Clinical Manifestations in Selective IgA Deficiency in Childhood; A Follow-up Report

1991 ◽  
Vol 80 (8-9) ◽  
pp. 798-804 ◽  
Author(s):  
P. C. J. DE LAAT ◽  
C. M. R. WEEMAES ◽  
R. GONERA ◽  
P. J. J. VAN MUNSTER ◽  
J. A. J. M. BAKKEREN ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Iga Deficiency ◽  
Selective Iga Deficiency

scholarly journals Immunoglobulin Levels during Follow-up of Children with Selective IgA Deficiency

1992 ◽  
Vol 35 (6) ◽  
pp. 719-725 ◽  
Author(s):  
P. C. J. LAAT ◽  
C. M. R. WEEMAES ◽  
J. A. J. M. BAKKEREN
Keyword(s):  
Iga Deficiency ◽  
Selective Iga Deficiency ◽  
Immunoglobulin Levels

scholarly journals Prophylaxis of Upper Airway Infections in a Patient with Partial IgA Deficiency: Concurrent Use of Sublingual Immunotherapy with Inactivated Whole-Cell Bacterial Extract and Der p1

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Paula F. Aarestrup ◽  
Matheus F. Aarestrup ◽  
Beatriz J. V. Aarestrup ◽  
Fernando M. Aarestrup
Keyword(s):  
Sublingual Immunotherapy ◽  
Respiratory Infections ◽  
Clinical Manifestations ◽  
Iga Deficiency ◽  
Whole Cell ◽  
Bacterial Extract ◽  
Selective Iga Deficiency ◽  
Concurrent Use ◽  
Airway Infections ◽  
Der P1

Selective IgA deficiency is the most common type of primary immunodeficiency, but there is not yet a specific effective treatment. The most prevalent clinical manifestations are infectious diseases of the respiratory system. We report herein the case of an 11-year-old female with selective IgA deficiency and recurring episodes of respiratory infections associated with rhinitis and asthma. We evaluated the efficacy of sublingual immunotherapy combined with inactivated whole-cell bacterial extract and Der p1-specific immunotherapy. After 18 months of clinical follow-up, we observed a significant reduction in the number of episodes of respiratory infections associated with control of atopic diseases. We also observed a 3-fold increase in serum IgA levels compared to treatment initiation. This case demonstrates the potential utility of the concurrent use of sublingual immunotherapy with inactivated whole-cell bacterial extract and Der p1 for successful control of allergy and infection in partial selective IgA deficiency.

Influence of the 2012 European Guidelines in Diagnosis and Follow-up of Coeliac Children with Selective IgA Deficiency

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Raquel Vecino ◽  
Carmen Miranda ◽  
Gloria Rodrigo ◽  
Ruth García Romero ◽  
Maria Luz Cilleruelo ◽  
...  
Keyword(s):  
Iga Deficiency ◽  
Selective Iga Deficiency ◽  
European Guidelines

scholarly journals Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

Medicina ◽  
2022 ◽  
Vol 58 (1) ◽  
pp. 129
Author(s):  
Bianca Laura Cinicola ◽  
Federica Pulvirenti ◽  
Martina Capponi ◽  
Marta Bonetti ◽  
Giulia Brindisi ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Atopic Disease ◽  
Iga Deficiency ◽  
Normal Serum ◽  
Secretory Iga ◽  
Primary Immune Deficiency ◽  
Recurrent Infections ◽  
Selective Iga Deficiency ◽  
The Relationship ◽  
Allergic Disorders

Selective IgA deficiency (SIgAD) is the most common human primary immune deficiency (PID). It is classified as a humoral PID characterized by isolated deficiency of IgA (less than 7 mg/dL but normal serum IgG and IgM) in subjects greater than 4 years of age. Intrinsic defects in the maturation of B cells and a perturbation of Th cells and/or cytokine signals have been hypothesized to contribute to SIgAD pathogenesis. The genetic basis of IgA deficiency remains to be clarified. Patients with SIgAD can be either asymptomatic or symptomatic with clinical manifestations including allergy, autoimmunity and recurrent infections mainly of the respiratory and gastrointestinal tract. Studies analyzing allergy on SIgAD patients showed prevalence up to 84%, supporting in most cases the relationship between sIgAD and allergic disease. However, the prevalence of allergic disorders may be influenced by various factors. Thus, the question of whether allergy is more common in SIgAD patients compared to healthy subjects remains to be defined. Different hypotheses support an increased susceptibility to allergy in subjects with SIgAD. Recurrent infections due to loss of secretory IgA might have a role in the pathogenesis of allergy, and vice versa. Perturbation of microbiota also plays a role. The aim of this review is to examine the association between SIgAD and atopic disease and to update readers on advances over time at this important interface between allergy and SIgAD.

