Re: Diagnosing Pulmonary Fibrosis

[Guest guest]

... I'm saving this one! This is really good and covers everything in a way I could understand. (Most of it).

Thanks for sending it along to us.

MamaSher, age 69.IPF 3-06,NSIP 4-08. OR.Don't fret about tomorrow, God is already there!

Diagnosing Pulmonary Fibrosis

Read this article that I thought was very good so passing it on to you guys P:

>> Diagnosing Pulmonary Fibrosis > Environmental factors and genetics increase the risk of developing > this fatal disease > By Bronzell-Wynder, NP > > > Mr. is a 60-year-old man referred to a pulmonologist after > preadmission testing for a total knee replacement. His chest > radiograph proves abnormal, showing bilateral reticular opacities. > His knee surgery is cancelled, and he is referred to a lung > practitioner. > > A review of systems during the lung practitioner's assessment reveals > Mr. ' progressive shortness of breath the past year. On visual > assessment, he has digital clubbing with cyanosis of his fingertips > and lips. On auscultation, he has bibasilar crackles. The lung > practitioner orders several tests, which reveal pulmonary fibrosis.> > Definition> > Pulmonary fibrosis is one of many interstitial lung diseases that > scar lung tissue. An incurable, restrictive lung disease, it causes > scarring and fibrosis between the alveoli (chronic interstitial > inflammation). Scarring and stiffening of the alveoli decreases > transport of oxygen across the alveolar membrane. > > One clear sign of pulmonary fibrosis on CT scan is honeycombing and > scarring, which irreversibly lessen elasticity and lung compliance.1> > Pulmonary fibrosis is known by different names, including idiopathic > pulmonary fibrosis (IPF), defined by progressive dyspnea and > declining pulmonary function test.2 Chest radiographs display > interstitial infiltrations and biopsy shows fibrosis, inflammation or > both. > > Idiopathic interstitial pneumonias fall into eight categories. > > Desquamative interstitial pneumonia (DIP): A rare form of fibrosis. > Average age at onset is 42. Of these patients, 90 percent have a > smoking history, and 75 percent respond to steroids. > > Bronchiolitis obliterans > > Organizing pneumonia > > Cryptogenic organizing pneumonia (CFA) > > Respiratory bronchiolitis interstitial lung disease (RBILD): Takes on > the same process of DIP. Average age at onset is 36.1,2 > > Acute interstitial pneumonia (AIP): Acute onset with rapid > progression of shortness of breath and respiratory failure. Life > expectancy is 6 months. > > Nonspecific interstitial pneumonia (NSIP): More common in women. > Average age at onset is 49. Inflammation is more common than > fibrosis. > > Lymphangioleiomyomatosis (LAM): A rare form of pulmonary fibrosis > affecting only women. Average age at onset is 34. > Other disease processes can cause pulmonary fibrosis, including > lupus, rheumatoid arthritis, scleroderma and sarcoidosis.> > Epidemiology > > Studies show that 13 to 20 people per 100,000 develop interstitial > lung diseases, including an IPF prevalence of 7.4 women per 100,000 > and 10.7 men per 100,000.1> > Pulmonary fibrosis typically affects adults 40 to 70 years of age. > About two-thirds are over age 60. The mean age at diagnosis is 66. > Incidence increases after age 75, to 175 per 100,000. Overall death > increases at age 75, as well, with the mean length of survival > following diagnosis at 3 to 5 years.1 > > Unfortunately, pulmonary fibrosis patients, not knowing their > condition for years, attribute shortness of breath to other causes, > such as weight gain, aging and lack of exercise. As a result, the > condition is often diagnosed in the late stages after a comparatively > minor medical event, for example, flu requiring hospitalization. > After diagnosis, life expectancy for most patients is 2 to 4 years, > with the 5-year survival rate at 30 percent to 50 percent.1> > Risk factors > > There are six known risk factors for pulmonary fibrosis: > > cigarette smoking > > environmental factors> > genetic predisposition> > chronic aspiration > > infectious agents > > some prescription medications.1 > While cigarette smoking plays a role in the development of pulmonary > fibrosis, it has actually extended survival in some patients, > compared with nonsmokers or former smokers.3 > > A likely explanation is that these patients may seek medical > attention earlier for smoking-related symptoms.3 These patients also > can have more digital clubbing and less crackling on examination, > increased incidence of pulmonary hypertension on chest radiograph and > decreased spirometry and diffusion capacities on pulmonary function > tests.3 > > Environmental factors that increase the risk of developing pulmonary > fibrosis include exposure to solvents and to dust from metal and > wood, and atopy.4> > Genetic predisposition also may be a factor. No specific genetic > markers have been linked to IPF, but there have been cases of two > immediate family members having IPF, though this is currently being > researched.> > Chronic aspiration is another risk factor for IPF. These patients > tend to aspirate secondary to gastric reflux disease. However, it is > still not clear how chronic aspiration leads to IPF. > > Several infectious diseases can lead to IPF, including Legionnaires > disease, herpes virus, parainfluenza 3 virus, HIV-1, hepatitis C, > cytomegalovirus, measles and the Epstein Barr virus.1,5 Some studies > evaluated DNA markers of CMV, EBV, HHV-7 and eight and have shown > that patients with these viruses were at an increased risk for > pulmonary fibrosis.5> > Antidepressants and tricyclic medications may lead to pulmonary > fibrosis.6 Dothiepin and imipramine have shown to increase incidence > of IPF, but how these medications affect the disease process is not > known.6 > > Other medications may cause pulmonary fibrosis using different > mechanisms. They include amiodarone, amphotericin B, nitrofurantoin, > methotrexate, bleomycin, vinca and alkaloids. Prolonged use of > amiodarone leads to pulmonary toxicity. Chemotherapeutic medications > such as bleomycin and vinca alkaloids, can cause pulmonary fibrosis > in 10 percent of the population. Methotrexate, standard dosaging in > patients with arthritis and nitrofurantoin, also can cause pulmonary > fibrosis.7 Other medications can lead to pulmonary fibrosis, with > high doses or chronic use related to the disease process. > > IPF does not have a known etiology unless the causative agent is > biopsied from the lung.> > Pathophysiology > > Pulmonary fibrosis is caused by stimuli, or exposure to a substance > that triggers a triad of responses.2 First, the stimulus is > introduced causing chronic inflammation within the lung tissue, which > releases cytokines that eventually lead to lung injury. This is > caused by development of increased fibroblast activity, which leads > to fibrosis of the lung parenchyma.2,8 > > A new hypothesis outlines how pulmonary fibrosis developes: First, > the patient is exposed to a stimulus, which initiates lung injury > that can cause inflammation depending on the patient's dominant > inflammatory phenotype. Eventually, th lung tissue begins to heal > causing fibrosis.2 Some of the factors modifying the > patient's "fibrotic" response are a history of smoking, environmental > exposures and viral infections.> > Clinical presentation > > Patients with pulmonary fibrosis will complain of a cough with or > without mucus, and about 25 percent to 44 percent will have digital > clubbing. The most common and insidious side effect is progressive > shortness of breath. On auscultation, bibasilar inspiratory crackles > can be heard. > In more advanced cases, right-sided heart failure and increased > pressure can be seen.8 Other symptoms include decreased appetite, > chest tightness and achy joints or muscles.