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

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).

The Immune Deficiency and Dysregulation Activity (IDDA2.1 ‘Kaleidoscope’) Score and Other Clinical Measures in Inborn Errors of Immunity

“…1 ) [ 25 ]. With a similar aim, a more complex version of a treatment algorithm for patients with primary antibody deficiencies was published as interdisciplinary consensus- and evidence-based guideline by the Association of Scientific Medical Societies in Germany (AWMF) and updated recently [ 26 , 27 ]. Although the latter instrument includes many similar criteria extracted from the history, clinical status, and laboratory parameters of hypogammaglobulinemic patients like the above-mentioned score, it was not designed as a ‘score’ but as a step-by-step algorithm that includes degrees of evidence for each criterion.…”

Case Report: Whole-Genome Sequencing of Serially Collected Haemophilus influenzae From a Patient With Common Variable Immunodeficiency Reveals Within-Host Evolution of Resistance to Trimethoprim-Sulfamethoxazole and Azithromycin After Prolonged Treatment With These Antibiotics

“…Contemporary CVID guidelines advocate the use of long-term antibiotics in selected patients, although criteria for identifying such patients are lacking ( Bonilla et al., 2016 ; Polverino et al., 2017 ; Hanitsch et al., 2020 ). The use of antimicrobial prophylaxis is nevertheless widespread, administered to 20% - 65% of CVID patients in different temporal and geographic settings, and azithromycin is by far the most frequently prescribed antibiotic ( Kuruvilla and de la Morena, 2013 ; Sperlich et al., 2018 ).…”

Impact of COVID‐19 pandemic on EJI and the extended immunological community

“…The flow cytometry guidelines are still our number one accessed article [10]. In addition, we continue to encourage submission of clinical papers [11] and have recently published a clinical guideline consisting of recommendations for the treatment and care of patients with primary antibody deficiencies [12].…”