给编辑:
麦格拉思et al.1recently 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 Polettiet al.2describing 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 andHaemophilus influenzaewas 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 FEV1and FVC improved to supra-predicted values (fig. 2×)。在此期间,他的ICS/LABA疗法没有改变。在接下来的时间里1。这可以改善每天两次将红霉素剂量增加到500毫克,目前他仍保持这种情况。尽管该患者没有进行手术肺活检,但临床 - 放射学表现和对大环内酯类治疗的反应与DPB的诊断高度一致。
该病例强调了在患有困难气道疾病的非亚洲起源患者中考虑这种罕见疾病的重要性。没有适当的大花环疗法,患者面临着一种疾病的前景,其特征是不可阻碍下降和早期死亡。但是,通过适当的疗法,患者可以期望预后和生活质量更好。
Statement of interest
没有人宣布。
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