> > Diagnosis > > The patient should be referred to a pulmonologist, who will perform a > history and physical examination and order a series of tests. A > pulmonary function test allows the pulmonologist to see the severity > of disease, measured by low residual volume and total lung capacity, > as well as a decreased diffusion capacity. > > A 6-minute walk test and cardiopulmonary exercise test show hypoxemia > related to a "widened" alveolar-arterial oxygen gradient. To watch > the progression of the disease, serial pulmonary function testing is > recommended.8 > > Blood work is ordered next, including a complete blood count, > antinuclear antibodies, rheumatoid factor and immunoglobulins. The > blood work may show increased inflammatory markers, mild anemia and > unspecified rises in the ANA and rheumatoid factor levels. These > levels can be high in the absence of any collagen vascular disease, > including the rise in autoantibodies.2 > > A chest radiograph will show bilateral reticular opacities > infiltrated with a lower lobe predominance seen in the periphery. > These patients also should have a high-resolution CT scan. With the > progression of the disease, cystic dilatation leads to honeycombing, > traction bronchiectasis and ground glass.2 > > A bronchoavelolar lavage (BAL) is not the best way to confirm > diagnosis of pulmonary fibrosis because the sample is normally too > small.2 BAL is useful in ruling out other diseases such as > infection, malignancy and sarcoidosis. > > The gold standard of testing is a surgical lung biopsy, which will > show usual interstitial pneumonitis in patients with idiopathic > pulmonary fibrosis. The pattern is usually one of a heterogeneity of > scarring with cysts demonstrating a honeycombing form along with > destruction of the alveoli. This pattern is normally seen in patients > with pulmonary fibrosis from drug toxicity, collagen vascular > diseases, asbestosis, chronic hypersensitive pneumonitis and familial > pulmonary fibrosis.8 > > Biopsy results allow the pulmonologist to rule out any other possible > causes of pulmonary fibrosis. The following types of patients should > have a biopsy: recurrent pneumothorax, general systemic complaints, > over age 50, rapid disease progression, CT scan showing changes and > unclear diagnosis.8 If a patient presents with four of the major > criteria or three of the minor criteria, surgical biopsy is > unnecessary.> > Treatment > > No immunosuppressive medications improve the overall survival of > patients with pulmonary fibrosis. Anti-inflammatory drugs are > mainstays of treatment, even though some studies have shown they > offer little improvment.9 Typically 20 mg/day of prednisone is > prescribed in conjunction with azathioprine at 3 mg/kg/day, not to > exceed 200 mg/day.8 > > It is imperative that health care providers know the side effects of > prednisone and azathioprine. With prednisone, monitor for > hyperglycemia, osteoporosis, myopathy and exaggerated hypertension. > The patient should be placed on calcium with vitamin D for > osteoporosis prevention. For long-term use, a dexa scan should be > performed at least yearly. > > Before initiating any prophylactic steroid therapy, a purified > protein derivative test should be performed to ensure the patient > does not have tuberculosis. With azathioprine, monitor for bone > marrow suppression and hepatotoxicity.8> > Another group of medications is the antifibrotic agents, which slow > down the fibrosis in the lungs. Some of these drugs interfere with > the cellular matrix to help slow the progression of pulmonary > fibrosis. Pirfenidone and interferon gamma-1b are currently under > investigation. Cholchicine has been found to stop the fibroblast > proliferation by inhibiting collagen formation.10> > Oxygen > > Another important treatment for patients with pulmonary fibrosis is > oxygen. With increased scarring within the lung parenchyma, the > patient will have decreased oxygen perfusion. This supplement helps > to maintain the patient's oxygen pressure at the level of the > alveoli. > > Oxygen saturation should be maintained at 90 percent or more. A 6-> minute walk test, a cardiopulmonary exercise test, or arterial blood > gas should be performed to determine if a patient requires oxygen.11 > These tests will give the practitioner information on how much oxygen > to order for the patient at rest and on exertion.> > Lung transplant > > Patients under age 65 with few conflicting medical problems are > normally good candidates for lung transplant and undergo vigorous > testing to evaluate their overall pulmonary and cardiac status. > > Of course, lung transplantation carries risks. According to the > United Network of Organ Sharing, 77 percent to 78.6 percent of > patients are alive one year after transplant, while 40 percent to > 46.2 percent live 5 years or longer. Research has shown lung > transplantation improves pulmonary fibrosis patients' overall > survival.12 > > Hence, it is imperative that physician assistants recognize signs and > symptoms of pulmonary fibrosis and provide early referral for > treatment with medication and placement on the lung transplant list.> > References> > 1. American Thoracic Society. Idiopathic pulmonary fibrosis: > diagnosis and treatment: international consensus statement. American > Journal of Respiratory Critical Care Medicine. 2000; 161:646-664.> > 2. Gross T, Hunninghake G. Idiopathic pulmonary fibrosis. New England > Journal of Medicine. 2001; 345:517-525.> > 3. King T, et al. Predicting survival in idiopathic pulmonary > fibrosis: scoring system and survival model. American Journal of > Respiratory and Critical Care Medicine. 2001; 164:1171-1181.> > 4. Miyake O, et al. Occupational and environmental factors and > idiopathic pulmonary fibrosis. British Occupational Hygiene Society. > 2005; (49)3:259-265.> > 5. Tang Y, et al. Increased detection of herpesvirus DNA in > idiopathic pulmonary fibrosis. Chest. 2001; 120(1 Suppl):74s-75s.> > 6. Hubbard R, et al. Exposre to commonly prescribed drugs and the > etiology of crytogenic fibrosing alveolitis. American Journal of > Respiratory and Critical Care Medicine. 1998; 157:743-747.> > 7. Ozkan M, et al. Drug-induced lung disease. Cleveland Clinic > Journal of Medicine. 2001; 68(9):782-795.> > 8. Khalil N, O'Connor R. Idiopathic pulmonary fibrosis: current > understanding of the pathogenesis and the status of treatment. > Canadian Medical Association Journal. 2004; 171(2):153-160.> > 9. Richeldi L, et al. Corticosteroids for idiopathic pulmonary > fibrosis. The Cochrane Database of Systematic Reviews. 2003; > 3:CD002880.> > 10. Selman M, King T, Pardo A. Idiopathic pulmonary fibrosis: > prevailing and evolving hypothesis about its pathogenesis and > implications for therapy. ls of Internal Medicine. 2001; 134:136-> 151.> > 11. Lindell K, s S. Pulomonary fibrosis: new guidelines for > diagnosing and managing the disease demand a fresh approach to > nursing care. American Journal of Nursing. 2003; 103(4):33-42.> > 12. Thabut G, et al. Survival benefit of lung transplantation for > patients with idiopathic pulmonary fibrosis. The Journal of Thoracic > and Cardiovascular Surgery. 2003; 126:469-475.> > > > Bronzell-Wynder, NP, works in the transplant surgery department > at Jefferson University Hospital in Philadelphia.> P PM (Polymositis) 12/98, UIP 8/00, o2 24/7 8/04, PH 3/06, ILL yo 59

[Guest guest]

