Description

Chronic berylliosis is a multisystemic disorder which occurs in patients who develop a T-cell immune response following exposure to beryllium. Noncaseating granulomas may develop in several organs, but especially involve the lung.


 

Diagnostic criteria for beryllium lung disease

 

(1) history of exposure to beryllium

(1a) exposures are typically for more than 2 years (acute exposures may result in acute beryllium disease which presents as an acute toxic pneumonitis)

(1b) exposure usually involves inhalation of either dust or fumes containing beryllium

(1c) analysis of urine or tissue for beryllium can document exposure but do not correlate with disease status

 

(2) beryllium-specific immune response

(2a) positive peripheral blood lymphocyte transformation test

(2b) positive lymphocyte transformation test using lymphocytes from bronchoalveolar lavage

 

(3) histopathology on lung biopsy compatible with berylliosis

(3a) mononuclear cell infiltrate

(3b) noncaseating granulomas

(3c) interstitial fibrosis

 

(4) clinical findings

(4a) respiratory signs or symptoms (dyspnea, chest pain, weight loss, fatigue, arthralgias; sometimes digital clubbing, cyanosis, hepatosplenomegaly, or respiratory crackles; in advanced disease pulmonary hypertension and cor pulmonale)

(4b) reticulonodular infiltrate on chest radiographs or other imaging technique, with mild hilar adenopathy; mild disease may show a diffuse, finely granular haziness with relative sparing of the lung bases and apices

(4c) restrictive and/or obstructive physiologic pattern

(4d) decreased diffusing capacity for carbon monoxide

 

Diagnosis:

(1) All four elements must be present for the diagnosis.

(2) Other causes for the respiratory symptoms (bacterial pneumonia, effects of smoking, etc) should be excluded or at least demonstrated as being unable to explain all available evidence.

 


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