A novel presentation of Berylliosis treated with bronchial artery embolisation

A 78-year-old male was referred to the respiratory clinic after presenting to his GP with progressive haemoptysis and expectorating metallic fragments. He worked as a dental technician milling Beryllium compounds for 10 years in his youth without personal protective equipment. There was no recollection of an acute respiratory illness. Oxygen saturations were 90-92%. Blood tests showed anaemia, Hb 86g/l.

Berylliosis is characterised in this case, on radiograph (Fig. 1) and on CT imaging by interlobular septal thickening, bronchiectasis and a pattern of predominantly upper lobe fibrosis (Fig. 3) [1]. Additionally, lung cavitation and progressive massive fibrosis (PMF) (note the increased nodularity caused by beryllium deposition) are also observed (Fig. 2a, 2b). Finally, the hypertrophied bronchial artery adjacent to the lung cavity can be seen, which was the target for bronchial artery embolisation (BAE) (Fig. 4). Pre and post-selective BAE shows resolution of blush (Fig. 5, 6). Expectorated metallic fragments (Fig. 7).

Background

Berylliosis is a rare pneumoconiosis and is considered a rare disease. Acute berylliosis causes pneumonitis, with associated symptoms of coughing and dyspnoea, immediately after exposure. However, chronic berylliosis, as in this case, is manifested by multisystem granulomata secondary to macrophage activation caused by Beryllium [2,3]. Whilst cessation of exposure to Beryllium is essential, there is no cure and treatment aims to slow disease progression and alleviate symptoms predominantly with corticosteroids, immunosuppressants and supportive therapies.

Clinical Perspective

Chronic berylliosis typically presents with a dry cough and shortness of breath as well as fatigue, weight loss and night sweats [2]. Haemoptysis and life-threatening pulmonary bleeding are not commonly seen [4]. Symptoms have a latency period of 1 month to 40 years although on average occur 10-15 years post exposure. Berylliosis is occasionally misdiagnosed as sarcoidosis as symptoms and radiological features can be similar. A detailed occupational history is crucial to diagnosis although lung biopsy and bloods tests (Beryllium lymphocyte proliferation test (BeLPT)) are alternatives. We present an uncommon presentation of progressive haemoptysis and expectorating metallic fragments (see Fig. 6) and novel use of BAE in treatment. The expectorated metallic fragments are thought to be calcified granulomas formed around Beryllium micro-particles.

In recurrent or progressive haemoptysis imaging is required for causative pathology, particularly exclusion of thromboembolic causes and vascular abnormalities. Computed tomography (CT) revealed a hypertrophied bronchial artery. Given the proximity of the hypertrophied bronchial artery to the lung cavity, it is also pertinent to consider the vascular supply of the lungs as this informed the decision to offer BAE.

In the normal physiological state ~99% of pulmonary blood flow is supplied by the pulmonary arteries, which is then used in gas exchange [5]. The lungs also receive a systemic bronchial arterial supply which delivers oxygenated blood to the lung parenchyma, at the level of the terminal bronchioles before return via the pulmonary venous supply. The bronchial arteries typically originate from the descending aorta and as such, are at pressures five to six times greater than the pulmonary arterial supply. Hypertrophy of these collateral vessels is common in conditions that compromise pulmonary artery supply, such as Berylliosis (Fig. 2a). After discussion with the clinical team, BAE was undertaken.

Imaging Perspective

Bronchial artery hypertrophy is an important finding and may be amenable to embolisation.

Expectorated metallic fragments, seen in the lung cavity are in direct communication with the airway.

Outcome

BAE was successful, and the patient reported significant improvement in haemoptysis; however, continued to expectorate metallic fragments.

Take Home Message / Teaching Points

Detailed occupational history is key to diagnosis of Berylliosis and BAE is a potential treatment for haemoptysis; however, not all hypertrophied bronchial arteries require embolisation given procedural risks.

Written informed patient consent for publication has been obtained.