McDonnell Melissa J, Ahmed Mohammed, Das Jeeban, O'Mahony Michael, Breen David, O'Regan Anthony, Gilmartin J J, Bruzzi John, Rutherford Robert M
Department of Respiratory Medicine, Galway University Hospitals, Galway, Ireland.
Respiration. 2017;93(6):406-414. doi: 10.1159/000464252. Epub 2017 Apr 20.
Middle-lobe predominant bronchiectasis affecting the right middle-lobe and/or lingula (RMLP) is classically described in asthenic, elderly females with skeletal abnormalities or associated nontuberculous mycobacterial (NTM) infection.
We aimed to evaluate the frequency and clinical characteristics of patients with an RMLP phenotype in a cohort of newly diagnosed bronchiectasis patients and determine associations with disease severity.
A retrospective observational cross-sectional cohort study of consecutive bronchiectasis patients in our institution was performed. Data were collected on baseline variables, microbiology status, lung function, and radiology according to the modified Bhalla score. Disease severity was assessed using bronchiectasis severity index (BSI) and FACED severity scores.
Of 81 patients (mean age [SD] 62.6 [12.4], females 55 [67.9%], BMI 26.9 [5.7%]), 20 (24.7%) had RMLP disease. These patients were significantly younger, female, and with lower BMIs than patients with the classical bronchiectasis phenotype (p = 0.03, 0.01, and p <0.01, respectively). Fewer symptoms of cough and daily sputum (p = 0.01 and <0.01), prior exacerbation frequency (p = 0.03), and higher baseline forced expiratory volume (p = 0.04) were noted. A higher incidence of NTM at diagnosis was demonstrated (p = 0.01). BSI and FACED severity scores in RMLP patients were significantly lower than their counterparts (both p < 0.001).
The RMLP phenotype is associated with younger patients than classically described in the literature. An increased rate of NTM infection in this phenotype was noted, particularly in females, but much lower than previously described. Lung function and disease severity scores in this patient group are relatively normal, suggesting a milder phenotype in patients with this form of the disease.
中叶为主型支气管扩张累及右中叶和/或舌叶（RMLP），典型表现于体型虚弱的老年女性，伴有骨骼异常或非结核分枝杆菌（NTM）感染。
我们旨在评估新诊断支气管扩张患者队列中RMLP表型患者的频率和临床特征，并确定其与疾病严重程度的相关性。
对我院连续的支气管扩张患者进行回顾性观察性横断面队列研究。根据改良的巴哈拉评分收集基线变量、微生物学状态、肺功能和放射学数据。使用支气管扩张严重指数（BSI）和FACED严重程度评分评估疾病严重程度。
81例患者（平均年龄[标准差]62.6[12.4]，女性55例[67.9%]，BMI 26.9[5.7%]）中，20例（24.7%）患有RMLP疾病。与典型支气管扩张表型患者相比，这些患者明显更年轻、女性居多且BMI更低（分别为p = 0.03、0.01和p <0.01）。咳嗽和每日咳痰症状较少（p = 0.01和<0.01），既往加重频率较低（p = 0.03），基线用力呼气量较高（p = 0.04）。诊断时NTM发病率较高（p = 0.01）。RMLP患者的BSI和FACED严重程度评分显著低于其他患者（均p <0.001）。
RMLP表型与比文献中经典描述更年轻的患者相关。该表型中NTM感染率增加，尤其是女性，但远低于先前描述。该患者组的肺功能和疾病严重程度评分相对正常，表明这种疾病形式的患者表型较轻。
