Pathology and Treatment of Pulmonary Arterial Hypertension

Abnormal immune function in both forms of scleroderma is defined as an alteration in cytokine function and the activation of T-cells with the presence of auto-antibodies (Woo, Laxer & Sherry, 2007). IL-4 and IL-6 stimulate collagen synthesis by the activation of fibroblasts, increasing production of collagen type I and III. Other factors that are thought to mediate fibroblast activation include growth factors such as platelet-derived growth factor and connective tissue growth factors. Additionally, the production of tissue inhibitors such as matrix metalloproteases has been noticeably upregulated therefore, fibrosis is thought to be a result of not only the increased levels of collagen production but also the decreased levels of cellular matrix turnover (Mayes, 2014). Localised scleroderma will appear with dyspigmented, atrophic sclerotic lesions generally present on the extremities, back, trunk, neck, head and scalp, where as the first manifestation present in systemic scleroderma is oedema of the hands, causing restricted movement followed by skin

Scleroderma, also called Progressive Systemic Sclerosis, is a chronic connective tissue disease in which excessive amounts of collagen are rapidly deposited into

Thousands of Americans are diagnosed with Scleroderma every year across the nation. Scleroderma, also known as Systematic Sclerosis, is an over production of collagen in body tissues causing patches of skin to tighten and harden and can harm other structures in the body.

SHealth is one thing I think we take for granted when we are young. We eat what we want, sleep when we want and do what we want. I’ve learned your body is only as good to you to you as you are to it. Yes, I love the times overseas drinking at all hour and eating grease foods thinking it was the cure for a hangover. But I had no idea how it was affecting my body. I was first diagnosed with high blood pressure at the age of twenty-three. I laughed at it because I did not understand the seriousness of it. It took family members including my mother to pass to see how dangerous the life I was living was and or will be. So I want to change. In this essay, I am going to outline how I am going to change my behavior by Describe the expected outcome

Systemic Sclerosis (SSc) is a complex, polygenic autoimmune disease that affects approximately 75,000 to 100,000 people in the United States. There are approximately 20 new cases per million per year and a total of 250 patients per million [1, 2]. SSc typically presents at approximately 40 years of age and is more common in women than in men [3]. SSc is a very heterogeneous disease where patients can display a variety of symptoms: the three most common symptoms are tissue and organ fibrosis, vasculopathy and autoimmunity/autoantibody production.

A lung or heart transplant is then recommended for patients whose condition doesn’t improve or worsens. The survival rate according to nurse Vacca for patients receiving lung transplant is 74% after 1 year and 45% after 5 years of receiving the new lung. However, he said the wait time for donor lung can take up to 3 years but sadly patients with PAH can not wait that long. Therefore nurse Vacca emphasized the importance of educating families and patients with PAH the proper management of this condition in order for patients to live longer and have a better quality of life. Some of the examples he mentioned in the proper management of this condition includes proper administration of medication including knowing its side effects, appropriate planning of activities and rest, reducing fluid retention by following a low sodium diet, daily weight monitoring and being alert in recognizing peripheral edema, the use of supplemental oxygen if needed, and knowing when to contact a health care provider upon onset of symptoms. Most importantly, encourage patients to discuss end of life issues with their family and/or social services and support

Systemic sclerosis, also known as, scleroderma or CREST syndrome is a chronic inflammatory, autoimmune connective tissue disease. Scleroderma is a connective tissue disease that involves changes in the skin, blood vessels, muscles, and internal organs. This disease can be classified into two main types, systemic or localized. Systemic scleroderma can affect all parts of the body, such as internal organs, skin, and blood vessels. Localized only affects the skin and is not life threatening, as systemic can be. Scleroderma can be progressive and it involves the skin and connective tissues that tighten and harden. It is a type of autoimmune condition that occurs when the immune system misguidedly attacks and destroys healthy body tissue.

LeRoy EC, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J. Rheumatol. 1988; 15: 202–205.

Also, the capillaries of the lungs help indirectly to control blood volume and blood pressure through the conversion of angiotensin I to angiotensin

Systemic sclerosis (SSc) is a heterogeneous disease, with early involvement of the vascular endothelium. Among other factors, inflammation and oxidative stress contribute to the endothelial dysfunction, one of the most important pathogenic pathways in the development of SSc. The increase of TNF (tumour necrosis factor)-alpha expression induces the production of ROS (reactive oxygen species), resulting in endothelial dysfunction in many pathophysiological conditions, including SSc. Myeloperoxidase (MPO) is a hem enzyme, released by activated neutrophils, monocytes and macrophages. Lipoprotein-associated Phospholipase A2 (Lp-PLA2) is an enzyme secreted by various inflammatory cells. MPO and Lp-PLA2 activities were found to be correlated with endothelial dysfunction.

The definite haemodynamic diagnosis of pulmonary hypertension (PH) requires right heart catheterisation and direct measurements of right ventricular, pulmonary artery and pulmonary capillary wedge pressures and cardiac output. As this is an invasive test with risk and cost implications diagnostic algorithms have been devised that combines clinical assessment, non-imaging cardio-respiratory assessment and non-invasive imaging in patients suspected of having pulmonary hypertension. The initial aim is to establish a tentative diagnosis of pulmonary hypertension, to assess its severity and to identify the underlying aetiology. Subsequently non-invasive imaging techniques are used in monitoring disease progression as well as response to treatment in patients with pulmonary hypertension as an alternative to repeat invasive right heart catheterisation. In the future these imaging techniques may also help us to understand the cellular and molecular mechanisms responsible for the pathophysiology of pulmonary hypertension in both the pulmonary circulation and the right ventricle (RV).

Pulmonary hypertension is a group of disorders that is known to increase blood pressure.1 According to the World Health Organization (WHO), there are five pulmonary hypertension groups. Pulmonary arterial hypertension (PAH) is classified as Group 1, with a mean artery pressure of 25 mmHg or greater while at rest or left ventricular end-diastolic pressure of 15 mmHg or less by cardiac catheterization. One of the medications, sildenafil, is used to treat pulmonary arterial hypertension and erectile dysfunction.2

arteries due to that veins has much thinner vessel walls. Pulmonary vein returns oxygenated blood

Radiographic features with heart failure will demonstrate changes in cardiac output and pulmonary venous pressure, evident in dilated pulmonary vessels, interstitial, pleural and alveolar fluid leakage, and increased systemic venous pressure with chronic disease (Cremers, Bradshaw & Herfkens, 2010). Based on the pathophysiological process present with Mrs. Smith, some of the following features might be noticeable: Redistributed pulmonary blood flow will be appreciated through an increased (normal 1-2 mm) artery-to-bronchus ratio at the hilar level in the upper and middle lobes; since gravity and positioning (supine versus erect and decubitus) will have an impact, comparison with serial or old films if existing will be helpful (Cremers et al.,

for each are vastly different. We believe this case series may provide insight into PH