SCLERO.ORG
Search

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.

"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)

Biomarkers of pulmonary hypertension (PH) in patients with scleroderma: a case–control study. Our study demonstrated clear associations between regulators of angiogenesis (sFlt-1 and PlGF) and measures of PH in scleroderma and that these growth factors are potential biomarkers for PH in patients with scleroderma. PubMed, Arthritis Res Ther, 2015 Aug 6;17:201.

A comparison of the predictive accuracy of three screening models for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc). In this cohort, the DETECT and Australian Scleroderma Interest Group algorithms out–perform the European Society of Cardiology/European Respiratory Society guidelines, detecting all patients with PAH. PubMed, Arthritis Res Ther, 2015 Jan 18;17:7.

PH (Pulmonary Hypertension) and Scleroderma. In scleroderma, 10–15% of patients have evidence of pulmonary arterial hypertension and another 10–15% of patients have pulmonary hypertension as a complication of interstitial lung disease and/or left ventricular dysfunction. Pulmonary Hypertension Society.

PH Secondary to Scleroderma: Dyspnea (Shortness of Breath)

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

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, 2014 Apr 4;14:57.

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

Cardiopulmonary exercise testing (CPET) for detecting pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc). CPET is a safe and valuable method in the non-invasive detection of SSc–associated PAH and it may be particularly beneficial for reducing unnecessary right heart catheterisation procedures. PubMed, Heart, 01/06/2017. (Also see Cardiac Involvement)

Go to Pulmonary Hypertension Personal Stories
 
Recent Donors
See ISN News for recent donors, including memorials for
Christine Kane, Lilian and Lee Stiles, Charles Kendrick Watts and Marta Marx.
 

SCLERO.ORG is the world leader for trustworthy research, support, education and awareness for scleroderma and related illnesses, such as pulmonary hypertension. 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, or to report bad links or to update this page content.

TOLL FREE HOTLINE 800-564-7099
Free to U.S. and Canadian Callers. Ask for our Free Info Packet by mail or email!
Scleroderma, Pulmonary Arterial Hypertension, and related illnesses.
Privacy Policy.

 
The most important thing in the world to know about scleroderma is sclero.org!
Donate Now
Copyright 1998-2017, International Scleroderma Network. AKA Scleroderma from A to Z and SCLERO.ORG. All Rights Reserved.