dc.contributor.author
Hanitsch, Leif
dc.contributor.author
Baumann, Ulrich
dc.contributor.author
Boztug, Kaan
dc.contributor.author
Burkhard‐Meier, Ulrike
dc.contributor.author
Fasshauer, Maria
dc.contributor.author
Habermehl, Pirmin
dc.contributor.author
Hauck, Fabian
dc.contributor.author
Klock, Gerd
dc.contributor.author
Liese, Johannes
dc.contributor.author
Meyer, Oliver
dc.contributor.author
Müller, Rainer
dc.contributor.author
Pachlopnik‐Schmid, Jana
dc.contributor.author
Pfeiffer‐Kascha, Dorothea
dc.contributor.author
Warnatz, Klaus
dc.contributor.author
Wehr, Claudia
dc.contributor.author
Wittke, Kirsten
dc.contributor.author
Niehues, Tim
dc.contributor.author
Bernuth, Horst von
dc.date.accessioned
2022-02-11T13:11:54Z
dc.date.available
2022-02-11T13:11:54Z
dc.identifier.uri
https://refubium.fu-berlin.de/handle/fub188/33970
dc.identifier.uri
http://dx.doi.org/10.17169/refubium-33689
dc.description.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).
en
dc.rights.uri
https://creativecommons.org/licenses/by/4.0/
dc.subject
autoimmunity
en
dc.subject
hypogammaglobulinemia
en
dc.subject
immunoglobulins
en
dc.subject
primary antibody deficiency
en
dc.subject.ddc
600 Technik, Medizin, angewandte Wissenschaften::610 Medizin und Gesundheit::610 Medizin und Gesundheit
dc.title
Treatment and management of primary antibody deficiency: German interdisciplinary evidence‐based consensus guideline
dc.type
Wissenschaftlicher Artikel
dcterms.bibliographicCitation.doi
10.1002/eji.202048713
dcterms.bibliographicCitation.journaltitle
European Journal of Immunology
dcterms.bibliographicCitation.number
10
dcterms.bibliographicCitation.originalpublishername
Wiley
dcterms.bibliographicCitation.pagestart
1432
dcterms.bibliographicCitation.pageend
1446
dcterms.bibliographicCitation.volume
50
refubium.affiliation
Charité - Universitätsmedizin Berlin
refubium.funding
DEAL Wiley
refubium.resourceType.isindependentpub
no
dcterms.accessRights.openaire
open access
dcterms.bibliographicCitation.pmid
32845010
dcterms.isPartOf.issn
0014-2980
dcterms.isPartOf.eissn
1521-4141