{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T20:26:42Z","timestamp":1790368002501,"version":"4.1.0"},"reference-count":40,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2011,6,2]],"date-time":"2011-06-02T00:00:00Z","timestamp":1306972800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2011,7,4]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Common variable immunodeficiency (CVID) is a common primary immune deficiency, caused by undefined defects in lymphocyte function, and is treated routinely by immunoglobulin substitution. CVID complications include airway disease (AD) and interstitial lung disease (ILD). It was not known if AD and ILD in CVID have a common immunological aetiology and should be considered separate features of the same disease, or as distinct syndromes that require specialized monitoring and treatment. We used high-resolution computed tomography (CT) to diagnose AD or ILD in paediatric CVID patients. Spirometry and body plethysmography did not differentiate between ILD and AD. Patients with AD (n\u2003=\u200311, 20%) developed more pneumonias while children with ILD (n\u2003=\u20038, 15%) showed immune dysregulation characterized by autoimmune complications, more severe memory B cell reduction and expansion of non-naive cytotoxic T cells. In conclusion, ILD and AD in CVID have dissimilar clinical and immunological characteristics, suggesting distinct aetiology requiring tailored monitoring and treatment of these patient subgroups.<\/jats:p>","DOI":"10.1111\/j.1365-2249.2011.04425.x","type":"journal-article","created":{"date-parts":[[2011,6,3]],"date-time":"2011-06-03T11:35:34Z","timestamp":1307100934000},"page":"235-242","source":"Crossref","is-referenced-by-count":33,"title":["Airway and interstitial lung disease are distinct entities in paediatric common variable immunodeficiency"],"prefix":"10.1093","volume":"165","author":[{"given":"A A J M","family":"van de Ven","sequence":"first","affiliation":[{"name":"Department of Pediatric Immunology and Infectious Diseases, University Medical Center Utrecht and Wilhelmina Children's Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P A","family":"de Jong","sequence":"additional","affiliation":[{"name":"Department of Radiology, University Medical Center Utrecht"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D P Hoytema","family":"van Konijnenburg","sequence":"additional","affiliation":[{"name":"Department of Pediatric Immunology and Infectious Diseases, University Medical Center Utrecht and Wilhelmina Children's Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"O A M","family":"Kessels","sequence":"additional","affiliation":[{"name":"Department of Radiology, University Medical Center Utrecht"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Boes","sequence":"additional","affiliation":[{"name":"Department of Pediatric Immunology and Infectious Diseases, University Medical Center Utrecht and Wilhelmina Children's Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E A M","family":"Sanders","sequence":"additional","affiliation":[{"name":"Department of Pediatric Immunology and Infectious Diseases, University Medical Center Utrecht and Wilhelmina Children's Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S W J","family":"Terheggen-Lagro","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pulmonology, University Medical Center Utrecht and Wilhelmina Children's Hospital, Utrecht, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J M","family":"van Montfrans","sequence":"additional","affiliation":[{"name":"Department of Pediatric Immunology and Infectious Diseases, University Medical Center Utrecht and Wilhelmina Children's Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2011,6,2]]},"reference":[{"key":"2021122708473743200_b1","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1016\/S0140-6736(08)61199-X","article-title":"Common variable immunodeficiency: a new look at an old disease","volume":"372","author":"Park","year":"2008","journal-title":"Lancet"},{"key":"2021122708473743200_b2","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1182\/blood-2007-11-124545","article-title":"Common variable immunodeficiency disorders: division into distinct clinical