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2017 ; 8
(ä): 586
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English Wikipedia
Specific Antibody Deficiency: Controversies in Diagnosis and Management
#MMPMID28588580
Perez E
; Bonilla FA
; Orange JS
; Ballow M
Front Immunol
2017[]; 8
(ä): 586
PMID28588580
show ga
Specific antibody deficiency (SAD) is a primary immunodeficiency disease
characterized by normal immunoglobulins (Igs), IgA, IgM, total IgG, and IgG
subclass levels, but with recurrent infection and diminished antibody responses
to polysaccharide antigens following vaccination. There is a lack of consensus
regarding the diagnosis and treatment of SAD, and its clinical significance is
not well understood. Here, we discuss current evidence and challenges regarding
the diagnosis and treatment of SAD. SAD is normally diagnosed by determining
protective titers in response to the 23-valent pneumococcal polysaccharide
vaccine. However, the definition of an adequate response to immunization remains
controversial, including the magnitude of response and number of pneumococcal
serotypes needed to determine a normal response. Confounding these issues,
anti-polysaccharide antibody responses are age- and probably serotype dependent.
Therapeutic strategies and options for patients with SAD are often based on
clinical experience due to the lack of focused studies and absence of a robust
case definition. The mainstay of therapy for patients with SAD is antibiotic
prophylaxis. However, there is no consensus regarding the frequency and severity
of infections warranting antibiotic prophylaxis and no standardized regimens and
no studies of efficacy. Published expert guidelines and opinions have recommended
IgG therapy, which are supported by observations from retrospective studies,
although definitive data are lacking. In summary, there is currently a lack of
evidence regarding the efficacy of therapeutic strategies for patients with SAD.
We believe that it is best to approach each patient as an individual and progress
through diagnostic and therapeutic interventions together with existing practice
guidelines.