Emphysema
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]
Overview
Emphysema is a long-term, progressive disease of the lungs that primarily causes shortness of breath. In people with emphysema, the tissues necessary to support the physical shape and function of the lungs are destroyed. It is included in a group of diseases called chronic obstructive pulmonary disease or COPD (pulmonary refers to the lungs). Emphysema is called an obstructive lung disease because the destruction of lung tissue around smaller sacs, called alveoli, makes these air sacs unable to hold their functional shape upon exhalation. Emphysema is most often caused by tobacco smoking and long-term exposure to air pollution.
The term emphysema means “swelling” and derives from the Greek ἐμφυσᾶν emphysan meaning “inflate” – itself composed of ἐν en, meaning “in“, and φυσᾶν physan, meaning “breath, blast“.[1]
Historical perspective
The terms chronic bronchitis and emphysema were formally defined at the CIBA guest symposium of physicians in 1959. COPD has probably always existed but has been called by different names in the past. Bonet described a condition of “voluminous lungs” in 1679. Matthew Baillie illustrated an emphysematous lung in 1789 and described the destructive character of the condition. The term COPD was first used by William Briscoe in 1965 and has gradually overtaken other terms to become established today as the preferred name for this disease.
Pathophysiology
Emphysema is caused by loss of elasticity of the lung tissue, from destruction of structures supporting the alveoli, and destruction of capillaries feeding the alveoli. The result is that the small airways collapse during exhalation, leading to an obstructive form of lung disease (airflow is impeded and air is generally “trapped” in the lungs in obstructive lung diseases). When toxins such as smoke are breathed into the lungs, the particles are trapped and cause a localized inflammatory response. Chemicals released during the inflammatory response (e.g., elastase) can break down the walls of alveoli (alveolar septum). This leads to fewer but larger alveoli, with a decreased surface area and a decreased ability to absorb oxygen and exude carbon dioxide by diffusion. The activity of another molecule called alpha 1-antitrypsin normally neutralizes the destructive action of one of these damaging molecules.
Risk Factors
Chronic obstructive pulmonary disease is a group of diseases characterized by the pathological limitation of airflow in the airway that is not fully reversible. A full comprehensive diagnosis is needed to eliminate related conditions and isolate the influence of lifestyle and behavior risk factors on condition outcome. Some common risk factors are cigarette smoking, occupational pollutants, air pollution and genetics. Other risk factors are increasing age, male gender, allergy, repeated airway infection.
Differential Diagnosis
In clinical practice, COPD is defined by its characteristically low airflow on lung function tests.[2] In contrast to asthma, this limitation is poorly reversible and usually gets progressively worse over time. It should be differentiated from certain conditions that have similar presentation for instance congestive heart failure, chronic asthma, bronchiectasis, and bronchiolitis obliterans.
References
Historical Perspective
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Mehrian Jafarizade, M.D [2], Cafer Zorkun, M.D., Ph.D. [3], Priyamvada Singh, MBBS [4]
Overview
The terms chronic bronchitis and emphysema were formally defined at the CIBA guest symposium of physicians in 1959. Matthew Baillie illustrated an emphysematous lung in 1789 and described the destructive character of the condition.
Historical Perspective
- In 1679, Bonet for the first time described voluminous lungs.
- In 1721, Ruysh described enlarged lung airspaces in emphysema.
- In 1769, Giovanni Morgagni described nineteen cases in which the lungs were “turgid” particularly from air.
- In 1789, Matthew Baillie illustrated an emphysematous lung and described the destructive character of the condition.
- In 1837, René Laennec, the physician who invented the stethoscope, used the term “emphysema” in his book A Treatise on the Diseases of the Chest and of Mediate Auscultation to describe lungs that did not collapse when he opened the chest during an autopsy. He noted that they did not collapse as usual because they were full of air and the airways were filled with mucus.[1]
- In 1842, John Hutchinson invented the spirometer, which allowed the measurement of the vital capacity of the lungs. However, his spirometer could only measure volume, not airflow.[2]
- In 1944, Ronald Christie defined the diagnosis for emphysema based on dyspnea on exertion, after exclusion of bronchospasm, or left ventricular failure.