phenotypes","volume":"112","author":"Chapel","year":"2008","journal-title":"Blood"},{"key":"2021122708473743200_b3","doi-asserted-by":"crossref","first-page":"620","DOI":"10.1097\/RCT.0b013e31802e3c11","article-title":"Pulmonary and sinusal changes in 45 patients with primary immunodeficiencies: computed tomography evaluation","volume":"31","author":"Bondioni","year":"2007","journal-title":"J Comput Assist Tomogr"},{"key":"2021122708473743200_b4","first-page":"69","article-title":"Pulmonary complications in primary hypogammaglobulinemia: a survey by high resolution CT scan","volume":"65","author":"Gharagozlou","year":"2006","journal-title":"Monaldi Arch Chest Dis"},{"key":"2021122708473743200_b5","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1378\/chest.106.2.490","article-title":"Radiologic findings of adult primary immunodeficiency disorders. Contribution of CT","volume":"106","author":"Obregon","year":"1994","journal-title":"Chest"},{"key":"2021122708473743200_b6","doi-asserted-by":"crossref","first-page":"828","DOI":"10.1097\/01.rct.0000228163.08968.26","article-title":"Lung diseases in patients with common variable immunodeficiency: chest radiographic, and computed tomographic findings","volume":"30","author":"Tanaka","year":"2006","journal-title":"J Comput Assist Tomogr"},{"key":"2021122708473743200_b7","first-page":"179","article-title":"Humoral immunity in bronchiectasis","volume":"59","author":"Barker","year":"1987","journal-title":"Ann Allergy"},{"key":"2021122708473743200_b8","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1016\/0091-6749(91)90306-9","article-title":"Retrospective analysis of the incidence of pulmonary disease in hypogammaglobulinemia","volume":"88","author":"Sweinberg","year":"1991","journal-title":"J Allergy Clin Immunol"},{"key":"2021122708473743200_b9","doi-asserted-by":"crossref","first-page":"1001","DOI":"10.1067\/mai.2002.124999","article-title":"Efficacy of intravenous immunoglobulin in the prevention of pneumonia in patients with common variable immunodeficiency","volume":"109","author":"Busse","year":"2002","journal-title":"J Allergy Clin Immunol"},{"key":"2021122708473743200_b10","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S1081-1206(10)60853-8","article-title":"Pulmonary complications of common variable immunodeficiency","volume":"98","author":"Busse","year":"2007","journal-title":"Ann Allergy Asthma Immunol"},{"key":"2021122708473743200_b11","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1006\/clim.1999.4725","article-title":"Common variable immunodeficiency: clinical and immunological features of 248 patients","volume":"92","author":"Cunningham-Rundles","year":"1999","journal-title":"Clin Immunol"},{"key":"2021122708473743200_b12","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1016\/j.jaci.2004.05.057","article-title":"Granulomatous\u2013lymphocytic lung disease shortens survival in common variable immunodeficiency","volume":"114","author":"Bates","year":"2004","journal-title":"J Allergy Clin Immunol"},{"key":"2021122708473743200_b13","doi-asserted-by":"crossref","first-page":"1108","DOI":"10.1259\/0007-1285-69-828-1108","article-title":"Chest high resolution CT in adults with primary humoral immunodeficiency","volume":"69","author":"Feydy","year":"1996","journal-title":"Br J Radiol"},{"key":"2021122708473743200_b14","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1093\/qjmed\/95.10.655","article-title":"Common variable immune deficiency: respiratory manifestations, pulmonary function and high-resolution CT scan findings","volume":"95","author":"Thickett","year":"2002","journal-title":"QJM"},{"key":"2021122708473743200_b15","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1016\/j.ejrad.2004.09.005","article-title":"The HRCT appearances of granulomatous pulmonary disease in common variable immune deficiency","volume":"54","author":"Park","year":"2005","journal-title":"Eur J Radiol"},{"key":"2021122708473743200_b16","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1023\/A:1025373601374","article-title":"Common variable immunodeficiency patient classification based on impaired B cell memory differentiation correlates with clinical aspects","volume":"23","author":"Piqueras","year":"2003","journal-title":"J