McCullagh Brian N, Comellas Alejandro P, Ballas Zuhair K, Newell John D, Zimmerman M Bridget, Azar Antoine E
Division of Pulmonary and Critical Care, Department of Internal Medicine, University of Iowa Hospitals and Clinics, Iowa City, Iowa, United States of America.
Division of Immunology, Department of Internal Medicine, University of Iowa Hospitals and Clinics, Iowa City, Iowa, United States of America.
PLoS One. 2017 Feb 17;12(2):e0172437. doi: 10.1371/journal.pone.0172437. eCollection 2017.
Chronic Obstructive Pulmonary Disease is the third leading cause of death in the US, and is associated with periodic exacerbations, which account for the largest proportion of health care utilization, and lead to significant morbidity, mortality, and worsening lung function. A subset of patients with COPD have frequent exacerbations, occurring 2 or more times per year. Despite many interventions to reduce COPD exacerbations, there is a significant lack of knowledge in regards to their mechanisms and predisposing factors. We describe here an important observation that defines antibody deficiency as a potential risk factor for frequent COPD exacerbations. We report a case series of patients who have frequent COPD exacerbations, and who were found to have an underlying primary antibody deficiency syndrome. We also report on the outcome of COPD exacerbations following treatment in a subset with of these patients with antibody deficiency. We identified patients with COPD who had 2 or more moderate to severe exacerbations per year; immune evaluation including serum immunoglobulin levels and pneumococcal IgG titers was performed. Patients diagnosed with an antibody deficiency syndrome were treated with either immunoglobulin replacement therapy or prophylactic antibiotics, and their COPD exacerbations were monitored over time. A total of 42 patients were identified who had 2 or more moderate to severe COPD exacerbations per year. Twenty-nine patients had an underlying antibody deficiency syndrome: common variable immunodeficiency (8), specific antibody deficiency (20), and selective IgA deficiency (1). Twenty-two patients had a follow-up for at least 1 year after treatment of their antibody deficiency, which resulted in a significant reduction of COPD exacerbations, courses of oral corticosteroid use and cumulative annual dose of oral corticosteroid use, rescue antibiotic use, and hospitalizations for COPD exacerbations. This case series identifies antibody deficiency as a potentially treatable risk factor for frequent COPD exacerbations; testing for antibody deficiency should be considered in difficult to manage frequently exacerbating COPD patients. Further prospective studies are warranted to further test this hypothesis.
慢性阻塞性肺疾病是美国第三大死因,与周期性加重相关,周期性加重占医疗保健利用率的最大比例,并导致显著的发病率、死亡率和肺功能恶化。一部分慢性阻塞性肺疾病患者频繁加重,每年发作2次或更多次。尽管有许多干预措施来减少慢性阻塞性肺疾病的加重,但对于其机制和易感因素仍存在重大知识空白。我们在此描述一项重要观察结果,将抗体缺乏定义为慢性阻塞性肺疾病频繁加重的潜在风险因素。我们报告了一系列慢性阻塞性肺疾病频繁加重且被发现存在潜在原发性抗体缺乏综合征的患者病例。我们还报告了这些抗体缺乏患者亚组治疗后慢性阻塞性肺疾病加重的结果。我们确定了每年有2次或更多次中度至重度加重的慢性阻塞性肺疾病患者;进行了包括血清免疫球蛋白水平和肺炎球菌IgG滴度在内的免疫评估。被诊断为抗体缺乏综合征的患者接受免疫球蛋白替代疗法或预防性抗生素治疗,并随时间监测其慢性阻塞性肺疾病的加重情况。共确定了42例每年有2次或更多次中度至重度慢性阻塞性肺疾病加重的患者。29例患者存在潜在的抗体缺乏综合征:常见变异型免疫缺陷(8例)、特异性抗体缺乏(20例)和选择性IgA缺乏(1例)。22例患者在抗体缺乏治疗后至少随访1年,结果慢性阻塞性肺疾病加重、口服糖皮质激素使用疗程和口服糖皮质激素累积年剂量、急救抗生素使用以及因慢性阻塞性肺疾病加重住院均显著减少。该病例系列确定抗体缺乏是慢性阻塞性肺疾病频繁加重的潜在可治疗风险因素;对于难以管理的频繁加重的慢性阻塞性肺疾病患者,应考虑检测抗体缺乏。有必要进行进一步的前瞻性研究以进一步验证这一假设。