- In 1947, Tiffeneau and Pinelli, and in 1950 and 1951, Gaensler described the principles of measuring airflow.
- in 1952, Gough explained the pathology of emphysema.
- In 1956, Barach and Bickerman described treatment for emphysema in their comprehensive textbook.
- In 1959, the terms chronic bronchitis and emphysema were formally defined at the CIBA guest symposium of physicians.
- In 1962, American Thoracic Society Committee on Diagnostic Standards defined the components of COPD and this definition is the foundation of COPD description today.
- In 1964, Gross produced destruction of alveoli and hyperinflation, by injection of pancreatic extracts (papain) into the airways of guinea pigs.
- In 1967, Reid described the pathology of emphysema and other components of COPD.
- In 1976, Thurlbeck illustrated the various types of emphysema in his book.
- In 2003, Choe used methylprednisolone to produce emphysema in rats. They conclude that systemic methylprednisolone increases the activity of matrix metalloproteinases in the lung and causes emphysema. [3]
- In 2004, GOLD offered a new classification of the severity of COPD. [4]
- In 2005, Voelkel and Taraseviciene-Stewart offered emphysema as an autoimmune vascular disease. [5]
Landmark Events in the Development of Treatment Strategies
- In 1956, Barach and Bickerman described treatment for emphysema in their comprehensive textbook.
- In 1980, for treatment of COPD patients, a clinical trial was performed and the use of nocturnal oxygen therapy for emphysema proved. [6]
- In 1998, Paggiaro performed a multicentre clinical trial of corticosteroids for emphysema treatment.[7]
Below table is a summery of emphysema historical prospective:
| Year | Investigator | Landmark event |
|---|---|---|
| 1679 | Bonet | Voluminous lungs |
| 1721 | Ruysh | Emphysema is enlarged lung spaces |
| 1769 | Giovanni Morgagni | Nineteen cases of air filled lungs |
| 1789 | Matthew Baillie | Destructive character of emphysema |
| 1837 | René Laennec | Hyper-resonant lungs with his invented stethoscope |
| 1842 | John Hutchinson | Spirometry invention for measurement of the vital capacity of the lungs |
| 1944 | Ronald Christie | Clinical definition of emphysema |
| 1947 | Tiffeneau and Pinelli | Measuring the air flow in spirometry |
| 1952 | Gough | Emphysema pathology description |
| 1956 | Barach and Bickerman | First description for treatment of emphysema |
| 1959 | CIBA guest symposium of physicians | The terms chronic bronchitis and emphysema were formally defined |
| 1962 | American Thoracic Society Committee on Diagnostic Standards | Defined the components of COPD |
| 1967 | Reid | Described the pathology of emphysema and other components of COPD |
| 1976 | Thurlbeck | Illustrated the various types of emphysema |
| 1980 | Oxygen therapy trial | Use of nocturnal oxygen therapy for emphysema is beneficial |
| 1998 | Paggiaro | Corticosteroids for emphysema treatment |
| 2004 | GOLD criteria | New classification of the severity of COPD |
| 2005 | Voelkel and Taraseviciene-Stewart | Emphysema as an autoimmune vascular disease |
References
- ↑ Petty TL (2006). “The history of COPD”. Int J Chron Obstruct Pulmon Dis. 1 (1): 3–14. PMC 2706597. PMID 18046898.
- ↑ Fishman AP (2005). “One hundred years of chronic obstructive pulmonary disease”. Am. J. Respir. Crit. Care Med. 171 (9): 941–8. doi:10.1164/rccm.200412-1685OE. PMID 15849329. Unknown parameter
|month=ignored (help) - ↑ Choe KH, Taraseviciene-Stewart L, Scerbavicius R, Gera L, Tuder RM, Voelkel NF (2003). “Methylprednisolone causes matrix metalloproteinase-dependent emphysema in adult rats”. Am. J. Respir. Crit. Care Med. 167 (11): 1516–21. doi:10.1164/rccm.200210-1207OC. PMID 12522028.
- ↑ “Global Initiative for Chronic Obstructive Lung Disease – Global Initiative for Chronic Obstructive Lung Disease – GOLD”.