Clin Immunol"},{"key":"2021122708473743200_b17","doi-asserted-by":"crossref","first-page":"1544","DOI":"10.1182\/blood.V99.5.1544","article-title":"Severe deficiency of switched memory B cells (CD27(+)IgM(\u2212)IgD(\u2212)) in subgroups of patients with common variable immunodeficiency: a new approach to classify a heterogeneous disease","volume":"99","author":"Warnatz","year":"2002","journal-title":"Blood"},{"key":"2021122708473743200_b18","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1182\/blood-2007-06-091744","article-title":"The EUROclass trial: defining subgroups in common variable immunodeficiency","volume":"111","author":"Wehr","year":"2008","journal-title":"Blood"},{"key":"2021122708473743200_b19","doi-asserted-by":"crossref","first-page":"1178","DOI":"10.1016\/j.jaci.2007.10.001","article-title":"Transmembrane activator and calcium-modulating cyclophilin ligand interactor mutations in common variable immunodeficiency: clinical and immunologic outcomes in heterozygotes","volume":"120","author":"Zhang","year":"2007","journal-title":"J Allergy Clin Immunol"},{"key":"2021122708473743200_b20","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1177\/1753465808096948","article-title":"Therapies for interstitial lung disease: past, present and future","volume":"2","author":"Kim","year":"2008","journal-title":"Ther Adv Respir Dis"},{"key":"2021122708473743200_b21","doi-asserted-by":"crossref","first-page":"793","DOI":"10.1111\/j.1399-3038.2009.00963.x","article-title":"Detection of pulmonary complications in common variable immunodeficiency","volume":"21","author":"Touw","year":"2010","journal-title":"Pediatr Allergy Immunol"},{"key":"2021122708473743200_b22","doi-asserted-by":"crossref","first-page":"794","DOI":"10.1007\/s002470050235","article-title":"Clinical utility of high-resolution pulmonary computed tomography in children with antibody deficiency disorders","volume":"27","author":"Manson","year":"1997","journal-title":"Pediatr Radiol"},{"key":"2021122708473743200_b23","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1007\/s004310051004","article-title":"Computed tomography scan assessment of lung disease in primary immunodeficiencies","volume":"158","author":"Newson","year":"1999","journal-title":"Eur J Pediatr"},{"key":"2021122708473743200_b24","doi-asserted-by":"crossref","first-page":"1101","DOI":"10.1136\/adc.88.12.1101","article-title":"Pulmonary and sinus diseases in primary humoral immunodeficiencies with chronic productive cough","volume":"88","author":"Rusconi","year":"2003","journal-title":"Arch Dis Child"},{"key":"2021122708473743200_b25","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1378\/chest.09-2398","article-title":"A CT scan score for the assessment of lung disease in children with common variable immunodeficiency disorders","volume":"138","author":"van de Ven","year":"2010","journal-title":"Chest"},{"key":"2021122708473743200_b26","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/j.clim.2009.11.010","article-title":"Lymphocyte characteristics in children with common variable immunodeficiency","volume":"135","author":"van de Ven","year":"2010","journal-title":"Clin Immunol"},{"key":"2021122708473743200_b27","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1006\/clim.1999.4799","article-title":"Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies)","volume":"93","author":"Conley","year":"1999","journal-title":"Clin Immunol"},{"key":"2021122708473743200_b28","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1016\/j.clim.2009.05.020","article-title":"Refined characterization and reference values of the pediatric T- and B-cell compartments","volume":"133","author":"van Gent","year":"2009","journal-title":"Clin Immunol"},{"key":"2021122708473743200_b29","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1007\/BF01314939","article-title":"The systematic evaluation of the chest radiograph in cystic fibrosis","volume":"2","author":"Chrispin","year":"1974","journal-title":"Pediatr Radiol"},{"key":"2021122708473743200_b30","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1002\/ppul.10280","article-title":"Correlation of six different cystic fibrosis chest radiograph scoring systems with clinical