| LUNG (PULMONARY): MAIN MENU |
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| Pulmonary Hypertension |
| This page was written by Shelley Ensz, and has not been medically edited. Scleroderma (SD) affects everyone differently. Just because something is listed here does not mean an individual patient will ever experience it. See Disclaimer |
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| What is Pulmonary Hypertension? |
| 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: Hypertension) |
| Pulmonary Hypertension (PH) is high blood pressure in the arteries which take blood between the heart and lungs. It can be caused by scleroderma and also by other diseases, such as pulmonary heart disease, pulmonary artery blood clots, mitral valve narrowing, and cirrhosis of the liver. (1) PH Association. |
| Types of Pulmonary Hypertension. Pulmonary Hypertension, or PH, is the general term used to describe high blood pressure in the pulmonary arteries from any cause. Pulmonary Arterial Hypertension, or PAH, is high blood pressure in the pulmonary arteries from diseases that affect the small vessels of the lung, causing them to narrow and impede blood flow. Pulmonary Hypertension Association. |
| PH Patients and Family Education Forum. This page provides an index of information on PH ranging from exercise to diet, testing and treatments. A wealth of information for anyone with PAH. Pulmonary Hypertension Association. |
| What is Pulmonary Arterial Hypertension? A resource for patients and physicians. Actelion Pharmaceuticals. |
| Pulmonary hypertension. A fatal disease if untreated, pulmonary hypertension is high blood pressure in the lungs. It causes shortness of breath - a symptom so common, the disease is often misdiagnosed. WIStv. 09/13/06. |
| What is Pulmonary Arterial Hypertension in Systemic Sclerosis (SSc)? PAH occurs in approximately one in seven scleroderma patients. If you suspect PAH, consider referring the patient to a centre with expertise in PAH. PAH-Info.com. |
| Primary Pulmonary Hypertension (PH) |
| When PH occurs by itself, it is called Primary Pulmonary Hypertension. |
| Pulmonary Hypertension (PH) is high blood pressure in the arteries that are between the heart and lungs. 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. |
| Primary Pulmonary Hypertension Is Not Associated With Scleroderma-Like Changes in Nailfold Capillaries. The prevalence of scleroderma-associated nailfold changes in patients with primary pulmonary hypertension (PPH) was dramatically lower than that in patients with scleroderma. The distribution of nailfold grades for the PPH patients was indistinguishable from that of the healthy control subjects. Chest. 2001;120:796-800. |
| Secondary Pulmonary Hypertension |
| Treatments— and their effectiveness—can vary depending on whether PH is primary or secondary. |
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. |
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| Isolated Pulmonary Hypertension in Systemic Sclerosis (Scleroderma) |
| Isolated Pulmonary Hypertension. In systemic sclerosis (scleroderma), pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) often occurs secondary to severe pulmonary fibrosis, kidney failure, or cardiomyopathy with diastolic dysfunction. When it occurs in scleroderma by itself, and not secondary to such other causes, it is referred to as isolated pulmonary hypertension. ISN. |
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| Exercised Induced Pulmonary Hypertension |
| Exercise-induced pulmonary hypertension common in systemic sclerosis. It is extremely important that these high risk patients (limited scleroderma with long-standing disease, patients with anti-centromere antibody, or a nucleolar pattern on ANA testing) have yearly pulmonary function tests and echocardiograms. (Reuters Health) Medical Imaging. 08/06/08. |
| Exercise Induced Pulmonary Arterial Hypertension (PAH) in Patients with Systemic Sclerosis. A 'positive' exercise test was defined as an increase of at least 20mmHg in the RVSP with exercise. A right heart catheterization with exercise was performed in those with a 'positive' exercise test. Exercise induced pulmonary hypertension is a common finding in patients at high risk for PAH. Steen V. (PubMed) Chest. 2008 Apr 10. |
| Causes of Pulmonary Hypertension |
| Causes of Pulmonary Hypertension. Depending on the type, pulmonary hypertension can have many potential causes. MayoClinic. |
| Effects of Iron Supplementation and Depletion on Hypoxic Pulmonary Hypertension Two Randomized Controlled Trials. Hypoxia is a major cause of pulmonary hypertension in respiratory disease and at high altitude. Hypoxic pulmonary hypertension may be attenuated by iron supplementation and exacerbated by iron depletion. Thomas G. Smith, MBBS, DPhil. JAMA. 2009;302(13):1444-1450. (Also see: Hypoxia, and Vitamins/Minerals) |
