Deficiency of IgG4 in children: Association of isolated IgG4 deficiency with recurrent respiratory tract infection

Moss, Richard B.; Carmack, Mary Ann; Esrig, Syliva

doi:10.1016/s0022-3476(05)80590-6

Cited by 52 publications

(28 citation statements)

References 21 publications

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“…The diagnosis of IgG4 was supported by tests of in vitro Ig synthesis stimulated with either PWM or IL-4. Isolated IgG4 deficiency, not a rare disease, leads to recurrent infections, particularly of the respiratory tract [1,12]. In the majority of cases, however, IgG4 deficiency is associated with another Ig isotype deficiency, i.e., IgG2-IgG4 or IgG2-IgG4-IgA deficiency [12].…”

Section: Discussionmentioning

confidence: 99%

IgG4 deficiency with Rothmund-Thomson syndrome: A case report

et al. 1993

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Section: Discussionmentioning

confidence: 99%

IgG4 deficiency with Rothmund-Thomson syndrome: A case report

et al. 1993

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“…It is unlikely that IgDd contributes since there is no clear function identified for serum IgD. IgAd is associated with increased frequency of sinopulmonary infections [31] in some patients and IgG4d has been reported to be associated with increased susceptibility to bacterial infections as well [32,33]. A previous study of Ig levels in T1D patients revealed no strong link between low levels and infections in T1D patients [34].…”

Section: Why Would Two Supposedly Genetically Identical Individuals Dmentioning

confidence: 99%

Incomplete penetrance of susceptibility genes for MHC-determined immunoglobulin deficiencies in monozygotic twins discordant for type 1 diabetes

Alper

Husain

Larsen

et al. 2006

Journal of Autoimmunity

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Incomplete intrinsic penetrance is the failure of some genetically susceptible individuals (e.g., monozygotic twins of those who have a trait) to exhibit that trait. For the first time, we examine penetrance of susceptibility genes for multiple MHC gene-determined traits in the same subjects. Serum levels of IgA, IgD, IgG3, but not IgG4, in 50 pairs of monozygotic twins discordant for type 1 diabetes (T1D) correlated more closely in the twins than in random paired controls. The frequencies of subjects deficient in IgA (6%), IgD (33%) and IgG4 (12%), but not in IgG3, were higher in the twins than in controls. We postulate that this was because the MHC haplotypes (and possible non-MHC genes) that predispose to T1D also carry susceptibility genes for certain immunoglobulin deficiencies. Immunoglobulin deficiencies were not associated with T1D. Pairwise concordance for the deficiencies in the twins was 50% for IgA, 57% for IgD and 50% for IgG4. There were no significant associations among the specific immunoglobulin deficiencies except that all IgA-deficient subjects had IgD deficiency. Thus, intrinsic penetrance is a random process independently affecting different MHC susceptibility genes. Because multiple different external triggers would be required to explain the results, differential environmental determinants appear unlikely.

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“…However, sera from diseased sub-jects reacted with the RgpA44/Kgp44 adhesin only and did not recognize EP1. Unlike other subclass antibodies, IgG4 (equivalent to IgG1 and IgG2a in rats) is considered to have a noninflammatory effector function profile, as it does not bind or activate complement, and a suggested biological function for IgG4 is a protective or defensive role in mucosal immunity (18,31). Furthermore, IgG4 has been shown to bind Fc receptor Fc␥R1, inducing phagocytosis and production of interleukin-10, which reverses the proinflammatory response of macrophages to bacteria or bacterial products such as LPS (10,12,53).…”

Section: Vol 70 2002 Immunization Prevents P Gingivalis-induced Bomentioning

confidence: 99%

Immunization with the RgpA-Kgp Proteinase-Adhesin Complexes ofPorphyromonas gingivalisProtects against Periodontal Bone Loss in the Rat Periodontitis Model

et al. 2002

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A major virulence factor of Porphyromonas gingivalis is the extracellular noncovalently associated complexes of Arg-X-and Lys-X-specific cysteine proteinases and adhesins designated the RgpA-Kgp complexes. In this study we investigated the ability of RgpA-Kgp as an immunogen to protect against P. gingivalis-induced periodontal bone loss in the rat. Specific-pathogen-free Sprague-Dawley rats were immunized with either formalin-killed whole P. gingivalis ATCC 33277 cells with incomplete Freund's adjuvant, RgpA-Kgp with incomplete Freund's adjuvant, or incomplete Freund's adjuvant alone. The animals were then challenged by oral inoculation with live P. gingivalis ATCC 33277 cells. Marked periodontal bone loss was observed in animals immunized with incomplete Freund's adjuvant alone; this bone loss was significantly (P < 0.05) greater than that detected in animals immunized with formalin-killed whole cells or RgpA-Kgp or in unchallenged animals. There was no significant difference in periodontal bone loss between animals immunized with formalin-killed whole cells and those immunized with RgpA-Kgp. The bone loss in these animals was also not significantly different from that in unchallenged animals. DNA probe analysis of subgingival plaque samples showed that 100% of the animals immunized with incomplete Freund's adjuvant alone and challenged with P. gingivalis ATCC 33277 were positive for the bacterium. However, P. gingivalis ATCC 33277 could not be detected in subgingival plaque samples from animals immunized with formalin-killed whole cells or with RgpA-Kgp. Immunization with formalin-killed whole cells or RgpA-Kgp induced a high-titer serum immunoglobulin G2a response. Western blot analysis of RgpA-Kgp using pooled protective antisera taken from rats immunized with RgpA-Kgp revealed immunodominant bands at 44, 39, and 27 kDa. In conclusion, immunization with RgpAKgp restricted colonization by P. gingivalis and periodontal bone loss in the rat.

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Deficiency of IgG4 in children: Association of isolated IgG4 deficiency with recurrent respiratory tract infection

Cited by 52 publications

References 21 publications

IgG4 deficiency with Rothmund-Thomson syndrome: A case report

IgG4 deficiency with Rothmund-Thomson syndrome: A case report

Incomplete penetrance of susceptibility genes for MHC-determined immunoglobulin deficiencies in monozygotic twins discordant for type 1 diabetes

Immunization with the RgpA-Kgp Proteinase-Adhesin Complexes ofPorphyromonas gingivalisProtects against Periodontal Bone Loss in the Rat Periodontitis Model

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