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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)

Patients with scleroderma require 'annual screening' for pulmonary arterial hypertension. Every patient with systemic sclerosis should be screened for pulmonary arterial hypertension every year, as early recognition, referral and aggressive treatment are necessary to improve long–term outcomes, according to Virginia Steen, MD. Healio Rheumatology, 05/22/2019.

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. familydoctor.org.

Diagnostic Algorithm and Clinical Characteristics for Pulmonary Hypertension with Scleroderma

Use of red cell distribution width (RDW) in a population at high risk for pulmonary hypertension (PH). The ease of obtaining RDW as a biomarker may help detect incident PH at earlier stages among patients who are at high risk for development of PH. PubMed, Respir Med, 2019 Apr;150:131-135.

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, 2016 Apr;68(4):1004-12.

DLCO in Pulmonary Hypertension

Value of lung diffusing capacity for nitric oxide in systemic sclerosis. Decreased DLCO in the absence of lung restriction does not allow to suspect pulmonary arterial hypertension without fibrosis. PubMed, Physiol Rep, 2019 Aug;7(13):e14149.

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.

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)

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
 

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