Armstead Joanne, Morris Julie, Denning David W
National Aspergillosis Centre, University Hospital South Manchester, The University of Manchester and Manchester Academic Health Science Centre, Manchester, United Kingdom.
PLoS One. 2014 Jun 10;9(6):e98502. doi: 10.1371/journal.pone.0098502. eCollection 2014.
Aspergillus spp. can lead to allergic bronchopulmonary aspergillosis (ABPA), Aspergillus sensitisation and Aspergillus bronchitis in CF. The relative frequencies of these entities have recently been ascertained in a large UK adult CF cohort. We have used this data to estimate the burden of aspergillosis and ABPA cases in adult CF patients in 30 countries reporting CF. National and international CF registry data was accessed and assessed for completeness and age distribution. Published proportions of ABPA (17.7%), Aspergillus sensitisation (14.6%) and Aspergillus bronchitis (30%) in CF were applied to those >18 years and compared with notified ABPA cases. Of the 76,201 estimated CF patients worldwide (not including India), 37,714 were >18 years. The proportion of adults to children varied from 63% in Norway to 20% in Brazil. ABPA caseload in adults is anticipated to be 6,675 cases of which only 2,221 cases (33%) are currently recorded, indicating substantial underdiagnosis. The ABPA diagnosis rate compared with estimated rates varies by country from 101% (France) to 14.5% (Greece), although genetic variation could account for genuine differences compared with the UK. Aspergillus bronchitis is not currently recognised or recorded in CF registries but there are an anticipated 10,988 adult cases. Aspergillus sensitisation, associated with increased bronchiectasis and reduced FEV1, affects an anticipated 5,506 patients without ABPA or Aspergillus bronchitis. Together ABPA and Aspergillus bronchitis are estimated to affect 17,989 adults, 47.7% of the adult CF population. ABPA also occurs in children and teenagers and 984 cases were documented in registries. Diagnosed ABPA rates by age were available for the ECFS registry, USA, UK, Ireland, Belgium and Netherlands. The rate was <1% under 4 years, and increased throughout childhood and adolescence, with marked variation between countries. Newly published diagnostic criteria and methods should facilitate better recognition of aspergillosis in CF, allowing better CF disease control.
曲霉属真菌可导致囊性纤维化（CF）患者发生变应性支气管肺曲霉病（ABPA）、曲霉致敏及曲霉性支气管炎。最近在一个大型英国成年CF队列中确定了这些疾病的相对发病率。我们利用这些数据估算了30个报告有CF病例的国家中成年CF患者曲霉病和ABPA病例的负担。获取了国家和国际CF登记数据，并评估了其完整性和年龄分布情况。将已公布的CF患者中ABPA（17.7%）、曲霉致敏（14.6%）和曲霉性支气管炎（30%）的比例应用于18岁以上患者，并与已通报的ABPA病例进行比较。在全球估计的76,201例CF患者中（不包括印度），37,714例年龄超过18岁。成人与儿童的比例从挪威的63%到巴西的20%不等。预计成人ABPA病例数为6,675例，其中目前仅记录到2,221例（33%），这表明存在大量漏诊情况。与估计发病率相比，ABPA的诊断率因国家而异，从101%（法国）到14.5%（希腊），不过与英国相比，基因变异可能是造成真正差异的原因。目前CF登记处未认可或记录曲霉性支气管炎，但预计有10,988例成年病例。曲霉致敏与支气管扩张增加和第一秒用力呼气容积（FEV1）降低相关，预计会影响5,506例无ABPA或曲霉性支气管炎的患者。据估计，ABPA和曲霉性支气管炎共同影响17,989名成年人，占成年CF患者群体的47.7%。ABPA也发生于儿童和青少年，登记处记录了984例病例。欧洲囊性纤维化协会（ECFS）登记处、美国、英国、爱尔兰、比利时和荷兰提供了按年龄划分的确诊ABPA发病率。4岁以下发病率<1%，在整个儿童期和青春期发病率上升，各国之间存在显著差异。新公布的诊断标准和方法应有助于更好地识别CF患者中的曲霉病，从而更好地控制CF疾病。