- ↑ Voelkel N, Taraseviciene-Stewart L (2005). “Emphysema: an autoimmune vascular disease?”. Proc Am Thorac Soc. 2 (1): 23–5. doi:10.1513/pats.200405-033MS. PMID 16113465.
- ↑ “Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Nocturnal Oxygen Therapy Trial Group”. Ann. Intern. Med. 93 (3): 391–8. 1980. PMID 6776858.
- ↑ Paggiaro PL, Dahle R, Bakran I, Frith L, Hollingworth K, Efthimiou J (1998). “Multicentre randomised placebo-controlled trial of inhaled fluticasone propionate in patients with chronic obstructive pulmonary disease. International COPD Study Group”. Lancet. 351 (9105): 773–80. PMID 9519948.
Classification
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]
Overview
Emphysema can be classified by location in to different types such as panacinary, centroacinary, congenital lobaremphysema, and paraseptal emphysema.
Classification
Emphysema can be classified by location into three categories:
Panacinary (panlobular):
- The entire respiratory acinus, from respiratory bronchiole to alveoli, is expanded. Occurs more commonly in the lower lobes (especially basal segments) and in the anterior margins of the lungs.[1]
Centroacinary (panacinar and centriacinar):
- The respiratory bronchiole (proximal and central part of the acinus) is expanded. The distal acinus or alveoli are unchanged. Occurs more commonly in the upper lobes.[1][2]
Other types:
Congenital lobar emphysema (CLE)
CLE results in over-expansion of a pulmonary lobe, and resultant compression of the remaining lobes of the ipsi-lateral lung (and possibly also the contralateral lung). There is bronchial narrowing because of weakened or absent bronchial cartilage.[3] There may be congenital extrinsic compression, commonly by an abnormally large pulmonary artery. This causes malformation of bronchial cartilage, making them soft and collapsible.[3] CLE is a potentially reversible (yet possibly life-threatening) cause of respiratory distress in the neonate.[3]
Paraseptal emphysema
Para-septal emphysema is a type of emphysema which involves the alveolar ducts and sacs at the lung periphery. The emphysematous areas are sub-pleural in location and often surrounded by inter-lobular septa (hence the name). It may be an incidental finding in young adults, and may be associated with spontaneous pneumothorax. It may also be seen in older patients with centri-lobular emphysema. Both centri-lobular and para-septal emphysema may progress to bullous emphysema. A bulla is defined as being at least 1 cm in diameter, and with a wall less than 1 mm thick. Bullae are thought to arise by air trapping in emphysematous spaces, causing local expansion.[4]
References
- ↑ 1.0 1.1 “Emphysema”. Retrieved 2008-11-20.
- ↑ Anderson AE, Foraker AG (1973). “Centrilobular emphysema and panlobular emphysema: two different diseases”. Thorax. 28 (5): 547–50. doi:10.1136/thx.28.5.547. PMC 470076. PMID 4784376. Unknown parameter
|month=ignored (help) - ↑ 3.0 3.1 3.2 eMedicine Specialties > Radiology > Pediatrics –> Congenital Lobar Emphysema Author: Beverly P Wood, MD, MS, PhD, University of Southern California. Updated: December 1, 2008
- ↑ Webb WR, Higgins CB. Thoracic Imaging. Lippincott, Williams & Wilkins 2005.
Pathophysiology
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Emphysema is caused by loss of elasticity of the lung tissue, from destruction of structures supporting the alveoli, and destruction of capillaries feeding the alveoli. The result is that the small airways collapse during exhalation (although alveolar collapsability has increased), leading to an obstructive form of lung disease (airflow is impeded and air is generally “trapped” in the lungs in obstructive lung diseases). When toxins such as smoke are breathed into the lungs, the particles are trapped and cause a localized inflammatory response. Chemicals released during the inflammatory response (e.g., elastase) can break down the walls of alveoli (alveolar septum). This leads to fewer but larger alveoli, with a decreased surface area and a decreased ability to absorb oxygen and exude carbon dioxide by diffusion. The activity of another molecule called alpha 1-antitrypsin normally neutralizes the destructive action of one of these damaging molecules.
Pathophysiology
Emphysema is caused by loss of elasticity (increased compliance) of the lung tissue, from destruction of structures supporting the alveoli, and destruction of capillaries feeding the alveoli. The result is that the small airways collapse during exhalation (although alveolar collapsability has increased), leading to an obstructive form of lung disease (airflow is impeded and air is generally “trapped” in the lungs in obstructive lung diseases).
