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

Hanitsch, Leif ; Baumann, Ulrich ; Boztug, Kaan ; Burkhard‐Meier, Ulrike ; Fasshauer, Maria ; Habermehl, Pirmin ; Hauck, Fabian ; Klock, Gerd ; Liese, Johannes ; Meyer, Oliver ; Müller, Rainer ; Pachlopnik‐Schmid, Jana ; Pfeiffer‐Kascha, Dorothea ; Warnatz, Klaus ; Wehr, Claudia ; Wittke, Kirsten ; Niehues, Tim ; Bernuth, Horst von (2024)
Treatment and management of primary antibody deficiency: German interdisciplinary evidence‐based consensus guideline.
In: European Journal of Immunology : Basic, Clinical, Translational, 2020, 50 (10)
doi: 10.26083/tuprints-00017813
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Item Type: Article
Type of entry: Secondary publication
Title: Treatment and management of primary antibody deficiency: German interdisciplinary evidence‐based consensus guideline
Language: English
Date: 12 February 2024
Place of Publication: Darmstadt
Year of primary publication: 2020
Place of primary publication: Weinheim
Publisher: Wiley-VCH
Journal or Publication Title: European Journal of Immunology : Basic, Clinical, Translational
Volume of the journal: 50
Issue Number: 10
DOI: 10.26083/tuprints-00017813
Corresponding Links:
Origin: Secondary publication DeepGreen
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).

Uncontrolled Keywords: autoimmunity, CVID, hypogammaglobulinemia, immunoglobulins, primary antibody deficiency
Status: Publisher's Version
URN: urn:nbn:de:tuda-tuprints-178138
Classification DDC: 600 Technology, medicine, applied sciences > 610 Medicine and health
Divisions: 07 Department of Chemistry > Clemens-Schöpf-Institut
Date Deposited: 12 Feb 2024 13:57
Last Modified: 03 Jul 2024 09:52
SWORD Depositor: Deep Green
URI: https://tuprints.ulb.tu-darmstadt.de/id/eprint/17813
PPN: 519319591
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