| 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. Rather, it is a very difficult to detect elevation in pressure in specific arteries which take blood between the heart and the lungs. (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. |
| 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. (Primary Pulmonary Hypertension is pulmonary hypertension that occurs by itself, and not secondary to scleroderma. Also see: Pulmonary Hypertension ) |
| 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. |
| Pulmonary Hypertension in Correlation with Other Diseases |
| 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 ) |
| Asymptomatic Pericardial Disease in Scleroderma-Associated Progressive Interstitial lung disease (ILD) and its Relationship to Pulmonary Hypertension. Asymptomatic pericardial disease is common in patients with SSc-associated progressive ILD, and its presence is associated with a high risk of echocardiographically defined pulmonary hypertension. Aryeh Fischer. 372/372. ACR 2005. (Also see: Cardiac Involvement and Pulmonary Fibrosis) |
| Symptoms of Pulmonary Hypertension |
| Initially there may be no symptoms at all. 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. |
| Pulmonary Functional Abnormalities in Asymptomatic Patients with Systemic Sclerosis. Functional defects, mainly of the restrictive type, were observed in almost half of the asymptomatic patients with SSc. Pulmonary fibrosis and pulmonary hypertension are also findings in patients without clinical expression. Active detection of pulmonary involvement is recommended for all SSc patients. M. Nicola. AB0190 EULAR 2005. (Also see: Pulmonary Fibrosis) |
| 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. |
| 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... |
