Introduction

Berylliosis, also referred to as chronic beryllium disease (CBD), is a medical condition affecting the lungs, most commonly caused by exposure in the workplace to a metal called beryllium. If you are concerned that you may be developing this condition or have been exposed to high amounts of beryllium at work, this article can help you spot the signs of berylliosis.

What is beryllium?

Beryllium is a rare type of metal. Nearly all of the global supply comes from mining minerals which contain beryllium, from sites located in the United States, China and Kazakhstan.¹ Its properties of being strong, lightweight and non-magnetic² make beryllium particularly useful in aerospace and defence industries.

Where is beryllium used?

The properties of beryllium make it a versatile metal, and it can be used for:³

• Aeroplanes and machinery parts
• Satellites, or any other device using large mirrors
• Missiles, including nuclear weapons
• Radiation shields in medical imaging departments

Why is beryllium dangerous?

Beryllium is extracted by mining, which generates dust in the process. The tiny particles of beryllium present in this dust can be inhaled and cause lung changes, leading to the long-term harmful effects associated with berylliosis.

What risk factors can lead to berylliosis?

Nearly all cases of berylliosis can be linked to occupational exposure to beryllium-containing dust in the workplace.

What is occupational exposure?

Occupational exposure implies being around high levels of a harmful substance as part of your daily work. The most common means of contracting berylliosis is by inhaling beryllium-containing dust. Although miners are typically at highest risk, this also applies if you work in an industry which involves activities where beryllium could be aerosolised, for example, cutting sheets of beryllium to make components of machinery parts in the areas listed above.

Exposure to high levels of beryllium over long periods of time is the greatest risk of developing berylliosis, with an estimated 2-5% of workers exposed to beryllium in the air developing the condition.⁴

Are there genetic reasons that would increase my risk of berylliosis?

Cases of developing berylliosis without being directly exposed are very rare. Despite this, some individuals are more susceptible than others because of their genetic makeup.

The genetic component of berylliosis

Studies have found that individuals with mutations in the HLA-DPB1 gene, which produces key proteins for the functioning of the immune system. These people are much more sensitive to beryllium inhaled as dust, and so are more likely to develop berylliosis.⁷

Can I get berylliosis if I don’t work with beryllium?

Unless you live very close to a high-risk site, it is very unlikely that you will develop berylliosis.⁵ Levels of beryllium are present in the air we breathe, but such levels are so low that they will not cause the changes to the lungs seen in berylliosis, even in susceptible individuals. 

What causes berylliosis?

The first phase of the disease

Berylliosis is a form of fibrotic lung disease. It is thought that beryllium can irritate the linings of the lungs, which causes inflammation - an immune response to material the body does not recognise, the same way it responds to infections. In some individuals, this response is exaggerated and a larger-than-expected amount of inflammation occurs, forming pockets of inflammation called granulomas.⁶

The chronic phase of the disease

If this continues for a long period of time, it will cause scarring to the lungs. This is referred to as chronic inflammation and is responsible for the long-term symptoms that can be seen decades after the first exposure to beryllium.

What are the signs of berylliosis?

Berylliosis has different symptoms depending on the stage of the disease[8]. These may initially be mild, but can get progressively worse, with complications affecting both the lungs and the heart as the disease becomes advanced. The time taken for these symptoms to develop can vary between individuals, and it can take up to thirty years for the signs of berylliosis to appear.⁹

Initial symptoms

Early signs can include:

• Shortness of breath
• Feeling tired all the time
• Persistent dry cough

Signs of disease progression

In later stages, you may develop:

• Chest pain, especially when coughing or breathing deeply
• Night sweats
• Weight loss

Advanced disease

As the disease progresses, it starts to limit the function of the lungs and the heart. Care for individuals with this degree of disease will usually be managed between heart and lung specialist teams, especially if your disease has progressed to cause significant pulmonary fibrosis or right-sided heart failure.

How is berylliosis diagnosed?

Your healthcare provider will ask you questions relating to your symptoms and examine your heart and lungs. The appropriate further investigations will be performed if your doctor is concerned about berylliosis.¹⁰

What tests will usually be performed to investigate berylliosis?

