Abstract
Background: Specific Antibody Deficiency (SAD) is a primary immunodeficiency disease (PID) characterized by the occurrence of recurrent infections and inadequate antibody response to polysaccharide new antigens.
Objective: This study aims to determine the titer of specific antibodies against unconjugated 23-valent pneumococcal polysaccharide vaccine (PPSV-23), the presence of SAD, and its association with clinical and laboratory findings in Ataxia-telangiectasia (A-T) and selective immunoglobulin A deficiency (SIgAD) patients.
Methods: 32 A-T patients and 43 SIgAD patients were included in this cross-sectional study. Samples of the patients were obtained before and three weeks after vaccination with PPSV-23. Specific immunoglobulin G (IgG) directed towards pneumococcal capsular antigen and specific antibodies against whole pneumococcal antigens was measured.
Results: Comparison of the response to vaccination revealed that 81.3% of A-T patients and 18.6% of the SIgAD patients had an inadequate response to PPSV-23 (p<0.001). The prevalence of recurrent infection (p=0.034) and pneumonia (p=0.003) in SIgAD patients was significantly higher in non-responders than responders. Likewise, the number of marginal zone B cells (p=0.037), transitional B cells (p=0.019), plasmablasts (p=0.019), CD8+ naïve T cells (p=0.036), and percentage of CD8+ T cells (p=0.047), switched memory B cells (SMB) (p=0.026) and immunoglobulin M (IgM) memory B cells (p=0.022) in SIgAD patients were significantly lower in non-responder group than responder group. In contrast, the percentage of CD4 T+ cells in A-T patients was lower in the non-responder group than responders (p=0.035).
Conclusion: SAD is more frequent in A-T patients than SIgAD patients. The role of SMB and T cells should not be underestimated in SAD.
Keywords: Primary immunodeficiency, inborn errors of immunity, Ataxia telangiectasia, selective IgA deficiency, specific antibody deficiency, 23-valent pneumococcal polysaccharide vaccine, PPSV-23.
Graphical Abstract
[http://dx.doi.org/10.18176/jiaci.0388] [PMID: 30741636]
[http://dx.doi.org/10.1007/s10875-008-9229-9] [PMID: 18683032]
[http://dx.doi.org/10.3389/fimmu.2019.00986] [PMID: 31118939]
[http://dx.doi.org/10.1016/j.clim.2006.05.003] [PMID: 16782407]
[http://dx.doi.org/10.1016/j.iac.2015.07.003] [PMID: 26454312]
[http://dx.doi.org/10.1016/j.rchipe.2016.08.006] [PMID: 27614984]
[http://dx.doi.org/10.1177/2152656719900338] [PMID: 32030313]
[http://dx.doi.org/10.1128/CMR.00001-09] [PMID: 19597006]
[http://dx.doi.org/10.1007/s10875-020-00758-x] [PMID: 32048120]
[http://dx.doi.org/10.1007/s10875-019-00737-x] [PMID: 31953710]
[http://dx.doi.org/10.1016/j.jaut.2019.01.011] [PMID: 30795880]
[http://dx.doi.org/10.2174/1871530319666190828125316] [PMID: 31456526]
[http://dx.doi.org/10.1111/sji.12499] [PMID: 27763681]
[http://dx.doi.org/10.1586/1744666X.2015.1081565] [PMID: 26306496]
