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The guideline group comprised 20 clinical and scientific expert associations of the German, Swiss, and Austrian healthcare system and representatives of patients. Recommendations were based on results of a systematic literature search, data extraction, and evaluation of methodology and study quality in combination with the clinical expertise of the respective representatives. Consensus\u2010based recommendations were determined via nominal group technique. PADs are the largest clinically relevant group of primary immunodeficiencies. Most patients with PADs present with increased susceptibility to infections, however immune dysregulation, autoimmunity, and cancer affect a significant number of patients and may precede infections. This guideline therefore covers interdisciplinary clinical and therapeutic aspects of infectious (e.g., antibiotic prophylaxis, management of bronchiectasis) and non\u2010infectious manifestations (e.g., management of granulomatous disease, immune cytopenia). PADs are grouped into disease entities with definitive, probable, possible, or unlikely benefit of IgG\u2010replacement therapy. Summary and consensus\u2010recommendations are provided for treatment indication, dosing, routes of administration, and adverse events of IgG\u2010replacement therapy. Special aspects of concomitant impaired T\u2010cell function are highlighted as well as clinical data on selected monogenetic inborn errors of immunity formerly classified into PADs (APDS, CTLA\u20104\u2010, and LRBA\u2010deficiency).<\/jats:p>","DOI":"10.1002\/eji.202048713","type":"journal-article","created":{"date-parts":[[2020,8,26]],"date-time":"2020-08-26T22:57:48Z","timestamp":1598482668000},"page":"1432-1446","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":32,"title":["Treatment and management of primary antibody deficiency: German interdisciplinary evidence\u2010based consensus guideline"],"prefix":"10.1002","volume":"50","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8181-0093","authenticated-orcid":false,"given":"Leif","family":"Hanitsch","sequence":"first","affiliation":[{"name":"Institute for Medical Immunology Charit\u00e9 Universitaetsmedizin Berlin  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich","family":"Baumann","sequence":"additional","affiliation":[{"name":"Department of Paediatric Pulmonology Allergy and Neonatology Hannover Medical School  Hannover Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaan","family":"Boztug","sequence":"additional","affiliation":[{"name":"CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases Department of Pediatrics and Adolescent Medicine and St. Anna Kinderspital and Children's Cancer Research Institute Department of Pediatrics Medical University of Vienna  Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrike","family":"Burkhard\u2010Meier","sequence":"additional","affiliation":[{"name":"Praxis f\u00fcr H\u00e4matologie und Onkologie  M\u00f6nchengladbach Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Fasshauer","sequence":"additional","affiliation":[{"name":"ImmunoDeficiencyCenter Leipzig (IDCL) Hospital St. Georg gGmbH Leipzig Academic Teaching Hospital of the University of Leipzig  Leipzig Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pirmin","family":"Habermehl","sequence":"additional","affiliation":[{"name":"Kinderarztpraxis Mainz\u2010Hechtsheim  Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabian","family":"Hauck","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Dr. von Hauner Children's Hospital University Hospital Ludwig\u2010Maximilians\u2010University Munich  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerd","family":"Klock","sequence":"additional","affiliation":[{"name":"Technische Universit\u00e4t Darmstadt Clemens\u2010Sch\u00f6pf\u2010Institut f\u00fcr Organische Chemie &amp; 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