Interstitial Lung Disease-Books Pdf

Interstitial Lung Disease
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Interstitial Lung Disease Chapter 10, Prevalence of specific interstitial lung diseases in Bernalillo County New Mexico. 1988 1990 1, Interstitial lung disease diagnosis per 100 000 per 100 000. Idiopathic pulmonary fibrosis 20 2 13 2,Pulmonary fibrosis 10 1 14 3. Interstitial pneumonitis 1 8 2 8,Occupational environmental 20 8 0 6. Sarcoidosis 8 3 8 8,Connective tissue diseases 7 1 11 6.
Drug radiation 1 2 2 2,Pulmonary hemorrhage 0 6 2 2. Other 10 7 11 6,Total 80 9 67 2, excessive inflammatory cells Lung biopsies with a predominance of fibrosis. typically indicate advanced disease and poor prognosis whereas patients with. a predominance of inflammation have a better prognosis and often respond to. Although interstitial lung disease was once considered rare epidemiologic. investigations have found these diseases to be more common than previously. recognized One study reported that 80 9 per 100 000 men and 67 2 per 100 000. women suffer from interstitial disease in the United States with 31 5 new cases. diagnosed per 100 000 men per year and 26 1 new cases diagnosed per 100 000. women per year 1 In this study the most prevalent interstitial diseases included. pulmonary fibrosis occupational and environmental associated disease con. nective tissue disease associated interstitial disease and sarcoidosis. Interstitial lung disease is predominantly a disease of adults although it. also occurs in children Certain interstitial diseases such as sarcoidosis pulmo. nary Langerhans cell histiocytosis and autoimmune associated lung diseases. tend to develop in young adults whereas idiopathic pulmonary fibrosis IPF. most often occurs between the ages of 40 and 70 In those with familial IPF. Whitebook breathing Ch10 indd 100 6 25 10 12 48 21 PM. Chapter 10 Interstitial Lung Disease, defined by its presence in two or more first degree relatives the onset of fibro. sis appears to be earlier Both incidence and mortality of interstitial disease. increase with age 2 3, IPF has been reported to occur throughout the world in many different racial. and ethnic groups Studies in the United States have suggested that Cauca. sians are more likely to be diagnosed with IPF and have higher mortality rates. from IPF than African Americans 3 4 It is unclear if these findings are due to. real differences in racial characteristics or to an under diagnosis of this condition. in minority populations, The causes of interstitial lung disease can be classified into one of the fol.
lowing four categories 1 diseases associated with a condition that affects other. parts of the body for example autoimmune or collagen vascular disease. 2 diseases associated with a specific exposure to an agent known to damage. the lungs for example medications such as bleomycin occupational exposures. such as asbestos tobacco smoke or agents in the environment that cause an. immune reaction called hypersensitivity pneumonitis 3 diseases associated. with known genetic abnormalities for example Hermansky Pudlak syndrome. and 4 idiopathic diseases diseases of an unknown cause The most common. interstitial lung diseases are idiopathic 5, Two recent studies found that both the number of new cases diagnosed per. year and the mortality rates for idiopathic pulmonary fibrosis are rising in the. United States 3 6 Investigators from the United Kingdom have reported simi. lar trends from 1990 to 2003 the incidence of this disease more than doubled 7. Of even greater concern mortality rates from idiopathic pulmonary fibrosis are. expected to increase because there is no established treatment that prolongs. life for patients with these diseases 3, Persons with exposure to environmental hazards for example asbestos. have a higher incidence of interstitial lung disease although less is known about. most of the other forms of interstitial disease Although not all patients who. develop IPF have a history of cigarette smoking smoking has been associated. with the development of disease Exposure to metal and wood dusts has also. been associated with an increased likelihood of developing IPF Although data. are limited other possible risk factors include exposure to certain prescription. drugs and chronic gastroesophageal reflux disease Genetics play a role in the. development of the familial cases of IPF About 8 percent of familial cases can. be attributed to a single set of genes 8, Whitebook breathing Ch10 indd 101 6 25 10 12 48 21 PM. Interstitial Lung Disease Chapter 10,CASE STUDY, An otherwise healthy 59 year old physical therapist sought medical. attention for increasing shortness of breath