- When toxins such as smoke are breathed into the lungs, the particles are trapped and cause a localized inflammatory response. Chemicals released during the inflammatory response (e.g., elastase) can break down the walls of alveoli (alveolar septum). This leads to fewer but larger alveoli, with a decreased surface area and a decreased ability to absorb oxygen and exude carbon dioxide by diffusion. The activity of another molecule called alpha 1-antitrypsin normally neutralizes the destructive action of one of these damaging molecules.
- After a prolonged period, hyperventilation becomes inadequate to maintain high enough oxygen levels in the blood. The body compensates by vasoconstricting appropriate vessels. This leads to pulmonary hypertension, which places increased strain on the right side of the heart, the one that pumps unoxygenated blood to the lungs, fails. The failure causes the heart muscle to thicken to pump more blood. Eventually, as the heart continues to fail, it becomes larger and blood backs up in the liver.
- Emphysema occurs in a higher proportion in patients with decreased alpha 1-antitrypsin (A1AT) levels (alpha 1-antitrypsin deficiency, A1AD). In A1AD, inflammatory enzymes (such as elastase) are able to destroy the alveolar tissue (the elastin fibre, for example). Most A1AD patients do not develop clinically significant emphysema, but smoking and severely decreased A1AT levels (10-15%) can cause emphysema at a young age. In all, A1AD causes about 2% of all emphysema. However, smokers with A1AD are in the highest risk category for emphysema.
- While A1AD provides some insight into the pathogenesis of the disease, hereditary A1AT deficiency only accounts for a small proportion of the disease. Studies for the better part of the past century have focused mainly upon the putative role of leukocyte elastase (also neutrophil elastase), a serine protease found in neutrophils, as a primary contributor to the connective tissue damage seen in the disease. This hypothesis, a result of the observation that NE is the primary substrate for A1AT, and A1AT is the primary inhibitor of NE, together have been known as the “protease-antiprotease” theory, implicating neutrophils as an important mediator of the disease. However, more recent studies have brought into light the possibility that one of the many other numerous proteases, especially matrix metalloproteases might be equally or more relevant than NE in the development of non-hereditary emphysema.
- The better part of the past few decades of research into the pathogenesis of emphysema involved animal experiments where various proteases were instilled into the trachea of various species of animals. These animals developed connective tissue damage, which was taken as support for the protease-antiprotease theory. However, just because these substances can destroy connective tissue in the lung, as anyone would be able to predict, doesn’t establish causality. More recent experiments have focused on more technologically advanced approaches, such as ones involving genetic manipulation. Perhaps the most interesting development with respect to our understanding of the disease involves the production of protease “knock-out” animals, which are genetically deficient in one or more proteases, and the assessment of whether they would be less susceptible to the development of the disease
Gross Pathology
Microscopic Pathology
References
Causes
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Causes
The most common cause is cigarette smoking.
Causes by Organ System
| Cardiovascular | No underlying causes |
| Chemical/Poisoning | No underlying causes |
| Dental | No underlying causes |
| Dermatologic | No underlying causes |
| Drug Side Effect | Thalidomide |
| Ear Nose Throat | No underlying causes |
| Endocrine | No underlying causes |
| Environmental | No underlying causes |
| Gastroenterologic | No underlying causes |
| Genetic | No underlying causes |
| Hematologic | No underlying causes |
| Iatrogenic | No underlying causes |
| Infectious Disease | No underlying causes |
| Musculoskeletal/Orthopedic | No underlying causes |
| Neurologic | No underlying causes |
| Nutritional/Metabolic | No underlying causes |
| Obstetric/Gynecologic | No underlying causes |
| Oncologic | No underlying causes |
| Ophthalmologic | No underlying causes |
| Overdose/Toxicity | No underlying causes |
| Psychiatric | No underlying causes |
| Pulmonary | No underlying causes |
| Renal/Electrolyte | No underlying causes |
| Rheumatology/Immunology/Allergy | No underlying causes |
| Sexual | No underlying causes |
| Trauma | No underlying causes |
| Urologic | No underlying causes |
| Miscellaneous | No underlying causes |
References
Differentiating Emphysema from other Diseases
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: : Mehrian Jafarizade, M.D [2]
Overview
Emphysema must be differentiated from other diseases presenting with cough, shortness of breath and tachypnea, such as congestive heart failure, asthma, bronchiectasis, and bronchiolitis obliterans, pulmonary embolism, pericarditis, and vasculitis.