How do I save this to a file. It won't let me copy it unless it is in the reply-- Does that make sense? KSher Bauman wrote: ... I'm saving this one! This is really good and covers everything in a way I could understand. (Most of it). Thanks for sending it along to us. MamaSher, age 69.IPF 3-06,NSIP 4-08. OR.Don't fret

about tomorrow, God is already there! Diagnosing Pulmonary Fibrosis Read this article that I thought was very good so passing it on to you guys P: >> Diagnosing Pulmonary Fibrosis > Environmental factors and genetics increase the risk of developing > this fatal disease > By Bronzell-Wynder, NP > > > Mr. is a 60-year-old man referred to a pulmonologist after > preadmission testing for a total knee replacement. His chest > radiograph proves abnormal, showing bilateral reticular opacities. > His knee surgery is cancelled, and he is referred to a lung > practitioner. > > A review of systems during the lung practitioner's assessment reveals > Mr. ' progressive shortness of breath the past year. On visual > assessment, he has digital clubbing with cyanosis of his fingertips > and lips. On auscultation, he has bibasilar crackles. The lung > practitioner orders several tests, which reveal pulmonary fibrosis.> >

Definition> > Pulmonary fibrosis is one of many interstitial lung diseases that > scar lung tissue. An incurable, restrictive lung disease, it causes > scarring and fibrosis between the alveoli (chronic interstitial > inflammation). Scarring and stiffening of the alveoli decreases > transport of oxygen across the alveolar membrane. > > One clear sign of pulmonary fibrosis on CT scan is honeycombing and > scarring, which irreversibly lessen elasticity and lung compliance.1> > Pulmonary fibrosis is known by different names, including idiopathic > pulmonary fibrosis (IPF), defined by progressive dyspnea and > declining pulmonary function test.2 Chest radiographs display > interstitial infiltrations and biopsy shows fibrosis, inflammation or > both. > > Idiopathic interstitial pneumonias fall into eight categories. > > Desquamative

interstitial pneumonia (DIP): A rare form of fibrosis. > Average age at onset is 42. Of these patients, 90 percent have a > smoking history, and 75 percent respond to steroids. > > Bronchiolitis obliterans > > Organizing pneumonia > > Cryptogenic organizing pneumonia (CFA) > > Respiratory bronchiolitis interstitial lung disease (RBILD): Takes on > the same process of DIP. Average age at onset is 36.1,2 > > Acute interstitial pneumonia (AIP): Acute onset with rapid > progression of shortness of breath and respiratory failure. Life > expectancy is 6 months. > > Nonspecific interstitial pneumonia (NSIP): More common in women. > Average age at onset is 49. Inflammation is more common than > fibrosis. > > Lymphangioleiomyomatosis (LAM): A rare form of pulmonary fibrosis > affecting only women. Average age at onset is 34.

> Other disease processes can cause pulmonary fibrosis, including > lupus, rheumatoid arthritis, scleroderma and sarcoidosis.> > Epidemiology > > Studies show that 13 to 20 people per 100,000 develop interstitial > lung diseases, including an IPF prevalence of 7.4 women per 100,000 > and 10.7 men per 100,000.1> > Pulmonary fibrosis typically affects adults 40 to 70 years of age. > About two-thirds are over age 60. The mean age at diagnosis is 66. > Incidence increases after age 75, to 175 per 100,000. Overall death > increases at age 75, as well, with the mean length of survival > following diagnosis at 3 to 5 years.1 > > Unfortunately, pulmonary fibrosis patients, not knowing their > condition for years, attribute shortness of breath to other causes, > such as weight gain, aging and lack of exercise. As a result, the > condition is often

diagnosed in the late stages after a comparatively > minor medical event, for example, flu requiring hospitalization. > After diagnosis, life expectancy for most patients is 2 to 4 years, > with the 5-year survival rate at 30 percent to 50 percent.1> > Risk factors > > There are six known risk factors for pulmonary fibrosis: > > cigarette smoking > > environmental factors> > genetic predisposition> > chronic aspiration > > infectious agents > > some prescription medications.1 > While cigarette smoking plays a role in the development of pulmonary > fibrosis, it has actually extended survival in some patients, > compared with nonsmokers or former smokers.3 > > A likely explanation is that these patients may seek medical > attention earlier for smoking-related symptoms.3 These patients also >

can have more digital clubbing and less crackling on examination, > increased incidence of pulmonary hypertension on chest radiograph and > decreased spirometry and diffusion capacities on pulmonary function > tests.3 > > Environmental factors that increase the risk of developing pulmonary > fibrosis include exposure to solvents and to dust from metal and > wood, and atopy.4> > Genetic predisposition also may be a factor. No specific genetic > markers have been linked to IPF, but there have been cases of two > immediate family members having IPF, though this is currently being > researched.> > Chronic aspiration is another risk factor for IPF. These patients > tend to aspirate secondary to gastric reflux disease. However, it is > still not clear how chronic aspiration leads to IPF. > > Several infectious diseases can lead to IPF, including

Legionnaires > disease, herpes virus, parainfluenza 3 virus, HIV-1, hepatitis C, > cytomegalovirus, measles and the Epstein Barr virus.1,5 Some studies > evaluated DNA markers of CMV, EBV, HHV-7 and eight and have shown > that patients with these viruses were at an increased risk for > pulmonary fibrosis.5> > Antidepressants and tricyclic medications may lead to pulmonary > fibrosis.6 Dothiepin and imipramine have shown to increase incidence > of IPF, but how these medications affect the disease process is not > known.6 > > Other medications may cause pulmonary fibrosis using different > mechanisms. They include amiodarone, amphotericin B, nitrofurantoin, > methotrexate, bleomycin, vinca and alkaloids. Prolonged use of > amiodarone leads to pulmonary toxicity. Chemotherapeutic medications > such as bleomycin and vinca alkaloids, can cause pulmonary fibrosis

> in 10 percent of the population. Methotrexate, standard dosaging in > patients with arthritis and nitrofurantoin, also can cause pulmonary > fibrosis.7 Other medications can lead to pulmonary fibrosis, with > high doses or chronic use related to the disease process. > > IPF does not have a known etiology unless the causative agent is > biopsied from the lung.> > Pathophysiology > > Pulmonary fibrosis is caused by stimuli, or exposure to a substance > that triggers a triad of responses.2 First, the stimulus is > introduced causing chronic inflammation within the lung tissue, which > releases cytokines that eventually lead to lung injury. This is > caused by development of increased fibroblast activity, which leads > to fibrosis of the lung parenchyma.2,8 > > A new hypothesis outlines how pulmonary fibrosis developes: First, > the

patient is exposed to a stimulus, which initiates lung injury > that can cause inflammation depending on the patient's dominant > inflammatory phenotype. Eventually, th lung tissue begins to heal > causing fibrosis.2 Some of the factors modifying the > patient's "fibrotic" response are a history of smoking, environmental > exposures and viral infections.> > Clinical presentation > > Patients with pulmonary fibrosis will complain of a cough with or > without mucus, and about 25 percent to 44 percent will have digital > clubbing. The most common and insidious side effect is progressive > shortness of breath. On auscultation, bibasilar inspiratory crackles > can be heard. > In more advanced cases, right-sided heart failure and increased > pressure can be seen.8 Other symptoms include decreased appetite, > chest tightness and achy joints or muscles.> >