and a dry cough over a year. He had no other symptom no exposure to an environmental cause of. interstitial lung disease no medication associated with interstitial lung. disease no family history of lung disease and no history of smoking The. physical examination revealed abnormal breath sounds at the bases of his. lungs crackles but was otherwise normal Crackles are subtle sounds. made by the opening of the smallest air spaces with inspiration which. indicate that they close on expiration an abnormality Pulmonary function. tests showed decreased lung volume a restrictive pattern and low blood. oxygenation hypoxemia on exertion A computed tomography scan. showed the characteristic pattern of IPF As there was no proven effective. medical therapy for IPF the patient elected to participate in a clinical trial. He was referred for lung transplant evaluation started on supplemental. oxygen and enrolled in a pulmonary physical rehabilitation program. As in this case IPF typically develops insidiously with a gradual onset of. shortness of breath and a nonproductive cough The symptoms often. progress to become debilitating and all consuming Although not necessary. in this patient a tissue biopsy is often needed to reach a conclusive. diagnosis Interstitial diseases are generally difficult to treat because. established fibrosis causes permanent structural changes of the lungs. What are we learning about this disease, Pathophysiology causes genetic environment microbes.
Inflammation is a characteristic cellular and molecular response to injury nox. ious exposure or infectious agents Fibrosis or scarring can be part of the. healing response to injury but fibrosis as a disease process occurs when resto. ration to normal tissue does not occur Many of the cells and chemical mediators. involved in inflammation are also involved in fibrosis Fibrosis occurs when the. laying down of collagen and other connective tissue does not stop and allow a. return to the normal structure This activity could result from ongoing inflammation. Whitebook breathing Ch10 indd 102 6 25 10 12 48 21 PM. Chapter 10 Interstitial Lung Disease, possibly because the injurious events continue or because of defects in the. inflammatory or repair processes When the injurious agent is known the extent. of the injury and time of exposure to the injurious agent are important determi. nants in disease outcome, More is known about the interstitial diseases with known causes such as. the occupational exposures For example it appears that minerals for example. asbestos and silica that cause interstitial lung disease directly injure the lung. and cannot be easily eliminated Thus ongoing inflammatory and fibrotic reac. tions occur In patients with farmer s lung recurrent exposure to the offending. particles antigens stimulates the immune system recurrently which results in. fibrosis A similar recurrent immune stimulation probably occurs with the autoim. mune diseases such as rheumatoid arthritis For most of the interstitial lung. diseases of unknown cause the mechanisms of lung injury and fibrosis are also. unknown In several interstitial diseases viral infection has been postulated to. be the inciting cause but this association has not yet been proven. How is it prevented treated and managed,Prevention treatment staying healthy prognosis. When the cause of the disease is known the injurious agent should be avoided. For example with hypersensitivity pneumonitis one should avoid dust and mold. With idiopathic disorders because the cause is unknown there is no known way. to prevent them However a number of possible risk factors for disease have. been reported and abstaining from cigarette smoking and treating gastroesoph. ageal reflux disease are recommended, As it has been long thought that inflammation precedes fibrosis therapeutic. regimens for interstitial lung disease have included corticosteroids for example. prednisone and immunosuppressive agents for example azathioprine and. cyclophosphamide These drugs are helpful in cases of connective tissue. related lung disease and certain other interstitial lung diseases In patients with. severe fibrosis treatments targeting inflammation however have not been. shown to improve survival or quality of life One study did find that patients with. IPF treated with high dose N acetyl cysteine an antioxidant in addition to a. standard regimen of corticosteroids and azathioprine had better lung function. compared to those on the standard regimen alone 9 Other drugs are being. Whitebook breathing Ch10 indd 103 6 25 10 12 48 21 PM. Interstitial Lung Disease Chapter 10, Lung transplantation is the only option shown to prolong survival in cases of.
