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Diffuse panbronchiolitis: East meets West

St. Vincent's University Hospital Dublin, Dublin, Ireland.

To the Editors:

In the paper by Poletti et al. 1 on diffuse panbronchiolitis, the authors describe the diagnostic criteria for this condition and emphasise its predominance in East Asia, having associations with human leukocyte antigen (HLA)-Bw54 and HLA-A11.

We would like to highlight the difficulties and pitfalls in reaching this rare diagnosis in a European cohort of patients by describing the unusual presentation of this condition in a Caucasian female, the first case described in Ireland.

A 48-yr-old female attended outpatient clinic for her fifth annual asthma review. She had experienced four infective exacerbations in the previous 12 months and reported recent large-volume sputum production, associated with worsening dyspnoea on exertion. Computed tomography (CT) thorax scan revealed bronchiectasis. The patient was treated over the subsequent 3 yrs for exacerbations of asthma and bronchiectasis.

Over this period, her pulmonary function tests began to show severe small airways obstruction. The patient was now experiencing severe dyspnoea on minimal exertion and was becoming unresponsive to inhaled bronchodilators and high-dose oral steroids. Pulmonary function testing at this point revealed airflow obstruction, with a reduced forced expiratory volume in one second (FEV₁) of 1.67 L·min^–1 (66% predicted), a forced vital capacity (FVC) of 2.31 L·min^–1 (78% pred) and a diffusing capacity of the lung for carbon monoxide that was 49% pred. Bronchiolitis/bronchiolectasis was now suspected. Further CT scanning demonstrated more extensive changes of bronchiectasis, associated with fine nodular opacities in both lung bases. In contrast to the usual pattern, this had a more distal distribution. The combination of these CT findings and small airways obstruction without large airway involvement on pulmonary function testing prompted referral for thoracoscopic lung biopsy. Exhaustive analysis of the tissue, including a second opinion from an international centre, revealed a histological diagnosis of diffuse panbronchiolitis. The patient was commenced on erythromycin 500 mg b.i.d. with a dramatic response. Macrolide treatment was continued over the next 2 yrs with a significant reduction in sputum production, improvement in spirometry (FEV₁ 2.35 L·min^–1 and FVC 3.9 L·min^–1), resolution of wheeze and improved exercise tolerance comparable to that of 10 yrs earlier. Over the next few years, attempts were made to reduce erythromycin therapy with little success. The patient's debilitating symptoms always returned. She remained on erythromycin 500 mg b.i.d. maintenance dose and managed to complete a 200-mile sponsored walk.

This case highlights the importance of considering this diagnosis or certainly the forme fruste mentioned by Poletti et al. 1 in their review, in the group of patients with asthma and/or bronchiectasis who show no improvement with conventional therapies, including high-dose oral steroid, and who actually decline rapidly despite treatment. Something as simple as erythromycin could be the difference between severe morbidity or death and walking 200 miles!

REFERENCES

1. Poletti V, Casoni G, Chilosi M, Zompatori M. Diffuse panbronchiolitis. Eur Respir J 2006;28:862–871.[Abstract/Free Full Text]

This article has been cited by other articles:

				N. P. Adams and J. Congelton Diffuse panbronchiolitis Eur. Respir. J., July 1, 2008; 32(1): 237 - 238. [Full Text] [PDF]

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