Diagnosis > > The patient should be referred to a pulmonologist, who will perform a > history and physical examination and order a series of tests. A > pulmonary function test allows the pulmonologist to see the severity > of disease, measured by low residual volume and total lung capacity, > as well as a decreased diffusion capacity. > > A 6-minute walk test and cardiopulmonary exercise test show hypoxemia > related to a "widened" alveolar-arterial oxygen gradient. To watch > the progression of the disease, serial pulmonary function testing is > recommended.8 > > Blood work is ordered next, including a complete blood count, > antinuclear antibodies, rheumatoid factor and immunoglobulins. The > blood work may show increased inflammatory markers, mild anemia and > unspecified rises in the ANA and rheumatoid factor levels. These > levels can be high in

the absence of any collagen vascular disease, > including the rise in autoantibodies.2 > > A chest radiograph will show bilateral reticular opacities > infiltrated with a lower lobe predominance seen in the periphery. > These patients also should have a high-resolution CT scan. With the > progression of the disease, cystic dilatation leads to honeycombing, > traction bronchiectasis and ground glass.2 > > A bronchoavelolar lavage (BAL) is not the best way to confirm > diagnosis of pulmonary fibrosis because the sample is normally too > small.2 BAL is useful in ruling out other diseases such as > infection, malignancy and sarcoidosis. > > The gold standard of testing is a surgical lung biopsy, which will > show usual interstitial pneumonitis in patients with idiopathic > pulmonary fibrosis. The pattern is usually one of a heterogeneity of > scarring

with cysts demonstrating a honeycombing form along with > destruction of the alveoli. This pattern is normally seen in patients > with pulmonary fibrosis from drug toxicity, collagen vascular > diseases, asbestosis, chronic hypersensitive pneumonitis and familial > pulmonary fibrosis.8 > > Biopsy results allow the pulmonologist to rule out any other possible > causes of pulmonary fibrosis. The following types of patients should > have a biopsy: recurrent pneumothorax, general systemic complaints, > over age 50, rapid disease progression, CT scan showing changes and > unclear diagnosis.8 If a patient presents with four of the major > criteria or three of the minor criteria, surgical biopsy is > unnecessary.> > Treatment > > No immunosuppressive medications improve the overall survival of > patients with pulmonary fibrosis. Anti-inflammatory drugs are

> mainstays of treatment, even though some studies have shown they > offer little improvment.9 Typically 20 mg/day of prednisone is > prescribed in conjunction with azathioprine at 3 mg/kg/day, not to > exceed 200 mg/day.8 > > It is imperative that health care providers know the side effects of > prednisone and azathioprine. With prednisone, monitor for > hyperglycemia, osteoporosis, myopathy and exaggerated hypertension. > The patient should be placed on calcium with vitamin D for > osteoporosis prevention. For long-term use, a dexa scan should be > performed at least yearly. > > Before initiating any prophylactic steroid therapy, a purified > protein derivative test should be performed to ensure the patient > does not have tuberculosis. With azathioprine, monitor for bone > marrow suppression and hepatotoxicity.8> > Another group of

medications is the antifibrotic agents, which slow > down the fibrosis in the lungs. Some of these drugs interfere with > the cellular matrix to help slow the progression of pulmonary > fibrosis. Pirfenidone and interferon gamma-1b are currently under > investigation. Cholchicine has been found to stop the fibroblast > proliferation by inhibiting collagen formation.10> > Oxygen > > Another important treatment for patients with pulmonary fibrosis is > oxygen. With increased scarring within the lung parenchyma, the > patient will have decreased oxygen perfusion. This supplement helps > to maintain the patient's oxygen pressure at the level of the > alveoli. > > Oxygen saturation should be maintained at 90 percent or more. A 6-> minute walk test, a cardiopulmonary exercise test, or arterial blood > gas should be performed to determine if a patient requires

oxygen.11 > These tests will give the practitioner information on how much oxygen > to order for the patient at rest and on exertion.> > Lung transplant > > Patients under age 65 with few conflicting medical problems are > normally good candidates for lung transplant and undergo vigorous > testing to evaluate their overall pulmonary and cardiac status. > > Of course, lung transplantation carries risks. According to the > United Network of Organ Sharing, 77 percent to 78.6 percent of > patients are alive one year after transplant, while 40 percent to > 46.2 percent live 5 years or longer. Research has shown lung > transplantation improves pulmonary fibrosis patients' overall > survival.12 > > Hence, it is imperative that physician assistants recognize signs and > symptoms of pulmonary fibrosis and provide early referral for > treatment with

medication and placement on the lung transplant list.> > References> > 1. American Thoracic Society. Idiopathic pulmonary fibrosis: > diagnosis and treatment: international consensus statement. American > Journal of Respiratory Critical Care Medicine. 2000; 161:646-664.> > 2. Gross T, Hunninghake G. Idiopathic pulmonary fibrosis. New England > Journal of Medicine. 2001; 345:517-525.> > 3. King T, et al. Predicting survival in idiopathic pulmonary > fibrosis: scoring system and survival model. American Journal of > Respiratory and Critical Care Medicine. 2001; 164:1171-1181.> > 4. Miyake O, et al. Occupational and environmental factors and > idiopathic pulmonary fibrosis. British Occupational Hygiene Society. > 2005; (49)3:259-265.> > 5. Tang Y, et al. Increased detection of herpesvirus DNA in > idiopathic pulmonary fibrosis. Chest.

2001; 120(1 Suppl):74s-75s.> > 6. Hubbard R, et al. Exposre to commonly prescribed drugs and the > etiology of crytogenic fibrosing alveolitis. American Journal of > Respiratory and Critical Care Medicine. 1998; 157:743-747.> > 7. Ozkan M, et al. Drug-induced lung disease. Cleveland Clinic > Journal of Medicine. 2001; 68(9):782-795.> > 8. Khalil N, O'Connor R. Idiopathic pulmonary fibrosis: current > understanding of the pathogenesis and the status of treatment. > Canadian Medical Association Journal. 2004; 171(2):153-160.> > 9. Richeldi L, et al. Corticosteroids for idiopathic pulmonary > fibrosis. The Cochrane Database of Systematic Reviews. 2003; > 3:CD002880.> > 10. Selman M, King T, Pardo A. Idiopathic pulmonary fibrosis: > prevailing and evolving hypothesis about its pathogenesis and > implications for therapy. ls of Internal

Medicine. 2001; 134:136-> 151.> > 11. Lindell K, s S. Pulomonary fibrosis: new guidelines for > diagnosing and managing the disease demand a fresh approach to > nursing care. American Journal of Nursing. 2003; 103(4):33-42.> > 12. Thabut G, et al. Survival benefit of lung transplantation for > patients with idiopathic pulmonary fibrosis. The Journal of Thoracic > and Cardiovascular Surgery. 2003; 126:469-475.> > > > Bronzell-Wynder, NP, works in the transplant surgery department > at Jefferson University Hospital in Philadelphia.> P PM (Polymositis) 12/98, UIP 8/00, o2 24/7 8/04, PH 3/06, ILL yo 59 K Central Il Hubby ipf- 2006 As for me and my house, we will serve the Lord 14

[Guest guest]

K... I have Outlook Express and I created a new folder for messages I want to save and just drag a message to that folder................

