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Immunoglobulin G Subclass Deficiencies in Adult Patients with Chronic Airway Diseases

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Immunoglobulin G Subclass Deficiencies in Adult Patients with Chronic Airway Diseases ORIGINAL ARTICLE Immunology, Allergic Disorders & Rheumatology http://dx.doi.org/10.3346/jkms.2016.31.10.1560 • J Korean Med Sci 2016; 31: 1560-1565 Immunoglobulin G Subclass Deficiencies in Adult Patients with Chronic Airway Diseases Joo-Hee Kim,1 Sunghoon Park,1 Immunoglobulin G subclass deficiency (IgGSCD) is a relatively common primary Yong Il Hwang,1 Seung Hun Jang,1 immunodeficiency disease (PI) in adults. The biological significance of IgGSCD in patients Ki-Suck Jung,1 Yun Su Sim,1 with chronic airway diseases is controversial. We conducted a retrospective study to Cheol-Hong Kim,1 Changhwan Kim,2 characterize the clinical features of IgGSCD in this population. This study examined the 1 and Dong-Gyu Kim medical charts from 59 adult patients with IgGSCD who had bronchial asthma or chronic obstructive pulmonary disease (COPD) from January 2007 to December 2012. Subjects 1Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, Hallym were classified according to the 10 warning signs developed by the Jeffrey Modell University Medical Center, Anyang, Korea; 2Division Foundation (JMF) and divided into two patient groups: group I (n = 17) met ≥ two JMF of Pulmonary and Allergy, Department of Medicine, criteria, whereas group II (n = 42) met none. IgG3 deficiency was the most common Jeju National University Hospital, Jeju, Korea subclass deficiency (88.1%), followed by IgG4 (15.3%). The most common infectious Received: 12 February 2016 complication was pneumonia, followed by recurrent bronchitis, and rhinosinusitis. The Accepted: 12 June 2016 numbers of infections, hospitalizations, and exacerbations of asthma or COPD per year were significantly higher in group I than in group II (P < 0.001, P = 0.012, and P < 0.001, Address for Correspondence: respectively). The follow-up mean forced expiratory volume (FEV1) level in group I was Dong-Gyu Kim, MD Division of Pulmonary, Allergy, and Critical Care Medicine, significantly lower than it was at baseline despite treatment of asthma or COPD Department of Medicine, Hallym University Medical Center, (P = 0.036). In conclusion, IgGSCD is an important PI in the subset of patients with chronic 22 Gwanpyeong-ro 170-beon-gil, Dongan-gu, Anyang 14068, Korea airway diseases who had recurrent upper and lower respiratory infections as they presented E-mail: [email protected] with exacerbation-prone phenotypes, decline in lung function, and subsequently poor Funding: This study was supported by Hallym University prognosis. Research Fund 2014 (HURF-2014-58). Keywords: Asthma; Chronic Obstructive Lung Diseases; IgG Subclass Deficiency; Respiratory Tract Infection INTRODUCTION characteristic features including chronic rhinosinusitis, irre- versible airflow limitation, and bronchiectatic changes on ra- Asthma and chronic obstructive pulmonary disease (COPD) diologic studies. Previous studies using COPD cohorts suggest- are the most common chronic airway diseases worldwide. They ed that patients suffering from frequent exacerbations belonged differ from each other in their patterns of inflammation, immu- to a distinct phenotype associated with an accelerated decline nological mechanisms, and the extent of reversible airflow limi- in lung function, reduced physical activity, poorer quality of life tations (1). However, exacerbations of both asthma and COPD (QOL), and increased risk of mortality (5). In patients with mod- result in significant morbidity and healthcare costs and, in some erate-to-severe COPD, the prevalence of bronchiectasis was cases, death. Upper respiratory infections are known as factors higher and it was associated with COPD exacerbation and hos- that trigger acute exacerbations, with viruses as the primary pitalization (6). These exacerbations were likely caused by the causative agents (2). In addition, bacterial infections are impor- complex interactions between the host, respiratory viruses, air- tant factors in the exacerbation of COPD; such infections can way bacteria, environmental pollution, increased susceptibility act as the primary cause of lower respiratory infections or result to viruses and bacteria, and both innate and adaptive immune from secondary complications of viral infections (3). dysfunction. Recurrent exacerbations and poorly controlled conditions Adult-onset primary immunodeficiency diseases (PIs) are cause declining lung function and subsequent airway remodel- mostly humoral immune deficiencies, such as common vari- ing (4). Previous asthma cohort studies have described the pres- able