Exercised Induced PH
Pulmonary Hypertension is not the same as "regular" high blood pressure or hypertension, which can be easily measured with a blood pressure test.
Pulmonary hypertension is a high pressure in the arteries between the heart and lungs or pulmonary arteries. It is very difficult to detect elevation in pressure in these arteries. Pulmonary Hypertension is a type of vascular disease that can be found in scleroderma patients. (Also see Scleroderma Lung Involvement, What is Scleroderma?, Types of Scleroderma, and Systemic Sclerosis)
Causes of Pulmonary Hypertension. There are dozens of causes of pulmonary hypertension, ranging from genetics, medications or street drugs, connective tissue diseases (such as scleroderma), heart and lung diseases, and blood disorders. ISN.
Altitude and Hypoxia
Estrogen and Enzyme CYP1B1
Primary pulmonary hypertension occurs by itself and not secondary to another illness, such as scleroderma. Nailfold capillary examination can easily tell whether pulmonary hypertension is primary or secondary. (Also see Nailfold Capillaroscopy)
Secondary Pulmonary Hypertension. 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. ISN.
|Overview of Secondary PH/PAH
PH Secondary to MCTD
|PH Secondary to Pulmonary Fibrosis
PH Secondary to Scleroderma
PH Secondary to Scleroderma: Dyspnea
Response to exercise in patients with pulmonary arterial hypertension (PAH) treated with combination therapy. We investigated pulmonary haemodynamic response to exercise, and the relationship to ventilatory efficiency and hypoxaemia in patients with PAH treated with combination therapy. PubMed, ERJ Open Res, 2021 Jan 25;7(1):00725-2020.
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 Secondary Pulmonary Hypertension)
The incidence rate of pulmonary arterial hypertension (PAH) and scleroderma renal crisis (SRC) in systemic sclerosis patients with digital ulcers on endothelin antagonist receptors (ERAs) and phosphodiesterase-5 inhibitors (PDE5i). Treatment with ERAs and/or PDE5i appeared to delay the occurrence of SRC. PubMed, Rheumatology (Oxford), 2020 Aug 26;keaa401. (Also see Kidney (Renal) Involvement and Research on Digital Ulcers)
Complement alternative pathway activation associated with pulmonary hypertension in lupus nephritis patients. Activation of the complement alternative pathway may be involved in the pathogenesis of pulmonary hypertension in lupus nephritis. PubMed, Lupus, 2019 Jul 11:961203319860192. (Also see Symptoms and Complications of Lupus)
Prevalence of auto–antibodies associated to pulmonary arterial hypertension in scleroderma – A review. The available evidence points in the direction of a strong association between auto–immune mechanisms and pulmonary hypertension in the setting of scleroderma. PubMed, Autoimmun Rev, 10/11/2018. (Also see Antibodies)
Pulmonary arterial hypertension (PAH) in the setting of scleroderma (SSc) is different than in the setting of lupus: A review. This review highlights the differences between SSc–PAH and Lupus–PAH. PubMed, Respir Med, 2018 Jan;134:42-46.
Relationship of main pulmonary artery diameter to pulmonary arterial pressure (PH) in scleroderma patients with and without interstitial fibrosis. In scleroderma patients, an enlarged main pulmonary artery predicts PH even in the presence of mild to moderate fibrosis. PubMed, J Comput Assist Tomogr. (Also see Correlation of Pulmonary Fibrosis and Pulmonary Hypertension)
Diagnosis of Pulmonary Hypertension. Doppler echocardiograms are notoriously inaccurate when the PH is mild or when there is simultaneous presence of lung scarring. PH cannot be diagnosed without right heart catheterization. ISN.
DLCO in PH
|Markers for PH in Scleroderma
Right Heart Catheterization
Prognosis of Pulmonary Hypertension. Early diagnosis and treatment substantially improves the prognosis for pulmonary hypertension associated with systemic sclerosis (scleroderma). ISN.
| Overview of PH Prognosis |
Functional Classification of PH
| Markers for Prognosis of PH |
Personal Stories of Prognosis of PH
Treatments include oxygen therapy, Tracleer (bosentan), Digitalis, diuretics, Flolan (in clinical trials), and, in a few cases, heart-lung or double-lung transplants.
Inhaled Prostacyclin (Ventavis, Iloprost)
|PDE 5 Inhibitors
Stem Cell Transplant
Pulmonary Hypertension Research Registries include PHAROS and CoTherix. SureSteps Patient Support Program by Actelion is for patients on Tracleer or Bosentan. ISN.
|PH Research Panel (by WWMR)
PH Registry of Scleroderma (PHAROS)
| Scleroderma Research Registries
Pulmonary Support Organizations
Pulmonary Hypertension Patient and Caregiver Stories provide vital support, and let others know they are not alone. ISN.
Archie Bailey: Scleroderma: Sue's Story Time has passed since Sue's death. Only those who have experienced the grieving process can understand the profound agony death presents to friends and loved ones…
Bill Jordan: Surviving Boyfriend of Scleroderma Patient Wendy was heartset on dancing that evening even though she was connected to an oxygen machine…
Brother Alan: Systemic Scleroderma I was diagnosed with Scleroderma three years ago and "too many times ten" doctors later, here I am…
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