MamaSher, age 69. IPF 3-06, OR.Don't fret about tomorrow, God is already there!

Diagnosing Pulmonary Fibrosis

Read this article that I thought was very good so passing it on to you guys P:

>> Diagnosing Pulmonary Fibrosis > Environmental factors and genetics increase the risk of developing > this fatal disease > By Bronzell-Wynder, NP > > > Mr. is a 60-year-old man referred to a pulmonologist after > preadmission testing for a total knee replacement. His chest > radiograph proves abnormal, showing bilateral reticular opacities. > His knee surgery is cancelled, and he is referred to a lung > practitioner. > > A review of systems during the lung practitioner's assessment reveals > Mr. ' progressive shortness of breath the past year. On visual > assessment, he has digital clubbing with cyanosis of his fingertips > and lips. On auscultation, he has bibasilar crackles. The lung > practitioner orders several tests, which reveal pulmonary fibrosis.> > Definition> > Pulmonary fibrosis is one of many interstitial lung diseases that > scar lung tissue. An incurable, restrictive lung disease, it causes > scarring and fibrosis between the alveoli (chronic interstitial > inflammation). Scarring and stiffening of the alveoli decreases > transport of oxygen across the alveolar membrane. > > One clear sign of pulmonary fibrosis on CT scan is honeycombing and > scarring, which irreversibly lessen elasticity and lung compliance.1> > Pulmonary fibrosis is known by different names, including idiopathic > pulmonary fibrosis (IPF), defined by progressive dyspnea and > declining pulmonary function test.2 Chest radiographs display > interstitial infiltrations and biopsy shows fibrosis, inflammation or > both. > > Idiopathic interstitial pneumonias fall into eight categories. > > Desquamative interstitial pneumonia (DIP): A rare form of fibrosis. > Average age at onset is 42. Of these patients, 90 percent have a > smoking history, and 75 percent respond to steroids. > > Bronchiolitis obliterans > > Organizing pneumonia > > Cryptogenic organizing pneumonia (CFA) > > Respiratory bronchiolitis interstitial lung disease (RBILD): Takes on > the same process of DIP. Average age at onset is 36.1,2 > > Acute interstitial pneumonia (AIP): Acute onset with rapid > progression of shortness of breath and respiratory failure. Life > expectancy is 6 months. > > Nonspecific interstitial pneumonia (NSIP): More common in women. > Average age at onset is 49. Inflammation is more common than > fibrosis. > > Lymphangioleiomyomatosis (LAM): A rare form of pulmonary fibrosis > affecting only women. Average age at onset is 34. > Other disease processes can cause pulmonary fibrosis, including > lupus, rheumatoid arthritis, scleroderma and sarcoidosis.> > Epidemiology > > Studies show that 13 to 20 people per 100,000 develop interstitial > lung diseases, including an IPF prevalence of 7.4 women per 100,000 > and 10.7 men per 100,000.1> > Pulmonary fibrosis typically affects adults 40 to 70 years of age. > About two-thirds are over age 60. The mean age at diagnosis is 66. > Incidence increases after age 75, to 175 per 100,000. Overall death > increases at age 75, as well, with the mean length of survival > following diagnosis at 3 to 5 years.1 > > Unfortunately, pulmonary fibrosis patients, not knowing their > condition for years, attribute shortness of breath to other causes, > such as weight gain, aging and lack of exercise. As a result, the > condition is often diagnosed in the late stages after a comparatively > minor medical event, for example, flu requiring hospitalization. > After diagnosis, life expectancy for most patients is 2 to 4 years, > with the 5-year survival rate at 30 percent to 50 percent.1> > Risk factors > > There are six known risk factors for pulmonary fibrosis: > > cigarette smoking > > environmental factors> > genetic predisposition> > chronic aspiration > > infectious agents > > some prescription medications.1 > While cigarette smoking plays a role in the development of pulmonary > fibrosis, it has actually extended survival in some patients, > compared with nonsmokers or former smokers.3 > > A likely explanation is that these patients may seek medical > attention earlier for smoking-related symptoms.3 These patients also > can have more digital clubbing and less crackling on examination, > increased incidence of pulmonary hypertension on chest radiograph and > decreased spirometry and diffusion capacities on pulmonary function > tests.3 > > Environmental factors that increase the risk of developing pulmonary > fibrosis include exposure to solvents and to dust from metal and > wood, and atopy.4> > Genetic predisposition also may be a factor. No specific genetic > markers have been linked to IPF, but there have been cases of two > immediate family members having IPF, though this is currently being > researched.> > Chronic aspiration is another risk factor for IPF. These patients > tend to aspirate secondary to gastric reflux disease. However, it is > still not clear how chronic aspiration leads to IPF. > > Several infectious diseases can lead to IPF, including Legionnaires > disease, herpes virus, parainfluenza 3 virus, HIV-1, hepatitis C, > cytomegalovirus, measles and the Epstein Barr virus.1,5 Some studies > evaluated DNA markers of CMV, EBV, HHV-7 and eight and have shown > that patients with these viruses were at an increased risk for > pulmonary fibrosis.5> > Antidepressants and tricyclic medications may lead to pulmonary > fibrosis.6 Dothiepin and imipramine have shown to increase incidence > of IPF, but how these medications affect the disease process is not > known.6 > > Other medications may cause pulmonary fibrosis using different > mechanisms. They include amiodarone, amphotericin B, nitrofurantoin, > methotrexate, bleomycin, vinca and alkaloids. Prolonged use of > amiodarone leads to pulmonary toxicity. Chemotherapeutic medications > such as bleomycin and vinca alkaloids, can cause pulmonary fibrosis > in 10 percent of the population. Methotrexate, standard dosaging in > patients with arthritis and nitrofurantoin, also can cause pulmonary > fibrosis.7 Other medications can lead to pulmonary fibrosis, with > high doses or chronic use related to the disease process. > > IPF does not have a known etiology unless the causative agent is > biopsied from the lung.> > Pathophysiology > > Pulmonary fibrosis is caused by stimuli, or exposure to a substance > that triggers a triad of responses.2 First, the stimulus is > introduced causing chronic inflammation within the lung tissue, which > releases cytokines that eventually lead to lung injury. This is > caused by development of increased fibroblast activity, which leads > to fibrosis of the lung parenchyma.2,8 > > A new hypothesis outlines how pulmonary fibrosis developes: First, > the patient is exposed to a stimulus, which initiates lung injury > that can cause inflammation depending on the patient's dominant > inflammatory phenotype. Eventually, th lung tissue begins to heal > causing fibrosis.2 Some of the factors modifying the > patient's "fibrotic" response are a history of smoking, environmental > exposures and viral infections.> > Clinical presentation > > Patients with pulmonary fibrosis will complain of a cough with or > without mucus, and about 25 percent to 44 percent will have digital > clubbing. The most common and insidious side effect is progressive > shortness of breath. On auscultation, bibasilar inspiratory crackles > can be heard. > In more advanced cases, right-sided heart failure and increased > pressure can be seen.8 Other symptoms include decreased appetite, > chest tightness and achy joints or muscles.