| Vascular Disease and Systemic Scleroderma. The vascular system is our circulatory system. It consists of all the vessels that carry blood and lymph through the body, to and from organs. Vessels include arteries, veins, and capillaries. Any condition that affects the vascular system, all of in part, is considered a vascular disease. ISN. |
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| Pulmonary Hypertension in Correlation with Other Diseases |
| Pulmonary hypertension in systemic sclerosis determines cardiac autonomic dysfunction assessed by heart rate turbulence (HRT). HRT assessment indicates a frequent impairment of cardiac autonomic nervous system in SSc patients. Moreover, older age in these patients and especially PH are independent significant predictors of cardiac autonomic dysfunction. Bienias P. (PubMed) Int J Cardiol. 2008 Dec 19. (Also see: Cardiac Involvement) |
| Right ventricular function in scleroderma-related pulmonary hypertension (SSc-PAH) . Recent echocardiographic and haemodynamic studies suggest that right ventriculer (RV) pump function and filling characteristics are altered in SSc-PAH as compared with idiopathic PAH. (UnBound MedLine) Vonk Noordegraaf A. Rheumatology (Oxford) 2008 Oct.:v42-3. (Also see: Cadiac Involvement) |
| Changes in Eye Pressure and Anterior Chamber Depth With Oral Endothelin Antagonist Therapy: Clinical Trials. We hypothesize that the rate of structural damage to the nerve fiber layer (NFL), a feature of glaucoma, is higher in patients with PH than expected in healthy populations lacking this disease. This study is currently recruiting participants. ClinicalTrials.gov University of Colorado at Denver and Health Sciences Center (Also see: Eye Involvement) |
| Elevated Plasma Adrenomedullin (ADM) and Vascular Manifestations in Patients with Systemic Sclerosis (SSc). Patients who had both severe Raynaud's Phenomenon (RP) and elevated pulmonary arterial pressure were found to have significantly higher ADM levels than patients who had neither manifestations and those with severe RP alone. Elevated ADM was found in SSc patients with increased pulmonary arterial pressure regardless of concomitant interstitial lung disease. J Rheumatol 2007;34:2224-9. (Also see: Raynaud's) |
| Pulmonary Hypertension (PH) and Thyroid Disease. Patients with PH have a higher prevalence of thyroid disease than other pulmonary patients. Evaluation of thyroid function in PH patients may be warranted to detect and assess coexisting thyroid disease. Chest, doi:10.1378/chest.07-0366. (Also see: Thyroid Disease) |
| 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. |
| What are common symptoms of Pulmonary Arterial Hypertension? Common early symptoms include breathlessness or shortness of breath (particularly on exertion), chronic fatigue, dizziness (especially when walking upstairs or when standing up), fainting, swollen ankles and legs, and chest pain (especially during physical activity). Actelion Pharmaceuticals. |
| Diagnosis of 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. |
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| Prognosis of Pulmonary Hypertension |
| Prognosis of Pulmonary Hypertension. Early diagnosis and treatment substantially improves the prognosis for pulmonary hypertension associated with systemic sclerosis (scleroderma). ISN. |
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| Treatments for Pulmonary Hypertension |
| Treatments include oxygen therapy, Tracleer (bosentan), Digitalis, diuretics, Flolan (in clinical trials), and, in a few cases, heart-lung or double-lung transplants. |
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| Pulmonary Hypertension Research Registries |
| Pulmonary Hypertension Research Registries include PHAROS and CoTherix. SureSteps Patient Support Program by Actelion is for patients on Tracleer or Bosentan. ISN. |
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| Pulmonary Hypertension Research |
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| Pulmonary Support Organizations |
| Pulmonary Support Resources ISN. |
| The American Lung Association and Myogen Announce Pulmonary Arterial Hypertension (PAH) Educational Partnership. They will collaborate on a new public awareness initiative to educate the public about PAH, a progressive and life-threatening disease that affects an estimated 200,000 patients worldwide. WebWire. American Lung Assoc. 09/06/06. |
| Pulmonary Hypertension Patient & Caregiver Stories |
| Pulmonary Hypertension Patient and Caregiver Stories provide vital support, and let others know they are not alone. ISN. |
| Woman battling hypertension. Shortly after her diagnosis, Mary Beth Schumacher went online to learn all she could about pulmonary hypertension. The lesson left her gasping. THonline.com. 11/22/08. |
| 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... |
| Read more pulmonary hypertension patient and caregiver story listings... |
