To the Editors:
McGrath et al. 1 recently reported the case of a middle-aged Caucasian female with a diagnosis of diffuse panbronchiolitis (DPB) and highlighted the difficulties of securing such a diagnosis in populations in which it is rarely described, i.e. those of Western/non-Asian origin. This was in response to a narrative review by Poletti et al. 2 describing current diagnostic criteria and demographic features. We would like to emphasise this point in describing the case of a 45-yr-old English-born Caucasian male who was recently assessed and treated at our centre. The patient was a lifelong nonsmoker with no Asian ancestry and had never travelled outside Europe. He had been referred to the respiratory clinic by his primary care physician on account of a 3-yr history of worsening exertional breathlessness and chronic productive cough associated with persistent, purulent rhinorrhoea. His symptoms had persisted and indeed worsened in the face of treatment with a high dose inhaled corticosteroid (ICS) used in conjunction with a long-acting β2-agonist (LABA) with repeated courses of oral corticosteroids. At first assessment, bibasal crackles and wheeze were present on examination of the chest. Spirometry revealed a moderately severe obstructive pattern with a forced expiratory volume in one second (FEV1) of 1.7 L (55% pred), a forced vital capacity (FVC) of 3.1 L (78% pred). Plain chest radiograph revealed bibasal nodular infiltrates and Haemophilus influenzae was isolated on culture of sputum and bronchoalveolar lavage fluid. High-resolution computed tomography demonstrated extensive small airway plugging with a “tree-in-bud” pattern associated with early lower lobe bronchiectasis (fig. 1⇓). A clinical diagnosis of DPB was made and he commenced 500 mg of erythromycin twice daily. Over the course of the following 6 months, he experienced a dramatic improvement; his productive cough and rhinnorhoea resolved completely, exercise tolerance returned to normal, and lung field infiltrates on plain chest radiograph resolved while his FEV1 and FVC improved to supra-predicted values (fig. 2⇓). During this period, his ICS/LABA therapy was not altered. Over the following period the erythromycin dose was reduced to 250 mg twice daily, but within a few weeks he had experienced a recurrence of symptoms and a small, but persistent, drop in FEV1. This improved on increasing the erythromycin dose to 500 mg twice daily, which he remains on at the present time. Although the patient did not undergo surgical lung biopsy, the clinico-radiological presentation and response to macrolide therapy is highly consistent with a diagnosis of DPB.
High resolution computed tomography image showing extensive small airway plugging with a “tree-in-bud” pattern (white arrowheads) associated with early lower lobe bronchiectasis (white arrow).
Forced expiratory volume in one second (FEV1; ○) and forced vital capacity (FVC; •) from October 2006 to May 2007 of a 45-yr-old English-born Caucasian male. ·····: predicted FEV1; - - - - -: predicted FVC.
This case highlights the importance of considering this rare illness in patients of non-Asian origin who present with difficult airways disease. Without appropriate macrolide therapy, the patient faces the prospect of a disease characterised by inexorable decline and early death; however, with appropriate therapy the patient can expect a far better prognosis and quality of life.
Statement of interest
None declared.
- © ERS Journals Ltd
