Berylliosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anthony Gallo, B.S. [2]
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]
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.
Epidemiology and Demographics
- The prevalence of beryllium sensitization is approximately 9% to 19 % depend on industry.
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.