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Pulmonary Hypertension Diagnosis in Systemic Sclerosis (Scleroderma)

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Video Overview
Diagnostic Algorithm
DLCO in PH
Echocardiograms
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)

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)

The inclusion of N-terminal pro-brain natriuretic peptide(NT-proBNP) in a sensitive screening strategy for systemic sclerosis-related pulmonary arterial hypertension(SSC-PAH): a cohort study. The combination of NT-proBNP with pulmonary function tests is a sensitive, yet simple and non-invasive, screening strategy for SSc-PAH. PubMed, Arthritis Res Ther, 2013 Nov 19;15(6):R193.

Screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension (PAH). Our systematic review revealed that most evidence exists for transthoracic echocardiogram (TTE), pulmonary function tests, and NT-ProBNP for screening and diagnosis of systemic sclerosis-PAH; however, more robust studies are needed. PubMed, Semin Arthritis Rheum, 2013 Sep 5.

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. familydoctor.org.

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

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)

Clinical Characteristics of Scleroderma Patients With and Without Pulmonary Hypertension. Scleroderma patients with pulmonary hypertension also present with more severe respiratory restriction and more impaired exercise capacity. Chest, 2013.

DLCO in Pulmonary Hypertension

Relevance of Partitioning DLCO to Detect Pulmonary Hypertension in Systemic Sclerosis. Partitioning of DLCO might be of interest to detect precapillary pulmonary hypertension (PH) in systemic scleroderma (SSc) patients with or without interstitial lung disease (ILD). PLOS, 10/18/2013.

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, 2014 Aug;20(5):268-74.

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)

Pulmonary Arterial Hypertension (PAH) In Systemic Sclerosis (SSc): Association With Nailfold VideoCapillaroscopy (NVC) And Biomarkers Of Vascular Damage. Our study suggests that more severe NVC abnormalities are significantly associated with PAH. Ann Rheum Dis, 2011;70 (Suppl3):473.

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. 2006 Aug 17.

Right Heart Catheterization (RHC) for Pulmonary Hypertension

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

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)

Lack of Specificity of the 6-Minute Walk Test as an Outcome Measure for Patients with Systemic Sclerosis. The 6MWD relates to broad factors in SSc and these results raise doubts about the specificity of the 6MWD in this systemic disease, and its relevance to monitoring therapy. The Journal of Rheumatology, Vol. 36 No. 7 1481-1485. July [2009]. (Also see Pulmonary Fibrosis)

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