Low Prevalence of Selective IgA Deficiency in Infected Children Born to HIV-Seropositive Mothers: An In Vivo Model for Speculation on Selective IgA Deficiency Pathogenesis

2008 ◽  
Vol 21 (4) ◽  
pp. 1035-1039
Author(s):  
E. Chiappini ◽  
L. Galli ◽  
P.A. Tovo ◽  
C. Gabiano ◽  
C. Lisi ◽  
...  
Keyword(s):  
Hiv Infection ◽  
Common Variable Immunodeficiency ◽  
Serum Levels ◽  
Iga Deficiency ◽  
In Vivo Model ◽  
Selective Iga Deficiency ◽  
Perinatal Hiv ◽  
Common Variable

Anecdotal reports of restored immunoglobulin production in individuals with common variable immunodeficiency after acquiring HIV infection suggest that perturbation of the immune system occurring during HIV infection may force some underlying functional defects. These findings raise intriguing questions about the pathogenesis of common variable immunodeficiency. No study has investigated the possible influence of HIV infection on the development of selective IgA deficiency, a primary immunologic defect genetically related to common variable immunodeficiency. IgA serum levels were evaluated in a large cohort of children born to HIV-infected mothers from 1985 to 2006. To avoid differences possibly due to different follow-up durations we considered only infected and non-infected children aged over 4 years at last-follow-up. The study included 1,157 non-infected children and 964 infected children, aged ≥ 4 years at last-follow-up and with availability of two or more serum IgA determinations over six months of age. No child displayed immunoglobulin values compatible with diagnosis of common variable immunodeficiency. However, 0/964 infected children vs. 5/1157 non-infected children had selective IgA deficiency (P=0.048). It may be speculated that several immunological alterations, occurring simultaneously in perinatal HIV infection, surpass the functional defect exhibited in some children with selective IgA deficiency. Our data may shed light on the pathogenesis of selective IgA deficiency.

scholarly journals DOP79 Primary hypogammaglobulinemia with IBD-like features: An ECCO CONFER Multicenter Case Series

2021 ◽  
Vol 15 (Supplement_1) ◽  
pp. S111-S111
Author(s):  
A Albshesh ◽  
P Eder ◽  
D G Ribaldone ◽  
B Oldenburg ◽  
N K De Boer ◽  
...  
Keyword(s):  
Case Reports ◽  
Case Series ◽  
Iga Deficiency ◽  
Clinical Feature ◽  
Aminosalicylic Acid ◽  
Igg Subclass Deficiency ◽  
Selective Iga Deficiency ◽  
Appropriate Treatment ◽  
One Year

Abstract Background The most commonly recognized clinical feature of hypogammaglobulinemia is recurrent infections with high prevalence of gastrointestinal manifestations. In some cases, clinical and endoscopic features are indistinguishable from those of inflammatory bowel disease (IBD). Methods This was a multicenter case series performed as a part of the Collaborative Network of Exceptionally Rare case reports (CONFER) project. Results This report includes 25 patients with primary hypogammaglobinemia and IBD-like features [20 males and 5 females, mean age 50 years (±21.7 SD)]. Crohn’s disease-like features were noted in 22 patients, three patients had ulcerative colitis-like features. The diagnosis of hypogammaglobulinemia preceded IBD-like features diagnosis in 20 patients (mean of 7.7 years prior, range 0.3–35 years), and followed IBD-like features appearance in 5 cases (mean of one year after, 0.4- 9.1 years). Hypogammaglobinemia etiologies were common variable immunodeficiency (72%), Agammaglobulinemia (8%), selective IgA-deficiency (8%), Goods syndrome (8%), IgG subclass deficiency with IgA deficiency (4%). In addition to antibiotics and intravenous immunoglobulin (IVIG) as a treatment for hypogammaglobinemia, fifteen patients received IBD treatment during the follow-up period, of whom two were on 5-aminosalicylic acid, five on corticosteroids, three on immunomodulatory, four on anti-tumor necrosis factor, and one on vedolizumab. By the end of the follow-up [35.5 months (Interquartile range 18–75)], 20 of 25 (80%) patients were in clinical remission. Conclusion This case series illustrates a strong male and CD-like features predilection. The diagnosis of IBD-like features mainly occurs after that of hypogammaglobulinemia, the majority of cases successfully recovered after appropriate treatment.

scholarly journals Innate Mechanisms in Selective IgA Deficiency

2021 ◽  
Vol 12 ◽  
Author(s):  
Jingyan Zhang ◽  
Dèlenn van Oostrom ◽  
JianXi Li ◽  
Huub F. J. Savelkoul
Keyword(s):  
Clinical Symptoms ◽  
Clinical Manifestations ◽  
Specific Treatment ◽  
Serum Levels ◽  
Iga Deficiency ◽  
Normal Serum ◽  
Immune Defense ◽  
Selective Iga Deficiency ◽  
Young Infants ◽  
Novel Therapeutic Strategies

Selective IgA deficiency (SIgAD), characterized by a serum IgA level below 0.07 mg/ml, while displaying normal serum levels of IgM and IgG antibodies, is the most frequently occurring primary immunodeficiency that reveals itself after the first four years after birth. These individuals with SIgAD are for the majority healthy and even when they are identified they are usually not investigated further or followed up. However, recent studies show that newborns and young infants already display clinical manifestations of this condition due to aberrancies in their immune defense. Interestingly, there is a huge heterogeneity in the clinical symptoms of the affected individuals. More than 50% of the affected individuals do not have clinical symptoms, while the individuals that do show clinical symptoms can suffer from mild to severe infections, allergies and autoimmune diseases. However, the reason for this heterogeneity in the manifestation of clinical symptoms of the individuals with SIgAD is unknown. Therefore, this review focusses on the characteristics of innate immune system driving T-cell independent IgA production and providing a mechanism underlying the development of SIgAD. Thereby, we focus on some important genes, including TNFRSF13B (encoding TACI), associated with SIgAD and the involvement of epigenetics, which will cover the methylation degree of TNFRSF13B, and environmental factors, including the gut microbiota, in the development of SIgAD. Currently, no specific treatment for SIgAD exists and novel therapeutic strategies could be developed based on the discussed information.

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