[http://dx.doi.org/10.1007/s10875-009-9357-x] [PMID: 20101521]
[http://dx.doi.org/10.1007/s12016-019-08756-7] [PMID: 31267472]
[http://dx.doi.org/10.1006/clim.1999.4799] [PMID: 10600329]
[http://dx.doi.org/10.1016/j.clim.2017.01.009] [PMID: 28126470]
[http://dx.doi.org/10.1080/1744666X.2020.1810570] [PMID: 32791865]
[http://dx.doi.org/10.1111/j.1365-2249.2005.02791.x] [PMID: 15932512]
[http://dx.doi.org/10.1186/s13023-016-0543-7] [PMID: 27884168]
[http://dx.doi.org/10.1002/mds.27319] [PMID: 29436738]
[PMID: 31823618]
[http://dx.doi.org/10.1111/j.1365-2249.2008.03684.x] [PMID: 18505428]
[http://dx.doi.org/10.1542/peds.2005-0698SSS]
[http://dx.doi.org/10.22034/igj.2019.85745]
[http://dx.doi.org/10.1111/cei.12262] [PMID: 24387201]
[http://dx.doi.org/10.1023/B:JOCI.0000029109.15355.ba] [PMID: 15163897]
[http://dx.doi.org/10.1111/j.1365-2567.2006.02436.x] [PMID: 17067312]
[http://dx.doi.org/10.1097/01.inf.0000115737.35353.55] [PMID: 15014308]
[http://dx.doi.org/10.1016/j.jaci.2012.07.002] [PMID: 22935624]
[PMID: 2702775]
[http://dx.doi.org/10.1023/A:1020599810261] [PMID: 10535610]
[http://dx.doi.org/10.1590/S0021-75572006000200010] [PMID: 16614768]
[http://dx.doi.org/10.3389/fimmu.2017.01056] [PMID: 28928736]
[http://dx.doi.org/10.2147/CIA.S29675] [PMID: 23152675]
[http://dx.doi.org/10.1016/j.jaip.2019.02.004] [PMID: 30776527]
[http://dx.doi.org/10.1007/s10875-018-0556-1] [PMID: 30302726]
[http://dx.doi.org/10.1177/000456329403100305] [PMID: 8067664]
[http://dx.doi.org/10.1093/clinchem/37.1.20] [PMID: 1988204]
[http://dx.doi.org/10.1046/j.1365-2249.2000.01131.x] [PMID: 10792368]
[http://dx.doi.org/10.1542/peds.37.5.715] [PMID: 4956666]
[http://dx.doi.org/10.1203/00006450-197903000-00010] [PMID: 471573]
[http://dx.doi.org/10.1258/000456302760042164] [PMID: 12117444]
[PMID: 18552408]
[http://dx.doi.org/10.1146/annurev.immunol.15.1.177] [PMID: 9143686]
[PMID: 8521380]
[http://dx.doi.org/10.1111/pai.13020] [PMID: 30685876]
[http://dx.doi.org/10.1080/08820139.2020.1723104] [PMID: 32116070]
[http://dx.doi.org/10.1016/j.clim.2003.12.005] [PMID: 15093556]
[http://dx.doi.org/10.1016/0091-6749(89)90203-0] [PMID: 2677097]
[http://dx.doi.org/10.3389/fimmu.2017.00586] [PMID: 28588580]
[http://dx.doi.org/10.4161/hv.28350] [PMID: 24632599]
[http://dx.doi.org/10.1007/s10875-012-9655-6] [PMID: 22328142]
[http://dx.doi.org/10.1111/j.0105-2896.2004.0112.x] [PMID: 14962196]
[http://dx.doi.org/10.3389/fimmu.2011.00063]
[http://dx.doi.org/10.1159/000323903] [PMID: 21832837]
[http://dx.doi.org/10.1073/pnas.1416276112] [PMID: 25624468]
[http://dx.doi.org/10.1093/infdis/jiv469] [PMID: 26410591]
[http://dx.doi.org/10.4049/jimmunol.175.5.3262] [PMID: 16116217]
[http://dx.doi.org/10.3389/fimmu.2016.00546] [PMID: 27994589]
[http://dx.doi.org/10.1159/000499044] [PMID: 31091523]
[http://dx.doi.org/10.3389/fimmu.2018.00909] [PMID: 29755476]
[http://dx.doi.org/10.1371/journal.pone.0152215] [PMID: 27031098]
[http://dx.doi.org/10.3390/vaccines7010018] [PMID: 30754689]