Berylliosis: Difference between revisions
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{{SK}} Chronic beryllium disorder; CBD | {{SK}} Chronic beryllium disorder; CBD | ||
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*In each subsequent exposure macrophage and CD4+ helper T - lymphocytes accumulation in the lungs. | *In each subsequent exposure macrophage and CD4+ helper T - lymphocytes accumulation in the lungs. | ||
*As a result macrophages, CD4+ T lymphocyte and plasma cell accumulate to form noncaseating granulomas that cause fibrosis.<ref name="pmid292618662">{{cite journal |vauthors=Gossman WG, Bhimji SS |title= |journal= |volume= |issue= |pages= |date= |pmid=29261866 |doi= |url=}}</ref> | *As a result macrophages, CD4+ T lymphocyte and plasma cell accumulate to form noncaseating granulomas that cause fibrosis.<ref name="pmid292618662">{{cite journal |vauthors=Gossman WG, Bhimji SS |title= |journal= |volume= |issue= |pages= |date= |pmid=29261866 |doi= |url=}}</ref> | ||
*Beryllium is presented to CD4+ T cell by antigen presenting cells,principally in HLA-DP molecules. T cell in the blood lungs or other organs in turn recognize the beryllium, proliferate and form T cell clones.These colnes the release proinflammatory cytokines. such as tumor necrosis factor alpha , IL-2 and interferon gamma.These cytokines amplify the immune response, as result formation of mononuclear cell infiltrates and noncaseating granulomas in target organs where beryllium has deposited. | |||
*on average about 2 to 6 % of beryllium exposed people develop beryllium sensitized ( defined by positive blood lymphocyte proliferation salt in vitro ) with most progressing to disease.In certain high risk groups such as beryllium metal and alloy machinists, chronic beryllium disease prevalence is > 17 % workers with bystander exposures, such as secretaries and security guards, also develop sensitization and disease but at lower rates.The typical pathologic consequence is a diffuse pulmonary, hilar and mediastinal lymph node granulomatous reaction that is histologically indistinguishable from sarcoidosis. | |||
*Early granuloma formation with mononuclear and giant cells can also occur. | |||
*Many lymphocytes are found when cell are washed from the lungs ( bronchoalveolar lavage BAL during bronchoscopy. | |||
*These T cells proliferate when exposed to beryllium in vitro , much as the blood cells do ( a test called beryllium lymphocyte proliferation test BeLPT ). | |||
* | * | ||
==Causes== | ==Causes== | ||
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==Differentiating Berylliosis from other Diseases== | ==Differentiating Berylliosis from other Diseases== | ||
[[Granuloma]]s are seen in other chronic diseases, such as [[tuberculosis]], [[sarcoidosis]], and it can occasionally be hard to distinguish [[berylliosis]] from these disorders. | [[Granuloma]]s are seen in other chronic diseases, such as [[tuberculosis]], [[sarcoidosis]], and it can occasionally be hard to distinguish [[berylliosis]] from these disorders. | ||
==Risk Factors== | ==Risk Factors== | ||
*Extended exposure with berylium | *Extended exposure with berylium |
Revision as of 22:13, 16 September 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Synonyms and keywords: Chronic beryllium disorder; CBD
Overview
Berylliosis or chronic beryllium disorder (CBD) is an occupational lung disease. It is a chronic allergic-type lung response and chronic lung disease caused by exposure to beryllium and its compounds. The condition is incurable but symptoms can be treated.
Historical Perspective
- Berylliosis was first discovered by Dr. Harriet Hardy, an American pathologist, in 1945 following an outbreak of respiratory illnesses affecting nearby factory workers.[1][2]
- In 1946, Hardy established the National Beryllium Registry at the Massachusetts General Hospital.[2]
Classification
- There is no formal classification system established for berylliosis.
- Berylliosis may be classified as a rare disorder and an occupational disease.[3][4]
Pathophysiology
- Exposure to beryllium can cause cell mediated immunity .
- which can cause sensitize the T cells to beryllium
- In each subsequent exposure macrophage and CD4+ helper T - lymphocytes accumulation in the lungs.
- As a result macrophages, CD4+ T lymphocyte and plasma cell accumulate to form noncaseating granulomas that cause fibrosis.[5]
- Beryllium is presented to CD4+ T cell by antigen presenting cells,principally in HLA-DP molecules. T cell in the blood lungs or other organs in turn recognize the beryllium, proliferate and form T cell clones.These colnes the release proinflammatory cytokines. such as tumor necrosis factor alpha , IL-2 and interferon gamma.These cytokines amplify the immune response, as result formation of mononuclear cell infiltrates and noncaseating granulomas in target organs where beryllium has deposited.
- on average about 2 to 6 % of beryllium exposed people develop beryllium sensitized ( defined by positive blood lymphocyte proliferation salt in vitro ) with most progressing to disease.In certain high risk groups such as beryllium metal and alloy machinists, chronic beryllium disease prevalence is > 17 % workers with bystander exposures, such as secretaries and security guards, also develop sensitization and disease but at lower rates.The typical pathologic consequence is a diffuse pulmonary, hilar and mediastinal lymph node granulomatous reaction that is histologically indistinguishable from sarcoidosis.
- Early granuloma formation with mononuclear and giant cells can also occur.
- Many lymphocytes are found when cell are washed from the lungs ( bronchoalveolar lavage BAL during bronchoscopy.
