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Treatment and management of primary antibody deficiency: German interdisciplinary evidence-based consensus guideline.

Authors
  • Hanitsch, Leif1
  • Baumann, Ulrich2
  • Boztug, Kaan3
  • Burkhard-Meier, Ulrike4
  • Fasshauer, Maria5
  • Habermehl, Pirmin6
  • Hauck, Fabian7
  • Klock, Gerd8
  • Liese, Johannes9
  • Meyer, Oliver10
  • Müller, Rainer11
  • Pachlopnik-Schmid, Jana12
  • Pfeiffer-Kascha, Dorothea13
  • Warnatz, Klaus14, 15
  • Wehr, Claudia15, 16
  • Wittke, Kirsten1
  • Niehues, Tim17
  • von Bernuth, Horst18, 19, 20
  • 1 Institute for Medical Immunology, Charité Universitaetsmedizin Berlin, Berlin, Germany. , (Germany)
  • 2 Department of Paediatric Pulmonology, Allergy and Neonatology, Hannover Medical School, Hannover, Germany. , (Germany)
  • 3 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. , (Austria)
  • 4 Praxis für Hämatologie und Onkologie, Mönchengladbach, Germany. , (Germany)
  • 5 ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Academic Teaching Hospital of the University of Leipzig, Leipzig, Germany. , (Germany)
  • 6 Kinderarztpraxis, Mainz-Hechtsheim, Germany. , (Germany)
  • 7 Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-University Munich, Munich, Germany. , (Germany)
  • 8 Technische Universität Darmstadt, Clemens-Schöpf-Institut für Organische Chemie & Biochemie, Darmstadt, Germany. , (Germany)
  • 9 Pediatric Immunology, Department of Pediatrics, University Hospital Würzburg, Würzburg, Germany. , (Germany)
  • 10 Institute of Transfusion Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany. , (Germany)
  • 11 Klinik und Poliklinik für HNO-Heilkunde, Universitätsklinikum Carl Gustav Carus an der Technischen Universität Dresden, Dresden, Germany. , (Germany)
  • 12 Division of Immunology, University Children's Hospital Zurich and University of Zurich, Switzerland. , (Switzerland)
  • 13 AG Atemphysiotherapie, Physiotherapiepraxis Röske, Wuppertal, Germany. , (Germany)
  • 14 Department of Rheumatology and Clinical Immunology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany. , (Germany)
  • 15 Center for Chronic Immunodeficiency, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany. , (Germany)
  • 16 Department of Medicine I, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany. , (Germany)
  • 17 Department of Pediatrics, Helios Klinikum Krefeld, Krefeld, Germany. , (Germany)
  • 18 Department of Immunology, Labor Berlin Charité - Vivantes GmbH, Berlin, Germany. , (Germany)
  • 19 Berlin Center for Regenerative Therapies (BCRT), Charité Universitätsmedizin Berlin, Berlin, Germany. , (Germany)
  • 20 Department of Pediatric Pneumology, Immunology and Intensive Care Medicine, Charité Universitätsmedizin Berlin, Berlin, Germany. , (Germany)
Type
Published Article
Journal
European Journal of Immunology
Publisher
Wiley (John Wiley & Sons)
Publication Date
Oct 01, 2020
Volume
50
Issue
10
Pages
1432–1446
Identifiers
DOI: 10.1002/eji.202048713
PMID: 32845010
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

This evidence-based clinical guideline provides consensus-recommendations for the treatment and care of patients with primary antibody deficiencies (PADs). 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-based 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-infectious manifestations (e.g., management of granulomatous disease, immune cytopenia). PADs are grouped into disease entities with definitive, probable, possible, or unlikely benefit of IgG-replacement therapy. Summary and consensus-recommendations are provided for treatment indication, dosing, routes of administration, and adverse events of IgG-replacement therapy. Special aspects of concomitant impaired T-cell function are highlighted as well as clinical data on selected monogenetic inborn errors of immunity formerly classified into PADs (APDS, CTLA-4-, and LRBA-deficiency). © 2020 The Authors. European Journal of Immunology published by Wiley-VCH GmbH.

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