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.
• In 1946, Hardy established the National Beryllium Registry at the Massachusetts General Hospital.

Classification

• There is no formal classification system established for berylliosis.
• Berylliosis may be classified as a rare disorder and an occupational disease.

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.

Causes

• Chronic exposure to beryllium
• Genetic predisposition ( Mutation at the HL-A DPB1 Glu69 position increase the prevalence of beryllium sensitization.

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 [disease name] is approximately [number or range] per 100,000 individuals worldwide.
• In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

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.

Diagnosis

History and Symptoms

• History of beryllium exposure
• Positive blood or bronchoalveolar lavage ( beryllium lymphocyte proliferation test BeLPT
• cough
• shortness of breath
• chest pain
• joint aches
• 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

History of beryllium exposure.

• Blood beryllium lymphocyte proliferation test ( BeLPT ) .
• High resolution computed tomography ( HRCT ).
• Bronchoalveolar lavage.
• Tissue biopsy.
• Pulmonary function test.
• Chest X ray.

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.
• 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 .
• Methotrxate 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.

References

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