- These T cells proliferate when exposed to beryllium in vitro , much as the blood cells do ( a test called beryllium lymphocyte proliferation test BeLPT ).
Causes
- Chronic exposure to beryllium
- Genetic predisposition ( Mutation at the HL-A DPB1 Glu69 position increase the prevalence of beryllium sensitization.[6]
Differentiating Berylliosis from other Diseases
Granulomas are seen in other chronic diseases, such as tuberculosis, sarcoidosis, and it can occasionally be hard to distinguish berylliosis from these disorders.
Risk Factors
- Extended exposure with berylium
- Genetic predisposition ( Mutation at the HL-A DPB1 Glu69 position increase the prevalence of beryllium sensitization.
Natural History, Complications, and Prognosis
Natural History
Ultimately, this process leads to restrictive lung disease, a decreased diffusion capacity.
Complications
Rarely, one can get granulomas in other organs including the liver.
Prognosis
Mortality rates range from 6 to 35 percent.The variability of mortality depend duration of beryllium exposure after development of chronic beryllium disorder disease, individual variation and duration of follow up.[7][8]
Diagnosis
History and Symptoms
- History of beryllium exposure
- Positive blood or bronchoalveolar lavage ( beryllium lymphocyte proliferation test BeLPT
- Cough
- Shortness of breath
- Chest pain
- Arthralgia
- Weight loss
- Fever
- The onset of symptoms can range from weeks up to tens of years from the initial exposure. In some individuals a single exposure can cause berylliosis.
Laboratory Test
- Blood beryllium lymphocyte proliferation test ( BeLPT )[9]
- High resolution computed tomography ( HRCT )
- Bronchoalveolar lavage
- Tissue biopsy
- Pulmonary function test
- Chest X ray[10]
Treatment
- There is no cure for CBD the goal is reducing symptoms and slow progression of disease.
- All patient with CBD should be removed from further exposure to beryllium decrease the progression of the disease.[11]
- There is no specific treatment for CBD but drug of choice is glucocorticoid therapy.
- Initial dose is 0.5 to 0.6 mg/k of prednisone for 6 to 12 week.
- Methotrexate is also used if patient not respond to glucocorticoid or has severe side effects from glucocorticoid.
- Once diagnosed and successfully treated, patients with CBD need long term followed-up with pulmonologist to monitor lung function.[12]
References
- ↑ HARDY HL, TABERSHAW IR (1946). "Delayed chemical pneumonitis occurring in workers exposed to beryllium compounds". J Ind Hyg Toxicol. 28: 197–211. PMID 21000285.
- ↑ 2.0 2.1 Oakes EH. Encyclopedia of World Scientists. Infobase Publishing; 2007.
- ↑ Berylliosis. National Organization for Rare Diseases (2009). https://rarediseases.org/rare-diseases/berylliosis/ Accessed on January 24, 2017.
- ↑ United States Department of Labor. Occupational Safety and Health Administration. https://www.osha.gov/SLTC/beryllium/healtheffects.html Accessed on January 24, 2017.
- ↑ Gossman WG, Bhimji SS. PMID 29261866. Missing or empty
|title=
(help) - ↑ Li L, Silveira LJ, Hamzeh N, Gillespie M, Mroz PM, Mayer AS, Fingerlin TE, Maier LA (June 2016). "Beryllium-induced lung disease exhibits expression profiles similar to sarcoidosis". Eur. Respir. J. 47 (6): 1797–808. doi:10.1183/13993003.01469-2015. PMC 5134922. PMID 27103383.
- ↑ Boffetta P, Fordyce TA, Mandel JS (December 2016). "A mortality study of beryllium workers". Cancer Med. 5 (12): 3596–3605. doi:10.1002/cam4.918. PMC 5224864. PMID 27766788.
- ↑ Boffetta P, Fordyce TA, Mandel JS (December 2016). "A mortality study of beryllium workers". Cancer Med. 5 (12): 3596–3605. doi:10.1002/cam4.918. PMC 5224864. PMID 27766788.
- ↑ Harber P, Su J, Alongi G (August 2014). "Beryllium BioBank: 2. Lymphocyte proliferation testing". J. Occup. Environ. Med. 56 (8): 857–60. doi:10.1097/JOM.0000000000000199. PMID 25099413.
- ↑ Mayer A, Hamzeh N (March 2015). "Beryllium and other metal-induced lung disease". Curr Opin Pulm Med. 21 (2): 178–84. doi:10.1097/MCP.0000000000000140. PMID 25602804.
- ↑ Balmes JR, Abraham JL, Dweik RA, Fireman E, Fontenot AP, Maier LA, Muller-Quernheim J, Ostiguy G, Pepper LD, Saltini C, Schuler CR, Takaro TK, Wambach PF (November 2014). "An official American Thoracic Society statement: diagnosis and management of beryllium sensitivity and chronic beryllium disease". Am. J. Respir. Crit. Care Med. 190 (10): e34–59. doi:10.1164/rccm.201409-1722ST. PMID 25398119.
- ↑ Thomas CA, Deubner DC, Stanton ML, Kreiss K, Schuler CR (July 2013). "Long-term efficacy of a program to prevent beryllium disease". Am. J. Ind. Med. 56 (7): 733–41. doi:10.1002/ajim.22175. PMID 23450749.