Hypogammaglobulinemia and rheumatic disease

Lee, Albert H.; Levinson, Arnold I.; Schumacher, H. Ralph

doi:10.1016/0049-0172(93)80073-o

Cited by 105 publications

(56 citation statements)

References 67 publications

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“…The present study also found lower concentrations of serum IgG in patients with IMN in comparison to those with LMN, similar to the study of Kuroki et al [39]. It is known that SLE patients present with reduced levels of IgG and the treatment may normalize this situation [41,42]. Thus, because most patients in the LMN group in the present study had undergone immunosuppressive therapy and were in remission, a relative reduction of serum IgG in the IMN group was expected.…”

Section: Resultssupporting

confidence: 77%

Mannose-Binding Lectin2 Gene Polymorphism and IgG4 in Membranous Nephropathy

Costa¹,

Valente²,

Vajgel³

et al. 2018

Nephron

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Background: Idiopathic membranous nephropathy (IMN) has been linked to the lectin pathway, IgG4 and genetic susceptibility. We investigated the frequency of mannose-binding lectin2 (MBL2) gene polymorphisms and the serum ratio of IgG4 in patients with membranous nephropathy (MN). Methods: Polymorphisms in the exon 1 of the MBL2 gene (codons 52, 54, and 57) and single base polymorphisms at positions -550 (HL) and -221 (XY) in the promoter region were evaluated in 60 patients compared to a control group (CG) of 101 blood donors. It established the frequency of polymorphisms and the serum ratio of IgG4 comparing 2 etiologies of MN: idiopathic (35 patients) and secondary to systemic lupus erythematosus (25 patients). Results: Patients with MN had a 2.54-fold higher probability (95% CI 1.51-4.31) of carrying the O alelle, exon 1 variant, and 11.16-fold higher probability (95% CI 4.77-28.41) of having A/O genotype when compared to CG. The frequency of polymorphisms in the promoter region was similar between the groups. Combined genotypes generally related to the defective production of MBL (YA/O, XA/O and O/O) were more frequent in patients with MN (OR 7.11;), when compared to controls. The median of serum ratio IgG4 was 5% for idiopathic MN and 3% for lupus MN patients (p = 0.016). Conclusions: Our data suggests that MBL2 polymorphisms may be associated with the activation of the lectin pathway by IgG4 subclass antibodies in MN.

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Section: Resultssupporting

confidence: 77%

Mannose-Binding Lectin2 Gene Polymorphism and IgG4 in Membranous Nephropathy

Costa¹,

Valente²,

Vajgel³

et al. 2018

Nephron

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“…Consistent with these opposing roles, immune responses are diminished as a result of CD28 deficiency (229), whereas CTLA4 deficiency results in exaggerated peripheral T-cell activation and clonal expansion (230,231 (60,79). Interestingly, autoantibody production also occurs in children with CD40L deficiency (237), perhaps because CD40L is required to induce CD95/Fas expression on anergic self-reactive B cells and abort their activation (J. C. Rathmell and C.C.G., unpublished data). IL-2 plays a comparable role in rendering T cells sensitive to elimination through CD95-Fas, and defects in this step may explain the paradoxical shift toward excessive T-cell numbers and autoantibody production in IL-2-deficient or IL2-receptor deficient mice (238)(239)(240), or in nonobese diabetic (NOD) mice that make an altered form of IL-2 (241).…”

Section: Examples Of Tolerance By Clone Retuningmentioning

confidence: 66%

Balancing immunity and tolerance: deleting and tuning lymphocyte repertoires.

Goodnow

1996

Proc. Natl. Acad. Sci. U.S.A.

415

267

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Immunological self-tolerance is ensured by eliminating or inhibiting self-reactive lymphocyte clones, creating physical or functional holes in the B- and T-lymphocyte antigen receptor repertoires. The nature and size of these gaps in our immune defenses must be balanced against the necessity of mounting rapid immune responses to an everchanging array of foreign pathogens. To achieve this balance, only a fraction of particularly hazardous self-reactive clones appears to be physically eliminated from the repertoire in a manner that fully prevents their recruitment into an antimicrobial immune response. Many self-reactive cells are retained with a variety of conditional and potentially flexible restraints: (i) their ability to be triggered by antigen is diminished by mechanisms that tune down signaling by their antigen receptors, (ii) their ability to carry out inflammatory effector functions can be inhibited, and (iii) their capacity to migrate and persist is constrained. This balance between tolerance and immunity can be shifted, altering susceptibility to autoimmune disease and to infection by genetic or environmental differences either in the way antigens are presented, in the tuning molecules that adjust triggering set points for lymphocyte responses to antigen, or in the effector molecules that eliminate, retain, or expand particular clones.

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“…Characteristic physical examination findings of XLA are paucity or absence of lymphatic tissue such as tonsils or palpable lymph nodes. Noninfectious complications have been described for XLA, including a higher incidence of juvenile rheumatoid arthritis, aseptic polyarthritis, sensorineural hearing loss, and colorectal cancer (12,20,75). Autoimmune diseases such as Crohn's disease and type I diabetes mellitus have been described for a few patients with XLA, albeit much less frequently than for CVID (15).…”

Section: Primary Antibody Deficiencies Agammaglobulinemiasmentioning

confidence: 99%

Pathogenesis, Diagnosis, and Management of Primary Antibody Deficiencies and Infections

2009

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SUMMARY Primary antibody deficiencies are the most common primary immunodeficiency diseases. They are a heterogeneous group of disorders with various degrees of dysfunctional antibody production resulting from a disruption of B-cell differentiation at different stages. While there has been tremendous recent progress in the understanding of some of these disorders, the etiology remains unknown for the majority of patients. As there is a large spectrum of underlying defects, the age at presentation varies widely, and the clinical manifestations range from an almost complete absence of B cells and serum immunoglobulins to selectively impaired antibody responses to specific antigens with normal total serum immunoglobulin concentrations. However, all of these disorders share an increased susceptibility to infections, affecting predominantly the respiratory tract. A delay of appropriate treatment for some diseases can result in serious complications related to infections, while timely diagnosis and adequate therapy can significantly decrease morbidity and increase life expectancy and quality of life.

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Hypogammaglobulinemia and rheumatic disease

Cited by 105 publications

References 67 publications

<b><i>Mannose-Binding Lectin2</i></b> Gene Polymorphism and IgG4 in Membranous Nephropathy

<b><i>Mannose-Binding Lectin2</i></b> Gene Polymorphism and IgG4 in Membranous Nephropathy

Balancing immunity and tolerance: deleting and tuning lymphocyte repertoires.

Pathogenesis, Diagnosis, and Management of Primary Antibody Deficiencies and Infections

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