> > Diagnosis > > The patient should be referred to a pulmonologist, who will perform a > history and physical examination and order a series of tests. A > pulmonary function test allows the pulmonologist to see the severity > of disease, measured by low residual volume and total lung capacity, > as well as a decreased diffusion capacity. > > A 6-minute walk test and cardiopulmonary exercise test show hypoxemia > related to a "widened" alveolar-arterial oxygen gradient. To watch > the progression of the disease, serial pulmonary function testing is > recommended.8 > > Blood work is ordered next, including a complete blood count, > antinuclear antibodies, rheumatoid factor and immunoglobulins. The > blood work may show increased inflammatory markers, mild anemia and > unspecified rises in the ANA and rheumatoid factor levels. These > levels can be high in the absence of any collagen vascular disease, > including the rise in autoantibodies.2 > > A chest radiograph will show bilateral reticular opacities > infiltrated with a lower lobe predominance seen in the periphery. > These patients also should have a high-resolution CT scan. With the > progression of the disease, cystic dilatation leads to honeycombing, > traction bronchiectasis and ground glass.2 > > A bronchoavelolar lavage (BAL) is not the best way to confirm > diagnosis of pulmonary fibrosis because the sample is normally too > small.2 BAL is useful in ruling out other diseases such as > infection, malignancy and sarcoidosis. > > The gold standard of testing is a surgical lung biopsy, which will > show usual interstitial pneumonitis in patients with idiopathic > pulmonary fibrosis. The pattern is usually one of a heterogeneity of > scarring with cysts demonstrating a honeycombing form along with > destruction of the alveoli. This pattern is normally seen in patients > with pulmonary fibrosis from drug toxicity, collagen vascular > diseases, asbestosis, chronic hypersensitive pneumonitis and familial > pulmonary fibrosis.8 > > Biopsy results allow the pulmonologist to rule out any other possible > causes of pulmonary fibrosis. The following types of patients should > have a biopsy: recurrent pneumothorax, general systemic complaints, > over age 50, rapid disease progression, CT scan showing changes and > unclear diagnosis.8 If a patient presents with four of the major > criteria or three of the minor criteria, surgical biopsy is > unnecessary.> > Treatment > > No immunosuppressive medications improve the overall survival of > patients with pulmonary fibrosis. Anti-inflammatory drugs are > mainstays of treatment, even though some studies have shown they > offer little improvment.9 Typically 20 mg/day of prednisone is > prescribed in conjunction with azathioprine at 3 mg/kg/day, not to > exceed 200 mg/day.8 > > It is imperative that health care providers know the side effects of > prednisone and azathioprine. With prednisone, monitor for > hyperglycemia, osteoporosis, myopathy and exaggerated hypertension. > The patient should be placed on calcium with vitamin D for > osteoporosis prevention. For long-term use, a dexa scan should be > performed at least yearly. > > Before initiating any prophylactic steroid therapy, a purified > protein derivative test should be performed to ensure the patient > does not have tuberculosis. With azathioprine, monitor for bone > marrow suppression and hepatotoxicity.8> > Another group of medications is the antifibrotic agents, which slow > down the fibrosis in the lungs. Some of these drugs interfere with > the cellular matrix to help slow the progression of pulmonary > fibrosis. Pirfenidone and interferon gamma-1b are currently under > investigation. Cholchicine has been found to stop the fibroblast > proliferation by inhibiting collagen formation.10> > Oxygen > > Another important treatment for patients with pulmonary fibrosis is > oxygen. With increased scarring within the lung parenchyma, the > patient will have decreased oxygen perfusion. This supplement helps > to maintain the patient's oxygen pressure at the level of the > alveoli. > > Oxygen saturation should be maintained at 90 percent or more. A 6-> minute walk test, a cardiopulmonary exercise test, or arterial blood > gas should be performed to determine if a patient requires oxygen.11 > These tests will give the practitioner information on how much oxygen > to order for the patient at rest and on exertion.> > Lung transplant > > Patients under age 65 with few conflicting medical problems are > normally good candidates for lung transplant and undergo vigorous > testing to evaluate their overall pulmonary and cardiac status. > > Of course, lung transplantation carries risks. According to the > United Network of Organ Sharing, 77 percent to 78.6 percent of > patients are alive one year after transplant, while 40 percent to > 46.2 percent live 5 years or longer. Research has shown lung > transplantation improves pulmonary fibrosis patients' overall > survival.12 > > Hence, it is imperative that physician assistants recognize signs and > symptoms of pulmonary fibrosis and provide early referral for > treatment with medication and placement on the lung transplant list.> > References> > 1. American Thoracic Society. Idiopathic pulmonary fibrosis: > diagnosis and treatment: international consensus statement. American > Journal of Respiratory Critical Care Medicine. 2000; 161:646-664.> > 2. Gross T, Hunninghake G. Idiopathic pulmonary fibrosis. New England > Journal of Medicine. 2001; 345:517-525.> > 3. King T, et al. Predicting survival in idiopathic pulmonary > fibrosis: scoring system and survival model. American Journal of > Respiratory and Critical Care Medicine. 2001; 164:1171-1181.> > 4. Miyake O, et al. Occupational and environmental factors and > idiopathic pulmonary fibrosis. British Occupational Hygiene Society. > 2005; (49)3:259-265.> > 5. Tang Y, et al. Increased detection of herpesvirus DNA in > idiopathic pulmonary fibrosis. Chest. 2001; 120(1 Suppl):74s-75s.> > 6. Hubbard R, et al. Exposre to commonly prescribed drugs and the > etiology of crytogenic fibrosing alveolitis. American Journal of > Respiratory and Critical Care Medicine. 1998; 157:743-747.> > 7. Ozkan M, et al. Drug-induced lung disease. Cleveland Clinic > Journal of Medicine. 2001; 68(9):782-795.> > 8. Khalil N, O'Connor R. Idiopathic pulmonary fibrosis: current > understanding of the pathogenesis and the status of treatment. > Canadian Medical Association Journal. 2004; 171(2):153-160.> > 9. Richeldi L, et al. Corticosteroids for idiopathic pulmonary > fibrosis. The Cochrane Database of Systematic Reviews. 2003; > 3:CD002880.> > 10. Selman M, King T, Pardo A. Idiopathic pulmonary fibrosis: > prevailing and evolving hypothesis about its pathogenesis and > implications for therapy. ls of Internal Medicine. 2001; 134:136-> 151.> > 11. Lindell K, s S. Pulomonary fibrosis: new guidelines for > diagnosing and managing the disease demand a fresh approach to > nursing care. American Journal of Nursing. 2003; 103(4):33-42.> > 12. Thabut G, et al. Survival benefit of lung transplantation for > patients with idiopathic pulmonary fibrosis. The Journal of Thoracic > and Cardiovascular Surgery. 2003; 126:469-475.> > > > Bronzell-Wynder, NP, works in the transplant surgery department > at Jefferson University Hospital in Philadelphia.> P PM (Polymositis) 12/98, UIP 8/00, o2 24/7 8/04, PH 3/06, ILL yo 59

K

Central Il

Hubby ipf- 2006

As for me and my house, we will serve the Lord

14

[Guest guest]

I would reply or forward to another email account I have then copy and save as needed. Or you can set up a save folder here and move it into that file.

