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Secondary Pulmonary Hypertension

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
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

Overview

Treatments— and their effectiveness—can vary depending on whether PH is primary or secondary.

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.

Antiphospholipid Antibodies (APLA) Markers in Secondary PH with Scleroderma

Antiphospholipid antibodies are markers for pulmonary hypertension in scleroderma patients.

(Expired Article) "Listen to the Patient" Anticoagulation Is Critical in the Antiphospholipid (Hughes) Syndrome (APS). Affecting, almost uniquely, both veins and arteries, the clinical features (of APS) range through deep vein thrombosis (DVT), chronic leg ulcers, recurrent miscarriages, headache, heart attacks, renal vein and artery thrombosis, to pulmonary embolism and even pulmonary hypertension. The Journal of Rheumatology. (Also see Antiphospholipid Syndrome, Leg Ulcers, Pregnancy and Scleroderma, and Cardiac Involvement)

PH Secondary to Mixed Connective Tissue Disease (MCTD)

Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort. Results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature. The prevalence of PH is much lower than expected from previous studies but confirm the seriousness of the disease complication. Rheumatology (Oxford).

PH Secondary to Pulmonary Fibrosis

Pulmonary Fibrosis. Pulmonary Hypertension can occur in pulmonary fibrosis, even without severe lung dysfunction or hypoxemia (low oxygen in the blood.) ISN.

Overview
Mortality/Prognosis
Symptoms
Disease Correlations
Diagnosis
Living with PF
Treatments
Research
Patient Stories
References

PH Secondary to Systemic Sclerosis (SSc, or Scleroderma)

Reduced right ventricular output reserve in patients with systemic sclerosis (SSc) and mildly elevated pulmonary arterial pressures (mPAP). These findings give further evidence for the clinical relevance of mildly elevated mPAP in patients with SSc. PubMed, Arthritis Rheumatol, 01/07/2019.

Angiogenic and inflammatory biomarkers for screening and follow-up in patients with pulmonary arterial hypertension. Plasma levels of PlGF, sVEGFR-1, TNF-α, and VEGF-D have potential in screening for SSc-associated PAH. PubMed, Scand J Rheumatol, 2018 Jul;47(4):319-324.

PH Secondary to Scleroderma: Dyspnea (Shortness of Breath)

Myositis overlap is another very common cause of shortness of breath in scleroderma patients, besides PH.

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 Pulmonary Hypertension)

Increased respiratory drive relates to severity of dyspnea in systemic sclerosis (SSc). In SSc patients an abnormal V'E/P0.1 better relates to the severity of dyspnea than traditional lung function parameters and can easily be assessed at first outpatient consultation. PubMed, BMC Pulm Med.

Pulmonary fibrosis is a frequent cause of PH in scleroderma patients.

Cardiopulmonary exercise testing (CPET) in a combined screening approach to individuate pulmonary arterial hypertension in systemic sclerosis (SSc). In association with the DETECT algorithm, CPET may be considered as a useful tool in the workup of SSc-related pulmonary hypertension. PubMed, Rheumatology (Oxford), 2020 Jul 1;59(7):1581-1586. (Also see Cardiac Involvement)

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