A low DLco, high FVC/DLco, exercise-induced hypoxia and entry echo sPAP arrow 40 were strongly associated with future PH. Seminars in Arthritis and Rheumatism, 03/07/2014. (Also see PHAROS)

DLCO in Pulmonary Hypertension

Determinants of impairment in lung diffusing capacity (DLCO) in patients with systemic sclerosis. Partitioning of DLCO in alveolar–capillary membrane conductance and pulmonary capillary blood volume is of little use in distinguishing the patients with only interstitial lung disease (ILD) from those with ILD complicated by PAH. PubMed, Clin Exp Rheumatol, 2015 Jul-Aug;33(4 Suppl 91):S80-6.

Echocardiograms for Pulmonary Hypertension

Echocardiography cannot be relied upon to exclude pulmonary hypertension when pre-test probability is high.

Pros and cons of echocardiography in the screening, diagnosis and follow–up of patients with systemic sclerosis (SSc) pulmonary arterial hypertension – a rheumatologist’s perspective. Despite popular belief and current recommendations, in the opinion of the author, echocardiograms are not necessary in every SSc patient every year. Journal of Scleroderma and Related Disorders, JSRD 2017; 2(2): 108 - 114.

Incidence of pulmonary hypertension (PHT) in patients with systemic sclerosis (SSc) and no pulmonary symptoms: is annual echocardiographic screening necessary? The incidence of echocardiography-diagnosed PHT with SSc, and no pulmonary symptoms was low. Annual echocardiography is less beneficial among such patients; repeated echocardiography should instead be performed on those with a declining functional class. PubMed, J Clin Rheumatol.

Exercise Echocardiography of Pulmonary Hypertension

Using a bicycle ergometer after physical activity and doing an echocardiogram may help identify PAH early, before it becomes irreversible.

Exercise echocardiography for the assessment of pulmonary hypertension in systemic sclerosis: a systematic review. Left ventricle diastolic dysfunction was common and was associated with greater elevation of systolic pulmonary arterial pressure on exercise. BioMed Central, Arthritis Research & Therapy, 07/02/2016.

Detection of pulmonary vasculopathy by novel analysis of oxygen uptake in patients with systemic sclerosis: association with pulmonary arterial pressures. Some scleroderma patients have decreased oxygen uptake, likely caused by sudden pulmonary arterial pressure increases during exercise. PubMed, Clin Exp Rheumatol, 2014 Jul 17.

Functional Classification of Pulmonary Hypertension

The Classification of Pulmonary Arterial Hypertension. Why does the literature sometimes refer to WHO functional class and sometimes to NYHA functional class? What is the difference? Medscape.

Markers for Pulmonary Hypertension in Systemic Sclerosis (Scleroderma)

Risk factors for development of pulmonary arterial hypertension (PAH) in Australian systemic sclerosis patients: results from a large multicenter cohort study. This model identifies a subset of patients at an appreciably higher risk of developing PAH, who should be screened and would in future, benefit from preventative therapies. PubMed, BMC Pulm Med, 2016 Sep 27;16(1):134.

Borderline Pulmonary Pressure in Scleroderma Patients Linked to a Pre-Pulmonary Arterial Hypertension Condition. A review study recently published suggests that borderline pulmonary pressure in patients with scleroderma can represent a condition of pre-pulmonary arterial hypertension (PAH). Scleroderma News, 06/01/2015.

Impact of anti-centromere antibodies (ACA) on pulmonary function test (PFT) results in patients with systemic sclerosis without established or suspected pulmonary disease. Patients with ACA, without established or suspected pulmonary complications, have PFT abnormalities consistent with indolent increased pulmonary vascular resistance despite the majority of such patients not subsequently developing PAH. PubMed, Clin Rheumatol, 2014 Apr 22. (Also see Scleroderma Autoantibodies)

Anticardiolipin antibodies (aCL) are significantly associated with pulmonary arterial hypertension in systemic sclerosis (scleroderma) patients.

Anticardiolipin antibodies (aCL) are significantly associated with pulmonary arterial hypertension in systemic sclerosis (scleroderma) patients. (Also see What is Scleroderma?, Types of Scleroderma, and Antiphospholipid Syndrome)

P-Wave Amplitude

P-wave amplitude and pulmonary artery pressure in scleroderma. P-wave amplitude analysis on the ECG may be helpful in the assessment of pulmonary hypertension in patients with scleroderma. PubMed, J Electrocardiol.

Right Heart Catheterization (RHC) for Pulmonary Hypertension

Clinical determinants of elevated systolic pulmonary artery pressure measured by transthoracic Doppler echocardiography in early systemic sclerosis (SSc). Our findings emphasize the need to consider right heart catheterisation in selected early SSc patients with pulmonary hypertension suspicion (PH), to clearly determine the cause of PH. PubMed, Clin Exp Rheumatol, 06/20/2017.

Determining the necessity for right heart catheterization (RHC) in pulmonary hypertension (PH) associated with connective tissue diseases (CTD) assessed by echocardiography. These findings demonstrate the necessity for RHC and differences in prognosis of PH in CTD. Wiley Online Library, 10/07/2015. (Also see Connective Tissue Diseases)

Early Detection of PAH in Systemic Sclerosis Achieved Using Ultrasound Technology. Using right heart catheterization as the gold standard in all patients showed that stress Doppler-echocardiography markedly improved sensitivity in detecting manifest Pulmonary Hypertension. Scleroderma News, 06/22/2015. (Also see Diagnosis of Scleroderma Heart Involvement)

Expert consensus for performing right heart catheterisation (RHC) for suspected pulmonary arterial hypertension in systemic sclerosis (SSc): a Delphi consensus study with cluster analysis. Among experts in pulmonary arterial hypertension-SSc, a core set of criteria for clinical practice to refer SSc patients for RHC has been defined by Delphi consensus methods. PubMed, Ann Rheum Dis, 2014 Jan 1;73(1):191-7. (Also see Right Heart Catheterization)

Risk Factors for Pulmonary Hypertension

Determinants of Pulmonary Arterial Hypertension (PAH) in Scleroderma. The present analysis identified pulmonary fibrosis and Raynaud's phenomenon preceding SSc skin manifestations by at least 3 years as risk factors for PAH in our scleroderma cohort. PubMed, Semin Arthritis Rheum.

Six-Minute Walk for Pulmonary Hypertension in Scleroderma Patients

Heart rate recovery is an important predictor of outcomes in patients with connective tissue disease–associated pulmonary hypertension (CTD-PH). In patients with CTD-PH, abnormal HRR1 (defined as HRR1 of lower than 16) after the six minute walk test is a strong predictor of clinical worsening, time to clinical worsening, survival, and hospitalization. PubMed, Pulm Circ, 2015 Sep;5(3):565-76.

Factors relating to impaired stroke volume during the 6–minute walk test in patients with systemic sclerosis. Impaired stroke volume in patients with systemic sclerosis was observed at rest and during exercise, and the factors relating to the cardiac response seemed to be pulmonary function and the extent of pulmonary hypertension. PubMed, Clin Exp Rheumatol, 07/22/2016. (Also see Diagnosis of Scleroderma Pulmonary Involvement)

Go to Pulmonary Hypertension Prognosis

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