Fay

IPF06/06 IL

Diagnosing Pulmonary Fibrosis

Read this article that I thought was very good so passing it on to you guys P:

>> Diagnosing Pulmonary Fibrosis > Environmental factors and genetics increase the risk of developing > this fatal disease > By Bronzell-Wynder, NP > > > Mr. is a 60-year-old man referred to a pulmonologist after > preadmission testing for a total knee replacement. His chest > radiograph proves abnormal, showing bilateral reticular opacities. > His knee surgery is cancelled, and he is referred to a lung > practitioner. > > A review of systems during the lung practitioner' s assessment reveals > Mr. ' progressive shortness of breath the past year. On visual > assessment, he has digital clubbing with cyanosis of his fingertips > and lips. On auscultation, he has bibasilar crackles. The lung >

practitioner orders several tests, which reveal pulmonary fibrosis.> > Definition> > Pulmonary fibrosis is one of many interstitial lung diseases that > scar lung tissue. An incurable, restrictive lung disease, it causes > scarring and fibrosis between the alveoli (chronic interstitial > inflammation) . Scarring and stiffening of the alveoli decreases > transport of oxygen across the alveolar membrane. > > One clear sign of pulmonary fibrosis on CT scan is honeycombing and > scarring, which irreversibly lessen elasticity and lung compliance.1> > Pulmonary fibrosis is known by different names, including idiopathic > pulmonary fibrosis (IPF), defined by progressive dyspnea and > declining pulmonary function test.2 Chest radiographs display > interstitial infiltrations and biopsy shows fibrosis, inflammation or > both. > >

Idiopathic interstitial pneumonias fall into eight categories. > > Desquamative interstitial pneumonia (DIP): A rare form of fibrosis. > Average age at onset is 42. Of these patients, 90 percent have a > smoking history, and 75 percent respond to steroids. > > Bronchiolitis obliterans > > Organizing pneumonia > > Cryptogenic organizing pneumonia (CFA) > > Respiratory bronchiolitis interstitial lung disease (RBILD): Takes on > the same process of DIP. Average age at onset is 36.1,2 > > Acute interstitial pneumonia (AIP): Acute onset with rapid > progression of shortness of breath and respiratory failure. Life > expectancy is 6 months. > > Nonspecific interstitial pneumonia (NSIP): More common in women. > Average age at onset is 49. Inflammation is more common than > fibrosis. > >

Lymphangioleiomyoma tosis (LAM): A rare form of pulmonary fibrosis > affecting only women. Average age at onset is 34. > Other disease processes can cause pulmonary fibrosis, including > lupus, rheumatoid arthritis, scleroderma and sarcoidosis.> > Epidemiology > > Studies show that 13 to 20 people per 100,000 develop interstitial > lung diseases, including an IPF prevalence of 7.4 women per 100,000 > and 10.7 men per 100,000.1> > Pulmonary fibrosis typically affects adults 40 to 70 years of age. > About two-thirds are over age 60. The mean age at diagnosis is 66. > Incidence increases after age 75, to 175 per 100,000. Overall death > increases at age 75, as well, with the mean length of survival > following diagnosis at 3 to 5 years.1 > > Unfortunately, pulmonary fibrosis patients, not knowing their > condition for years,

attribute shortness of breath to other causes, > such as weight gain, aging and lack of exercise. As a result, the > condition is often diagnosed in the late stages after a comparatively > minor medical event, for example, flu requiring hospitalization. > After diagnosis, life expectancy for most patients is 2 to 4 years, > with the 5-year survival rate at 30 percent to 50 percent.1> > Risk factors > > There are six known risk factors for pulmonary fibrosis: > > cigarette smoking > > environmental factors> > genetic predisposition> > chronic aspiration > > infectious agents > > some prescription medications. 1 > While cigarette smoking plays a role in the development of pulmonary > fibrosis, it has actually extended survival in some patients, > compared with nonsmokers or former smokers.3

> > A likely explanation is that these patients may seek medical > attention earlier for smoking-related symptoms.3 These patients also > can have more digital clubbing and less crackling on examination, > increased incidence of pulmonary hypertension on chest radiograph and > decreased spirometry and diffusion capacities on pulmonary function > tests.3 > > Environmental factors that increase the risk of developing pulmonary > fibrosis include exposure to solvents and to dust from metal and > wood, and atopy.4> > Genetic predisposition also may be a factor. No specific genetic > markers have been linked to IPF, but there have been cases of two > immediate family members having IPF, though this is currently being > researched.> > Chronic aspiration is another risk factor for IPF. These patients > tend to aspirate secondary

to gastric reflux disease. However, it is > still not clear how chronic aspiration leads to IPF. > > Several infectious diseases can lead to IPF, including Legionnaires > disease, herpes virus, parainfluenza 3 virus, HIV-1, hepatitis C, > cytomegalovirus, measles and the Epstein Barr virus.1,5 Some studies > evaluated DNA markers of CMV, EBV, HHV-7 and eight and have shown > that patients with these viruses were at an increased risk for > pulmonary fibrosis.5> > Antidepressants and tricyclic medications may lead to pulmonary > fibrosis.6 Dothiepin and imipramine have shown to increase incidence > of IPF, but how these medications affect the disease process is not > known.6 > > Other medications may cause pulmonary fibrosis using different > mechanisms. They include amiodarone, amphotericin B, nitrofurantoin, > methotrexate, bleomycin,

vinca and alkaloids. Prolonged use of > amiodarone leads to pulmonary toxicity. Chemotherapeutic medications > such as bleomycin and vinca alkaloids, can cause pulmonary fibrosis > in 10 percent of the population. Methotrexate, standard dosaging in > patients with arthritis and nitrofurantoin, also can cause pulmonary > fibrosis.7 Other medications can lead to pulmonary fibrosis, with > high doses or chronic use related to the disease process. > > IPF does not have a known etiology unless the causative agent is > biopsied from the lung.> > Pathophysiology > > Pulmonary fibrosis is caused by stimuli, or exposure to a substance > that triggers a triad of responses.2 First, the stimulus is > introduced causing chronic inflammation within the lung tissue, which > releases cytokines that eventually lead to lung injury. This is > caused by

development of increased fibroblast activity, which leads > to fibrosis of the lung parenchyma.2, 8 > > A new hypothesis outlines how pulmonary fibrosis developes: First, > the patient is exposed to a stimulus, which initiates lung injury > that can cause inflammation depending on the patient's dominant > inflammatory phenotype. Eventually, th lung tissue begins to heal > causing fibrosis.2 Some of the factors modifying the > patient's "fibrotic" response are a history of smoking, environmental > exposures and viral infections.> > Clinical presentation > > Patients with pulmonary fibrosis will complain of a cough with or > without mucus, and about 25 percent to 44 percent will have digital > clubbing. The most common and insidious side effect is progressive > shortness of breath. On auscultation, bibasilar inspiratory crackles > can be