• Blood tests  
• Imaging scans
  • Chest x-ray
  • Computed tomography of the chest (CT scan)
• Lung function tests, including spirometry

Are any specialist tests performed?

Beryllium lymphocyte proliferation test (BeLPT)

A specific blood test looking for changes in the number of white blood cells in individuals with known beryllium exposure. Higher volumes of white cells in the sample usually mean more advanced disease. 

Bronchoscopy

This is a camera test to look at the insides of the lungs, usually performed under mild sedation. The operator may also take a small sample of the lining of the lungs (biopsy) or wash the lungs with fluid and send this for analysis (bronchoalveolar lavage).

Are there other diseases with similar symptoms to berylliosis?

There are several other lung diseases where an individual can develop similar symptoms to berylliosis.¹¹ Your healthcare provider may also conduct tests to investigate:¹²

• Sarcoidosis
• Idiopathic pulmonary fibrosis
• Asthma
• Chronic obstructive pulmonary disease
• Histoplasmosis
• Tuberculosis
• Asbestosis
• Silicosis
• Lung cancer

What is the treatment for berylliosis?

Unfortunately, once you have a diagnosis of berylliosis, there is no means to cure. Prevention is best for preventing the onset of berylliosis.¹³ However, there are ways of managing the symptoms of this disease.

Monitoring of disease

Individuals with early symptoms may not require any direct treatments, and instead will be followed up at regular intervals. This may involve seeing a specialist once a year to have the relevant blood tests, scans and lung function tests to assess the extent of the disease.

Prevention of worsening disease

Stopping smoking is essential, as this can damage lungs and speed up the damage caused by berylliosis. Limiting or avoiding exposure to high levels of beryllium is also recommended where possible.¹⁴ You should also ensure that you have vaccinations against common infections such as influenza and pneumococcus (responsible for pneumonia). Individuals with lung damage are particularly vulnerable to these common infections, and if your lungs are already damaged with berylliosis, you will find it harder to recover from chest infections.  

Steroids

Steroid medications reduce inflammation and control short-term deteriorations in the disease. They will not reverse the damage to the lungs, but a short course of treatment can slow the progression of the disease.¹⁵ A high initial dose will be given, which is then gradually tapered down over a period of weeks until you are weaned off the steroid altogether. These medications come with a wide range of side effects, especially if used long-term, so they should only be used to control particularly bad flares of the disease.

Other future therapies

In similar conditions to berylliosis, there is potential for antifibrotic drugs and immunotherapy, or even lung transplant.¹⁶ There is, however, very little research into the impact of these methods on berylliosis specifically, so these are not currently available globally as treatment options.  

Advanced disease supportive treatments

As the disease progresses, the treatment focus will be on managing symptoms rather than controlling the disease. This is similar to the management of advanced pulmonary fibrosis, with most of the focus on oxygen therapy, pulmonary rehabilitation and control of symptoms.¹⁷  

How can I avoid getting berylliosis?

If you work in an environment with a high risk of exposure to beryllium, then appropriate adaptations should be made to your workplace. The Occupational Health and Safety Administration recommend the following for all workers in areas of high beryllium exposure:¹⁸

• High-quality ventilation in enclosed spaces
• Protective equipment, such as respirators
• Workplace education and training to raise awareness
• Regular medical exams are conducted in the workplace to screen workers for disease

What is the prognosis of berylliosis?

The progression of berylliosis varies between individuals. The risk for those exposed who develop acute berylliosis and develop advanced disease can be up to 38% over a lifetime.¹⁹ The prognosis can be improved by having regular assessments with healthcare providers and avoiding behaviours that can further damage the lungs, such as continuing to work in beryllium environments and smoking.

Summary

Berylliosis is a disorder of scarring to the lungs, typically caused by inhaling small particles of beryllium. Symptoms can take decades to develop, and include breathlessness, fatigue and cough. There is no cure, although some treatments can delay the progress of berylliosis, including smoking cessation, regular follow-up with trained healthcare professionals and the use of steroids to treat a flare. End-stage disease can be managed with supplementary oxygen.