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).
Highlights
The International Union of Immunological Societies (IUIS) lists ten groups and 416 monogenetic primary immunodeficiencies (PID)/inborn errors of immunity (IEI) [1] (Table 1)
The present guideline focuses on diseases of IUIS group 3, there are overlaps with other PIDs, in particular with combined immunodeficiency and monogenetic disorders previously categorized as Common variable immunodeficiency disorders (CVID) (e.g., activated PI3KCD syndrome (APDS), CTLA-4-deficiency, and LRBA-deficiency)
This interdisciplinary guideline was developed by mandated members of 20 medical societies from Austria, Germany, and Switzerland including a representative of the German patient organisation for patients with primary immunodeficiencies
Summary
The International Union of Immunological Societies (IUIS) lists ten groups and 416 monogenetic primary immunodeficiencies (PID)/inborn errors of immunity (IEI) [1] (Table 1). Antibody deficiency is of clinical relevance in many other PIDs. The present guideline focuses on diseases of IUIS group 3, there are overlaps with other PIDs, in particular with combined immunodeficiency and monogenetic disorders previously categorized as CVID (e.g., activated PI3KCD syndrome (APDS), CTLA-4-deficiency, and LRBA-deficiency). This guideline does not cover secondary antibody deficiencies. Patients with antibody deficiencies present with an increased susceptibility to infections, affecting predominantly the respiratory and gastrointestinal tract [3]. Autoimmunity, and cancer affect a significant proportion of patients, including for instance immune cytopenia, lymphoproliferation, or granulomatous diseases [6]. This guideline covers aspects of IgG-replacement as well as monitoring and treatment of “non-infectious” manifestations
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