Parambil Joseph G, Yi Eunhee S, Ryu Jay H
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Respirology. 2009 Apr;14(3):443-8. doi: 10.1111/j.1440-1843.2008.01445.x. Epub 2008 Dec 16.
Obstructive bronchiolar disease or constrictive bronchiolitis, particularly in non-transplant patients, is poorly understood. This study identified the associated diseases, presenting features, and clinical course of obstructive bronchiolar disease identified by CT in the non-transplant adult population.
Retrospective single-centre study of 29 consecutive patients clinically diagnosed to have an obstructive bronchiolar disease based on the presence of respiratory symptoms and abnormal CT findings consisting of mosaic perfusion pattern with air trapping.
The median age was 54 years (range, 25-80 years); 20 were women (69%) and four patients (14%) had a smoking history. All 29 patients presented with respiratory symptoms, predominantly dyspnoea. The most common cause of obstructive bronchiolar disease was rheumatoid arthritis (34%). Other causes included hypersensitivity pneumonitis, multiple carcinoid tumorlets, Sjögren's syndrome, paraneoplastic pemphigus, inflammatory bowel disease and Swyer-James syndrome. The underlying cause was not identifiable in nine patients (31%), that is, cryptogenic constrictive bronchiolitis. An obstructive pattern was seen on pulmonary function testing in most patients (86%) with the exception of those with hypersensitivity pneumonitis and extreme obesity. Management usually included corticosteroid therapy, inhaled and oral, and bronchodilator therapy. Additional medications included macrolides, cytotoxic agents and other immunomodulator therapy. Pharmacologic therapy did not provide improvement in pulmonary function in the majority of patients but the follow-up data were limited.
Diverse causes and underlying diseases are associated with obstructive bronchiolar disease diagnosed radiologically in the non-transplant adult population. Rheumatoid arthritis-associated and cryptogenic constrictive bronchiolitis are found in over one-half of these patients. Most patients with obstructive bronchiolar disease do not appear to improve with currently available therapy.
阻塞性细支气管疾病或缩窄性细支气管炎，尤其是在非移植患者中，目前了解甚少。本研究确定了非移植成年人群中通过CT诊断的阻塞性细支气管疾病的相关疾病、临床表现及临床病程。
对29例连续患者进行回顾性单中心研究，这些患者基于呼吸道症状及异常CT表现（包括马赛克灌注模式伴空气潴留）临床诊断为阻塞性细支气管疾病。
中位年龄为54岁（范围25 - 80岁）；20例为女性（69%），4例患者（14%）有吸烟史。所有29例患者均有呼吸道症状，主要为呼吸困难。阻塞性细支气管疾病最常见的病因是类风湿关节炎（34%）。其他病因包括过敏性肺炎、多发性类癌瘤、干燥综合征、副肿瘤性天疱疮、炎症性肠病及斯维综合征。9例患者（31%）病因不明，即隐源性缩窄性细支气管炎。除过敏性肺炎和极度肥胖患者外，大多数患者（86%）肺功能检查呈阻塞性模式。治疗通常包括吸入和口服糖皮质激素及支气管扩张剂治疗。其他药物包括大环内酯类、细胞毒性药物及其他免疫调节剂治疗。大多数患者药物治疗后肺功能未改善，但随访数据有限。
多种病因及潜在疾病与非移植成年人群中经影像学诊断的阻塞性细支气管疾病相关。其中超过一半的患者为类风湿关节炎相关及隐源性缩窄性细支气管炎。大多数阻塞性细支气管疾病患者目前的治疗似乎未改善病情。