advanced interstitial lung disease especially IPF and is possible for patients. younger than 65 without other significant medical conditions However lung. transplantation outcomes remain unsatisfactory as the five year survival rate is. approximately 40 percent and the median survival is 3 9 years in post transplant. patients with IPF 10 Of those patients with IPF who are waiting to receive. a transplant more than 30 percent die before receiving one In 2007 IPF sur. passed chronic obstructive pulmonary disease as the most common diagnostic. group to receive a lung transplant 11 further highlighting the urgent need for. effective medical therapies for this and related progressive lung diseases. Interstitial lung diseases have varying prognoses Sarcoidosis usually has a. good prognosis with reversal of disease in most cases On the other hand IPF. one of the most common interstitial lung diseases has the worst prognosis with. a median survival of two to three years 2,Are we making a difference. Research past present and future, An understanding of the mechanisms of the idiopathic forms of interstitial lung. disease is only now emerging Studies of cells in culture and in animals have. revealed a number of molecules and molecular pathways such as transforming. growth factor beta that promote fibrosis It appears that an unidentified proba. bly inhaled agent may injure the lung and activate cells such as macrophages. and airway lining epithelial cells The activated cells then produce factors that. recruit immune cells into the lung The immune cells produce or activate sets of. molecules that in turn activate other molecules to stimulate other cells called. fibroblasts to produce and deposit collagen and other connective tissue compo. nents which constitute lung fibrosis, Studies of the genetic background of patients show that alterations in spe. cific genes for example surfactant protein genes and telomerase may predis. pose individuals to IPF Further evidence for the role of genetics in interstitial. lung disease comes from studies in patients with other disorders for example. sarcoidosis and Hermansky Pudlak syndrome where mutations in specific. genes are associated with a higher incidence of lung fibrosis 12 Further. research on these rare diseases caused by a single gene defect may shed light. on disease processes that are also important in interstitial lung disease. Whitebook breathing Ch10 indd 104 6 25 10 12 48 22 PM. Chapter 10 Interstitial Lung Disease,The end result of. interstitial lung disease is,fibrosis in which scar.
tissue accumulates and air,spaces break down into,large cysts a process. sometimes called,honeycombing,Dean E Schraufnagel, What do we need to do to cure or eliminate interstitial lung disease. Although considerable progress has been made in understanding these condi. tions curing and eliminating interstitial lung disease is still a distant goal A. clearer understanding of how the cells fail to adequately repair the lung is still. needed Understanding basic mechanisms should lead to better markers to. diagnose and follow patients With these much needed markers therapeutic. trials will be easier and more cost effective to conduct Current clinical trials are. studying agents that reduce the fibrotic signaling within the lung reduce pulmo. nary hypertension associated with interstitial lung disease and alleviate oxida. tive stress These clinical studies are usually coupled with basic science studies. to learn more about the mechanisms of disease and to develop biological. Whitebook breathing Ch10 indd 105 6 25 10 12 48 26 PM. Interstitial Lung Disease Chapter 10,ILD has affected many notable A mericans. Michael Ochs Archives Getty Images,Steve Zusy Getty Images. Darren McCollester Getty Images,Hulton Archive Getty Images.
Interstitial lung disease is killing more Americans each year Former Utah Governor Olene Smith Hulton Archive Getty Images. Walker top left is living with the disease Others unfortunately have died of the disease including. passed chronic obstructive pulmonary disease as the most common diagnostic group to receive a lung transplant 11 further highlighting the urgent need for effective medical therapies for this and related progressive lung diseases Interstitial lung diseases have varying prognoses Sarcoidosis usually has a

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