Differential Diagnosis
Emphysema should be differentiated from other diseases presenting with chronic cough, shortness of breath and tachypnea. The differentials include the following:[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]
| Diseases | Diagnostic tests | Physical Examination | Symptoms | Past medical history | Other Findings | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| CT scan and MRI | EKG | Chest X-ray | Tachypnea | Tachycardia | Fever | Chest Pain | Hemoptysis | Dyspnea on Exertion | Wheezing | Chest Tenderness | Nasalopharyngeal Ulceration | Carotid Bruit | |||
| Pulmonary embolism |
|
|
|
✔ | ✔ | ✔ (Low grade) | ✔ | ✔ (In case of massive PE) | ✔ | – | – | – | – |
|
|
| Congestive heart failure |
|
✔ | ✔ | ✔ | – | – | ✔ | – | – | – | – |
|
| ||
| Percarditis |
|
|
|
✔ | ✔ | ✔ (Low grade) | ✔ (Relieved by sitting up and leaning forward) | – | ✔ | – | – | – | – |
|
|
| Pneumonia |
|
|
|
✔ | ✔ | ✔ | ✔ | – | ✔ | ✔ | – | – | – |
|
|
| Vasculitis |
|
|
✔ | ✔ | ✔ | ✔ | ✔ | ✔ | – | ✔ | ✔ | ✔ |
|
||
| Chronic obstructive pulmonary disease (COPD) |
|
|
✔ | ✔ | – | – | – | ✔ | ✔ | – | – | – |
|
| |
Features Specific for Congestive Heart Failure
Chronic obstructive pulmonary disease (COPD) may be confused with congestive heart failure due to similar presentations like wheezing and shortness of breath. Features specific to congestive heart failure are:
- Orthopnea
- Paroxysmal nocturnal dyspnea
- Fine crackles on ausculatation
- Chest X ray findings of cardiac enlargement, pulmonary congestion (Kerley B lines, and pleural effusion)
- The peak expiratory flow is low in COPD whereas there is higher flow in heart failure
- Comet-tail sign on ultrasonography is a good indicator of heart failure–related dyspnea
Features Specific for Bronchiectasis
- Copious purulent sputum
- Coarse crackles
- Clubbing
- CT findings suggestive of Bronchiectasis.
Features Specific for Bronchiolitis Obliterans
- History of collagen vascular disease.
- Young patient usually without a history of smoking
- CT scan shows finding of mosaic attenuation and no evidence of emphysema.
Features Specific for Chronic Asthma
- Chronic asthma responds well to bronchodilators.
- Normal diffusion capacity of lung on pulmonary function test.
References
- ↑ Brenes-Salazar JA (2014). “Westermark’s and Palla’s signs in acute and chronic pulmonary embolism: Still valid in the current computed tomography era”. J Emerg Trauma Shock. 7 (1): 57–8. doi:10.4103/0974-2700.125645. PMC 3912657. PMID 24550636.
- ↑ “CT Angiography of Pulmonary Embolism: Diagnostic Criteria and Causes of Misdiagnosis | RadioGraphics”.
- ↑ Bĕlohlávek J, Dytrych V, Linhart A (2013). “Pulmonary embolism, part I: Epidemiology, risk factors and risk stratification, pathophysiology, clinical presentation, diagnosis and nonthrombotic pulmonary embolism”. Exp Clin Cardiol. 18 (2): 129–38. PMC 3718593. PMID 23940438.
- ↑ “Pulmonary Embolism: Symptoms – National Library of Medicine – PubMed Health”.
- ↑ Ramani GV, Uber PA, Mehra MR (2010). “Chronic heart failure: contemporary diagnosis and management”. Mayo Clin. Proc. 85 (2): 180–95. doi:10.4065/mcp.2009.0494. PMC 2813829. PMID 20118395.
