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
*More Sclero Videos*

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.

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.

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.

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.

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, 2016 Jul 2;18(1):153.

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? Pulmonary Hypertension News.

Markers for Pulmonary Hypertension in Systemic Sclerosis (Scleroderma)

Serum biomarker for diagnostic evaluation of pulmonary arterial hypertension in systemic sclerosis (SSc–PAH). The combination of Midkine and Follistatin-like 3 can serve as an SSc–PAH biomarker and are potential drug targets for this rare disease population. PubMed, Arthritis Res Ther, 2018 Aug 16;20(1):185.

Angiogenic and inflammatory biomarkers for screening and follow-up in patients with pulmonary arterial hypertension (PAH). Plasma levels of PlGF, sVEGFR-1, TNF-α, and VEGF-D have potential in screening for SSc–associated PAH. Plasma sVEGFR-1 may be a biomarker of treatment response. PubMed, Scand J Rheumatol, 2018 Mar 12:1-6.

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.

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

Incidence of pulmonary hypertension (PH) and determining factors in patients with systemic sclerosis (SSc). It was possible to identify manifest PH in almost 25% of patients using prospective right heart catheterisation during follow-up. PubMed, Eur Respir J, 2018 Apr 4;51(4).

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)

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

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)

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.

Go to Pulmonary Hypertension Prognosis

Many thanks to our recent donors, Jack and Elizabeth Lewis, Network for Good, Shirley Miller, Reata Pharmaceuticals, and Chris Dokter! See ISN News for more donors. Click Here to Donate.


TOLL FREE HOTLINE, U.S. and Canada: 1-800-564-7099
Ask for our Free Info Packet by email or postal mail!

SCLERO.ORG is the world's leading nonprofit for trustworthy research, support, education and awareness for scleroderma and related illnesses.

We are a service of the nonprofit International Scleroderma Network (ISN), which is a 501(c)(3) U.S.-based public charitable foundation, established in 2002. Meet Our Team, or Volunteer. Donations may also be mailed to:

International Scleroderma Network (ISN)
7455 France Ave So #266
Edina, MN 55435-4702 USA
Email [email protected] to request our Welcome email, report bad links, or update page content. Disclaimer. Privacy Policy.

The most important thing in the world to know about scleroderma is!