immunodeficiencies, hypogammaglobulinemia, immu- ence of several asthma phenotypes in asthmatics, with one sub- noglobulin G subclass deficiency (IgGSCD), and selective IgA set showing an intrinsic phenotype of exacerbation-prone asth- deficiency (7,8). Previous studies have shown that IgGSCD may ma (2). This subset had a history of multiple exacerbations re- be associated with increased susceptibility to sinopulmonary quiring three or more bursts of oral corticosteroids per year and infections and airway obstruction in patients with chronic bron- © 2016 The Korean Academy of Medical Sciences. pISSN 1011-8934 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. eISSN 1598-6357 Kim J-H, et al. • IgG Subclass Deficiencies in Chronic Airway Diseases chitis or COPD (9-11). In severe asthmatics, immunoglobulin Table 1. Immunoglobulin G subclass deficiencies in the study subjects replacement provided corticosteroid sparing effect and reduc- No. (%) of patients ed hospital admissions (12). However, asymptomatic individu- IgG subclasses All Group I Group II P value als who have decreased levels of one or more IgG subclasses (n = 59) (n = 17) (n = 42) can have normal responses to polysaccharide antigens and pres- IgG1 (382.4-928.6 mg/dL) 1 (1.7) 0 (0.0) 1 (2.4) 1.000 ent no infectious complications. Presentation due to recurrent IgG2 (241.8-700.3 mg/dL) 1 (1.7) 0 (0.0) 1 (2.4) 1.000 IgG3 (21.8-176.1 mg/dL) 52 (88.1) 14 (82.4) 38 (90.5) 0.399 infections has long been the hallmark of immunodeficiency, IgG4 (3.9-86.4 mg/dL) 9 (15.3) 6 (35.2) 3 (7.1) 0.013 yet, more recently it is becoming recognized that some patients may present with other alterations in immunity, such as auto- immunity (13). Diagnosis of PI is challenging and relies on clin- Baseline data and exacerbation variables ical suspicion and laboratory confirmation. The 10 Warning The medical records of each patient included age, sex, smoking Signs of PI, developed for the purpose of early diagnosis and history, previous medications, comorbidity, type of infection, awareness of PI, are based on expert consensus developed at exacerbation, sputum eosinophil count, total IgE, and atopic the Jeffrey Modell Foundation (JMF). This system is currently status. Asthma or COPD exacerbations were defined as wors- used as a screening tool for the diagnosis of PI (14). ening of respiratory symptoms that required treatment with an- We conducted this study to assess IgGSCD and its correla- tibiotics or systemic glucocorticoids alone or in combination tion with infectious complications in patients with chronic air- for > 3 days in an outpatient clinic or hospitalization. Sputum way diseases such as asthma or COPD using the 10 warning signs specimens for bacteria culture were obtained during the exac- of PI. Furthermore, we aimed to evaluate the clinical value of erbations. Pneumonia was defined as a new chest radiographic serum IgG subclass levels in this population. infiltration plus respiratory symptoms such as cough, dyspnea, fever, discolored sputum or pleuritic chest pain. Patients with- MATERIALS AND METHODS out the evidence of pneumonic infiltration on chest X-ray were diagnosed as having bronchitis and recurrent bronchitis was Study subjects defined as three or more episodes of acute bronchitis per year. We enrolled 59 patients with IgGSCD who were diagnosed with Atopy was defined as 1) at least one positive specific IgE, or 2) bronchial asthma, asthma COPD overlap syndrome (ACOS) or one positive skin-prick test to aeroallergens (cats, dogs, house COPD between January 2007 and December 2012. Asthma was dust mites, trees, grasses, weeds and fungi). The skin-prick test defined as episodic respiratory symptoms and reversible air- was considered positive with a wheal diameter ≥ 3 mm and er- flow obstruction with bronchodilator response (BDR) or posi- ythema ≥ 10 mm compared with the negative control. tivity to the methacholine bronchoprovocation test. Patients with COPD had incompletely reversible airflow obstruction Pulmonary function tests with a post-bronchodilator ratio of forced expiratory volume in Spirometry was conducted using handheld spirometers (Vmax 1 second (FEV1) to forced vital capacity (FVC) < 70%, a post- 2130; Sensor Medics, Yorba Linda, CA, USA) and was perform- bronchodilator FEV1 < 80% of predicted, and no airway hyper- ed and interpreted according to the American Thoracic Society responsiveness (AHR) or BDR. Patients with ACOS had respira- recommendations (18). The instruments were calibrated ac- tory symptoms and positive bronchodilator responses as well cording to the manufacturers’ instructions before each testing as incompletely reversible airflow obstruction (post-broncho- day. All spirometry tests were performed by three trained
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