- ↑ Blinderman CD, Homel P, Billings JA, Portenoy RK, Tennstedt SL (2008). “Symptom distress and quality of life in patients with advanced congestive heart failure”. J Pain Symptom Manage. 35 (6): 594–603. doi:10.1016/j.jpainsymman.2007.06.007. PMC 2662445. PMID 18215495.
- ↑ Hawkins NM, Petrie MC, Jhund PS, Chalmers GW, Dunn FG, McMurray JJ (2009). “Heart failure and chronic obstructive pulmonary disease: diagnostic pitfalls and epidemiology”. Eur. J. Heart Fail. 11 (2): 130–9. doi:10.1093/eurjhf/hfn013. PMC 2639415. PMID 19168510.
- ↑ Takasugi JE, Godwin JD (1998). “Radiology of chronic obstructive pulmonary disease”. Radiol. Clin. North Am. 36 (1): 29–55. PMID 9465867.
- ↑ Wedzicha JA, Donaldson GC (2003). “Exacerbations of chronic obstructive pulmonary disease”. Respir Care. 48 (12): 1204–13, discussion 1213–5. PMID 14651761.
- ↑ Nakawah MO, Hawkins C, Barbandi F (2013). “Asthma, chronic obstructive pulmonary disease (COPD), and the overlap syndrome”. J Am Board Fam Med. 26 (4): 470–7. doi:10.3122/jabfm.2013.04.120256. PMID 23833163.
- ↑ Khandaker MH, Espinosa RE, Nishimura RA, Sinak LJ, Hayes SN, Melduni RM, Oh JK (2010). “Pericardial disease: diagnosis and management”. Mayo Clin. Proc. 85 (6): 572–93. doi:10.4065/mcp.2010.0046. PMC 2878263. PMID 20511488.
- ↑ Bogaert J, Francone M (2013). “Pericardial disease: value of CT and MR imaging”. Radiology. 267 (2): 340–56. doi:10.1148/radiol.13121059. PMID 23610095.
- ↑ Gharib AM, Stern EJ (2001). “Radiology of pneumonia”. Med. Clin. North Am. 85 (6): 1461–91, x. PMID 11680112.
- ↑ Schmidt WA (2013). “Imaging in vasculitis”. Best Pract Res Clin Rheumatol. 27 (1): 107–18. doi:10.1016/j.berh.2013.01.001. PMID 23507061.
- ↑ Suresh E (2006). “Diagnostic approach to patients with suspected vasculitis”. Postgrad Med J. 82 (970): 483–8. doi:10.1136/pgmj.2005.042648. PMC 2585712. PMID 16891436.
- ↑ Stein PD, Dalen JE, McIntyre KM, Sasahara AA, Wenger NK, Willis PW (1975). “The electrocardiogram in acute pulmonary embolism”. Prog Cardiovasc Dis. 17 (4): 247–57. PMID 123074.
- ↑ Warnier MJ, Rutten FH, Numans ME, Kors JA, Tan HL, de Boer A, Hoes AW, De Bruin ML (2013). “Electrocardiographic characteristics of patients with chronic obstructive pulmonary disease”. COPD. 10 (1): 62–71. doi:10.3109/15412555.2012.727918. PMID 23413894.
- ↑ Stein PD, Matta F, Ekkah M, Saleh T, Janjua M, Patel YR, Khadra H (2012). “Electrocardiogram in pneumonia”. Am. J. Cardiol. 110 (12): 1836–40. doi:10.1016/j.amjcard.2012.08.019. PMID 23000104.
- ↑ Hazebroek MR, Kemna MJ, Schalla S, Sanders-van Wijk S, Gerretsen SC, Dennert R, Merken J, Kuznetsova T, Staessen JA, Brunner-La Rocca HP, van Paassen P, Cohen Tervaert JW, Heymans S (2015). “Prevalence and prognostic relevance of cardiac involvement in ANCA-associated vasculitis: eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis”. Int. J. Cardiol. 199: 170–9. doi:10.1016/j.ijcard.2015.06.087. PMID 26209947.