parameters","volume":"35","author":"Terheggen-Lagro","year":"2003","journal-title":"Pediatr Pulmonol"},{"key":"2021122708473743200_b31","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.rmed.2010.07.020","article-title":"Reference values for paediatric pulmonary function testing: the Utrecht dataset","volume":"105","author":"Koopman","year":"2011","journal-title":"Respir Med"},{"key":"2021122708473743200_b32","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1126\/science.278.5335.138","article-title":"NF-AT activation induced by a CAML-interacting member of the tumor necrosis factor receptor superfamily","volume":"278","author":"von Bulow","year":"1997","journal-title":"Science"},{"key":"2021122708473743200_b33","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1016\/S1074-7613(01)00130-3","article-title":"Regulation of the T-independent humoral response by TACI","volume":"14","author":"von Bulow","year":"2001","journal-title":"Immunity"},{"key":"2021122708473743200_b34","doi-asserted-by":"crossref","first-page":"1594","DOI":"10.1378\/chest.122.5.1594","article-title":"Pulmonary interstitial disease in Ig deficiency","volume":"122","author":"Popa","year":"2002","journal-title":"Chest"},{"key":"2021122708473743200_b35","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1097\/RCT.0b013e3181cad9da","article-title":"Common variable immunodeficiency: computed tomography evaluation of bronchopulmonary changes including nodular lesions in 40 patients. Correlation with clinical and immunological data","volume":"34","author":"Bondioni","year":"2010","journal-title":"J Comput Assist Tomogr"},{"key":"2021122708473743200_b36","doi-asserted-by":"crossref","first-page":"1199","DOI":"10.1164\/ajrccm.159.4.9807067","article-title":"Viruses and bacteria in bronchial samples from patients with primary hypogammaglobulinemia","volume":"159","author":"Kainulainen","year":"1999","journal-title":"Am J Respir Crit Care Med"},{"key":"2021122708473743200_b37","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1016\/j.clim.2009.05.001","article-title":"Granulomatous disease in common variable immunodeficiency","volume":"13","author":"Ardeniz","year":"2009","journal-title":"Clin Immunol"},{"key":"2021122708473743200_b38","doi-asserted-by":"crossref","first-page":"613","DOI":"10.7326\/0003-4819-127-8_Part_1-199710150-00005","article-title":"Granulomatous disease in common variable immunodeficiency","volume":"127","author":"Mechanic","year":"1997","journal-title":"Ann Intern Med"},{"key":"2021122708473743200_b39","doi-asserted-by":"crossref","first-page":"6236","DOI":"10.4049\/jimmunol.159.12.6236","article-title":"TNF and lymphotoxin-alpha polymorphisms associated with common variable immunodeficiency: role in the pathogenesis of granulomatous disease","volume":"159","author":"Mullighan","year":"1997","journal-title":"J Immunol"},{"key":"2021122708473743200_b40","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1084\/jem.20050381","article-title":"Possible role of human herpesvirus 8 in the lymphoproliferative disorders in common variable immunodeficiency","volume":"202","author":"Wheat","year":"2005","journal-title":"J Exp Med"}],"container-title":["Clinical and Experimental Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1365-2249.2011.04425.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/academic.oup.com\/cei\/article-pdf\/165\/2\/235\/41908415\/j.1365-2249.2011.04425.x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/cei\/article-pdf\/165\/2\/235\/41908415\/j.1365-2249.2011.04425.x.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,6,7]],"date-time":"2023-06-07T05:24:59Z","timestamp":1686115499000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/cei\/article\/165\/2\/235\/6422923"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,6,2]]},"references-count":40,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2011,6,2]]},"published-print":{"date-parts":[[2011,7,4]]}},"URL":"https:\/\/doi.org\/10.1111\/j.1365-2249.2011.04425.x","relation":{},"ISSN":["0009-9104","1365-2249"],"issn-type":[{"value":"0009-9104","type":"print"},{"value":"1365-2249","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2011,8]]},"published":{"date-parts":[[2011,6,2]]}}}