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Berylliosis

A form of pneumoconiosis, berylliosis, or beryllium poisoning, is a systemic granulomatous disorder with dominant pulmonary manifestations. It occurs in two forms: acute nonspecific pneumonitis and chronic noncaseating granulomatous disease with interstitial fibrosis, which may cause death from respiratory failure and corpulmonale. Most patients with chronic interstitial disease become only slightly to moderately disabled by impaired lung function and other symptoms, but with each acute exacerbation, the prognosis worsens.

Causes of Berylliosis

Berylliosis results from inhalation of beryllium or from its absorption through the skin. Its severity varies with the amount inhaled. The mechanism by which beryllium exerts its toxic effect is unknown.

This disease occurs among beryllium alloy workers, cathode ray tube makers' gas mantle makers, fluorescent light workers, missile technicians, and nuclear reactor workers; it's generally associated with the milling and use of beryllium and, less commonly, with the mining of berylore. Families of beryllium workers and people who live near plants where beryllium alloy is used are also at risk for berylliosis.

Signs and symptoms of Berylliosis

Absorption of beryllium through broken skin produces an itchy rash that usually subsides within 2 weeks after exposure. A "beryllium ulcer" results from accidental implantation of beryllium metal in the skin.

Respiratory features :- Respiratory signs and symptoms of acute berylliosis include swelling and ulceration of nasal mucosa, which may progress to septal perforation, tracheitis, and bronchitis (dry cough). Acute pulmonary disease may develop rapidly (within 3 days) or weeks later, producing a progressive dry cough, tightness in the chest, substernal pain, tachycardia, and signs of bronchitis. This form of the disease has a significant mortality related to respiratory failure.

About 10% of patients with acute berylliosis develop chronic disease 10 to IS years after exposure. The chronic form causes increasing dyspnea that becomes progressively unremitting, along with mild chest pain, dry unproductive cough, and tachypnea. Pneumothorax may occur, with pulmonary scarring and bleb formation.

CLINICAL TIP Cardiovascular complications of berylliosis include pulmonary hypertension, right ventricular hypertrophy, and cor pulmonale. Other clinical features include hepatosplenomegaly, renal calculi, lymphadenopathy, anorexia, and fatigue.

Diagnosis

The patient history reveals occupational, family, or neighborhood exposure to beryllium dust, fumes, or mist. In acute berylliosis. chest X-rays may suggest pulmonary edema, showing acute miliary process or a patchy acinus filling, and diffuse infiltrates with prominent peribronchial markings. In chronic berylliosis, X-rays show reticulonodular infiltrates, hilar adenopathy, and large coalescent infiltrates in both lungs.

Pulmonary function studies show decreased vital capacity, forced vital capacity, residual volume and total lung capacity, and diffusing capacity of the lungs for carbon monoxide as well as decreased compliance as the lungs stiffen from fibrosis. Arterial blood gas analysis shows decreased partial pressure of arterial oxygen (Pao2) and partial pressure of arterial carbon dioxide The following additional tests may be performed:

• In vitro lymphoblast transformation test diagnoses berylliosis and monitors workers for occupational exposure to beryllium.
• Beryllium patch test establishes only hypersensitivity to beryllium, not the presence of disease.
• Tissue biopsy and spectrographic analysis are positive for most exposed workers but not absolutely diagnostic.
• Urinalysis may show beryllium in urine, but this only indicates exposure.

Differential diagnosis must rule out sarcoidosis and granulomatous infections.

Treatment of Berylliosis

• Beryllium ulcer requires excision or curettage. Acute berylliosis requires prompt corticosteroid therapy.
• Hypoxia may require oxygen administration by nasal cannula or mask (1 to 2 L/minute). Severe respiratory failure requires mechanical ventilation if Pao2 can't be maintained above 40 mm Hg.
• Chronic berylliosis is usually treated with corticosteroids, although it's not certain that steroids alter the progression of the disease. Lifelong maintenance therapy may be necessary.
• Respiratory symptoms may be treated with bronchodilators, increased fluid intake (at least 3 qt [3 L] daily), and chest physiotherapy techniques. Diuretics, digitalis glycosides, and salt restriction may be useful in patients with cor pulmonale.

Special considerations and prevention

• Teach the patient to prevent infection by avoiding crowds and persons with infection and by receiving influenza and pneumococcal vaccines.
• Encourage the patient to practice physical reconditioning, energy conservation in daily activities, and relaxation techniques.