heard. > In more advanced cases, right-sided heart failure and increased > pressure can be seen.8 Other symptoms include decreased appetite, > chest tightness and achy joints or muscles.> > Diagnosis > > The patient should be referred to a pulmonologist, who will perform a > history and physical examination and order a series of tests. A > pulmonary function test allows the pulmonologist to see the severity > of disease, measured by low residual volume and total lung capacity, > as well as a decreased diffusion capacity. > > A 6-minute walk test and cardiopulmonary exercise test show hypoxemia > related to a "widened" alveolar-arterial oxygen gradient. To watch > the progression of the disease, serial pulmonary function testing is > recommended. 8 > > Blood work is ordered next, including a complete blood count, >

antinuclear antibodies, rheumatoid factor and immunoglobulins. The > blood work may show increased inflammatory markers, mild anemia and > unspecified rises in the ANA and rheumatoid factor levels. These > levels can be high in the absence of any collagen vascular disease, > including the rise in autoantibodies. 2 > > A chest radiograph will show bilateral reticular opacities > infiltrated with a lower lobe predominance seen in the periphery. > These patients also should have a high-resolution CT scan. With the > progression of the disease, cystic dilatation leads to honeycombing, > traction bronchiectasis and ground glass.2 > > A bronchoavelolar lavage (BAL) is not the best way to confirm > diagnosis of pulmonary fibrosis because the sample is normally too > small.2 BAL is useful in ruling out other diseases such as > infection, malignancy and

sarcoidosis. > > The gold standard of testing is a surgical lung biopsy, which will > show usual interstitial pneumonitis in patients with idiopathic > pulmonary fibrosis. The pattern is usually one of a heterogeneity of > scarring with cysts demonstrating a honeycombing form along with > destruction of the alveoli. This pattern is normally seen in patients > with pulmonary fibrosis from drug toxicity, collagen vascular > diseases, asbestosis, chronic hypersensitive pneumonitis and familial > pulmonary fibrosis.8 > > Biopsy results allow the pulmonologist to rule out any other possible > causes of pulmonary fibrosis. The following types of patients should > have a biopsy: recurrent pneumothorax, general systemic complaints, > over age 50, rapid disease progression, CT scan showing changes and > unclear diagnosis.8 If a patient presents with four of

the major > criteria or three of the minor criteria, surgical biopsy is > unnecessary.> > Treatment > > No immunosuppressive medications improve the overall survival of > patients with pulmonary fibrosis. Anti-inflammatory drugs are > mainstays of treatment, even though some studies have shown they > offer little improvment.9 Typically 20 mg/day of prednisone is > prescribed in conjunction with azathioprine at 3 mg/kg/day, not to > exceed 200 mg/day.8 > > It is imperative that health care providers know the side effects of > prednisone and azathioprine. With prednisone, monitor for > hyperglycemia, osteoporosis, myopathy and exaggerated hypertension. > The patient should be placed on calcium with vitamin D for > osteoporosis prevention. For long-term use, a dexa scan should be > performed at least yearly. > > Before

initiating any prophylactic steroid therapy, a purified > protein derivative test should be performed to ensure the patient > does not have tuberculosis. With azathioprine, monitor for bone > marrow suppression and hepatotoxicity. 8> > Another group of medications is the antifibrotic agents, which slow > down the fibrosis in the lungs. Some of these drugs interfere with > the cellular matrix to help slow the progression of pulmonary > fibrosis. Pirfenidone and interferon gamma-1b are currently under > investigation. Cholchicine has been found to stop the fibroblast > proliferation by inhibiting collagen formation.10> > Oxygen > > Another important treatment for patients with pulmonary fibrosis is > oxygen. With increased scarring within the lung parenchyma, the > patient will have decreased oxygen perfusion. This supplement helps > to

maintain the patient's oxygen pressure at the level of the > alveoli. > > Oxygen saturation should be maintained at 90 percent or more. A 6-> minute walk test, a cardiopulmonary exercise test, or arterial blood > gas should be performed to determine if a patient requires oxygen.11 > These tests will give the practitioner information on how much oxygen > to order for the patient at rest and on exertion.> > Lung transplant > > Patients under age 65 with few conflicting medical problems are > normally good candidates for lung transplant and undergo vigorous > testing to evaluate their overall pulmonary and cardiac status. > > Of course, lung transplantation carries risks. According to the > United Network of Organ Sharing, 77 percent to 78.6 percent of > patients are alive one year after transplant, while 40 percent to > 46.2

percent live 5 years or longer. Research has shown lung > transplantation improves pulmonary fibrosis patients' overall > survival.12 > > Hence, it is imperative that physician assistants recognize signs and > symptoms of pulmonary fibrosis and provide early referral for > treatment with medication and placement on the lung transplant list.> > References> > 1. American Thoracic Society. Idiopathic pulmonary fibrosis: > diagnosis and treatment: international consensus statement. American > Journal of Respiratory Critical Care Medicine. 2000; 161:646-664.> > 2. Gross T, Hunninghake G. Idiopathic pulmonary fibrosis. New England > Journal of Medicine. 2001; 345:517-525.> > 3. King T, et al. Predicting survival in idiopathic pulmonary > fibrosis: scoring system and survival model. American Journal of > Respiratory and Critical

Care Medicine. 2001; 164:1171-1181.> > 4. Miyake O, et al. Occupational and environmental factors and > idiopathic pulmonary fibrosis. British Occupational Hygiene Society. > 2005; (49)3:259-265.> > 5. Tang Y, et al. Increased detection of herpesvirus DNA in > idiopathic pulmonary fibrosis. Chest. 2001; 120(1 Suppl):74s-75s.> > 6. Hubbard R, et al. Exposre to commonly prescribed drugs and the > etiology of crytogenic fibrosing alveolitis. American Journal of > Respiratory and Critical Care Medicine. 1998; 157:743-747.> > 7. Ozkan M, et al. Drug-induced lung disease. Cleveland Clinic > Journal of Medicine. 2001; 68(9):782-795.> > 8. Khalil N, O'Connor R. Idiopathic pulmonary fibrosis: current > understanding of the pathogenesis and the status of treatment. > Canadian Medical Association Journal. 2004; 171(2):153-160.>

> 9. Richeldi L, et al. Corticosteroids for idiopathic pulmonary > fibrosis. The Cochrane Database of Systematic Reviews. 2003; > 3:CD002880.> > 10. Selman M, King T, Pardo A. Idiopathic pulmonary fibrosis: > prevailing and evolving hypothesis about its pathogenesis and > implications for therapy. ls of Internal Medicine. 2001; 134:136-> 151.> > 11. Lindell K, s S. Pulomonary fibrosis: new guidelines for > diagnosing and managing the disease demand a fresh approach to > nursing care. American Journal of Nursing. 2003; 103(4):33-42.> > 12. Thabut G, et al. Survival benefit of lung transplantation for > patients with idiopathic pulmonary fibrosis. The Journal of Thoracic > and Cardiovascular Surgery. 2003; 126:469-475.> > > > Bronzell-Wynder, NP, works in the transplant surgery department > at

Jefferson University Hospital in Philadelphia.> P PM (Polymositis) 12/98, UIP 8/00, o2 24/7 8/04, PH 3/06, ILL yo 59

K

Central Il

Hubby ipf- 2006

As for me and my house, we will serve the Lord

14