- ↑ Dennert RM, van Paassen P, Schalla S, Kuznetsova T, Alzand BS, Staessen JA, Velthuis S, Crijns HJ, Tervaert JW, Heymans S (2010). “Cardiac involvement in Churg-Strauss syndrome”. Arthritis Rheum. 62 (2): 627–34. doi:10.1002/art.27263. PMID 20112390.
Epidemiology and Demographics
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References
Risk Factors
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Risk Factors
Patients with alpha 1-antitrypsin deficiency (A1AD) are more likely to suffer from emphysema. A1AD allows inflammatory enzymes (such as elastase) to destroy the alveolar tissue. Most A1AD patients do not develop clinically significant emphysema, but smoking and severely decreased A1AT levels (10-15%) can cause emphysema at a young age. The type of emphysema caused by A1AD is known as panacinar emphysema (involving the entire acinus) as opposed to centrilobular emphysema, which is caused by smoking. Panacinar emphysema typically affects the lower lungs, while centrilobular emphysema affects the upper lungs. A1AD causes about 2% of all emphysema. Smokers with A1AD are at the greatest risk for emphysema. Mild emphysema can often develop into a severe case over a short period of time (1–2 weeks).
References
Screening
Natural History, Complications, and Prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Emphysema is an irreversible degenerative condition. The most important measure that can be taken to slow the progression of emphysema is for the patient to stop smoking and avoid all exposure to cigarette smoke and lung irritants.
Prognosis
Emphysema is an irreversible degenerative condition. The most important measure that can be taken to slow the progression of emphysema. Pulmonary rehabilitation can be very helpful to optimize the patient’s quality of life and teach the patient how to actively manage his or her care.
Emphysema is also treated by supporting the breathing with anticholinergics, bronchodilators and (inhaled or oral) steroid medication, and supplemental oxygen as required. Treating the patient’s other conditions including gastric reflux and allergies may also improve lung function. Supplemental oxygen used as prescribed (20+ hours/day) is the only non-surgical treatment which has been shown to prolong life in emphysema patients. Other medications are being researched. There are lightweight portable oxygen systems which allow patients increased mobility. Patients fly, cruise, and work while using supplemental oxygen.
Lung volume reduction surgery (LVRS) can improve the quality of life for certain carefully selected patients. It can be done by several different methods, some of which are minimally invasive. In July of 2006 a new treatment, placing tiny valves in passages leading to diseased lung areas, was announced to have good results- but 7% of patients suffered from partial lung collapse. The only known “cure” for emphysema is a lung transplant, although few patients are strong enough physically to survive the surgery. The combination of a patient’s age, oxygen deprivation and the side-effects of the medications used to treat emphysema cause damage to the kidneys, heart and other organs. Transplants also require the patient to take an anti-rejection drug regimen which suppresses the immune system and creates other medical problems.
A study published by the European Respiratory Journal suggests that tretinoin (commercially available as Accutane, an anti-acne drug) derived from vitamin A can reverse the effects of emphysema in mice by returning elasticity (and regenerating lung tissue through gene mediation) to the alveoli.[1][2] While vitamin A consumption is not known to be an effective treatment or prevention for the disease, this research could in the future lead to a cure. A newer follow-up study done in 2006 found inconclusive results (“no definitive clinical benefits”) using Vitamin A (retinoic acid) in treatment of emphysema in humans and stated that further research is needed to reach conclusions on this treatment.[3]
References
- ↑ Mao J, Goldin J, Dermand J, Ibrahim G, Brown M, Emerick A, McNitt-Gray M, Gjertson D, Estrada F, Tashkin D, Roth M (2002). “A pilot study of all-trans-retinoic acid for the treatment of human emphysema”. Am J Respir Crit Care Med. 165 (5): 718–23. PMID 11874821.
- ↑ “Vitamin may cure smoking disease”. BBC News. December 22, 2003. Retrieved 2006-11-18. Check date values in:
|date=(help) - ↑ Roth M, Connett J, D’Armiento J, Foronjy R, Friedman P, Goldin J, Louis T, Mao J, Muindi J, O’Connor G, Ramsdell J, Ries A, Scharf S, Schluger N, Sciurba F, Skeans M, Walter R, Wendt C, Wise R (2006). “Feasibility of retinoids for the treatment of emphysema study”. Chest. 130 (5): 1334–45. PMID 17099008.
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