{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T16:20:29Z","timestamp":1779294029741,"version":"3.51.4"},"reference-count":30,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2021,11,16]],"date-time":"2021-11-16T00:00:00Z","timestamp":1637020800000},"content-version":"vor","delay-in-days":1544,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100008322","name":"CSL Behring","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008322","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2017,10,6]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:p>Many patients with primary immunodeficiency (PID) who have antibody deficiency develop progressive lung disease due to underlying subclinical infection and inflammation. To understand how these patients are monitored we conducted a retrospective survey based on patient records of 13 PID centres across Europe, regarding the care of 1061 adult and 178 paediatric patients with PID on immunoglobulin (Ig) G replacement. The most common diagnosis was common variable immunodeficiency in adults (75%) and hypogammaglobulinaemia in children (39%). The frequency of clinic visits varied both within and between centres: every 1\u201312 months for adult patients and every 3\u20136 months for paediatric patients. Patients diagnosed with lung diseases were more likely to receive pharmaceutical therapies and received a wider range of therapies than patients without lung disease. Variation existed between centres in the frequency with which some clinical and laboratory monitoring tests are performed, including exercise tests, laboratory testing for IgG subclass levels and specific antibodies, and lung function tests such as spirometry. Some tests were carried out more frequently in adults than in children, probably due to difficulties conducting these tests in younger children. The percentage of patients seen regularly by a chest physician, or who had microbiology tests performed following chest and sinus exacerbations, also varied widely between centres. Our survey revealed a great deal of variation across Europe in how frequently patients with PID visit the clinic and how frequently some monitoring tests are carried out. These results highlight the urgent need for consensus guidelines on how to monitor lung complications in PID patients.<\/jats:p>","DOI":"10.1111\/cei.13012","type":"journal-article","created":{"date-parts":[[2017,7,14]],"date-time":"2017-07-14T15:02:36Z","timestamp":1500044556000},"page":"226-234","source":"Crossref","is-referenced-by-count":27,"title":["Screening protocols to monitor respiratory status in primary immunodeficiency disease: findings from a European survey and subclinical infection working group"],"prefix":"10.1093","volume":"190","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7394-6804","authenticated-orcid":false,"given":"S","family":"Jolles","sequence":"first","affiliation":[{"name":"Immunodeficiency Centre for Wales, University Hospital of Wales, Cardiff, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S","family":"S\u00e1nchez-Ram\u00f3n","sequence":"additional","affiliation":[{"name":"Department of Immunology and IdISSC, Hospital Cl\u00ednico San Carlos, Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"I","family":"Quinti","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine, Sapienza University of Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P","family":"Soler-Palac\u00edn","sequence":"additional","affiliation":[{"name":"Pediatric Infectious Diseases and Immunodeficiencies Unit, Jeffrey Modell Diagnostic and Research Centre, Hospital Universitari Vall d'Hebron, Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C","family":"Agostini","sequence":"additional","affiliation":[{"name":"Department of Medicine (DIMED), Clinical Immunology Unit, University of Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B","family":"Florkin","sequence":"additional","affiliation":[{"name":"University Department of Pediatrics, CHR Liege, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L-J","family":"Couderc","sequence":"additional","affiliation":[{"name":"Respiratory Diseases Department, H\u00f4pital FOCH, University Versailles-St Quentin, Suresnes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N","family":"Brodszki","sequence":"additional","affiliation":[{"name":"The Children's Hospital, Sk\u00e5ne University Hospital, Lund, Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A","family":"Jones","sequence":"additional","affiliation":[{"name":"Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H","family":"Longhurst","sequence":"additional","affiliation":[{"name":"Department of Immunology, Barts and The London National Health Service Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K","family":"Warnatz","sequence":"additional","affiliation":[{"name":"Center for Chronic Immunodeficiency, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F","family":"Haerynck","sequence":"additional","affiliation":[{"name":"Center for Primary Immunodeficiency, Department of Paediatric Immunology and Pulmonology, Jeffrey Modell Diagnostic and Research Centre, Ghent University Hospital, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A","family":"Matucci","sequence":"additional","affiliation":[{"name":"Department of Biomedicine, Immunoallergology Unit, AOU Craeggi, University of Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E","family":"de Vries","sequence":"additional","affiliation":[{"name":"Jeroen Bosch Academy, Jeroen Bosch Hospital, 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