|Overview of Secondary PH/PAH
PH Secondary to MCTD
|PH Secondary to Pulmonary Fibrosis
PH Secondary to Scleroderma
PH Secondary to Scleroderma: Dyspnea
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.
"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)
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).
Pulmonary Fibrosis. Pulmonary Hypertension can occur in pulmonary fibrosis, even without severe lung dysfunction or hypoxemia (low oxygen in the blood.) ISN.
|Living with PF
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.
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.
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)
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