scholarly journals The Epidemiology and Clinical Manifestations of Autoimmunity in Selective IgA Deficiency

2019 ◽  
Vol 58 (1) ◽  
pp. 107-133 ◽  
Author(s):  
David D. Odineal ◽  
M. Eric Gershwin
Keyword(s):  
Clinical Manifestations ◽  
Iga Deficiency ◽  
Selective Iga Deficiency

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.

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 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.

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.

Reexamining the role of TACI coding variants in common variable immunodeficiency and selective IgA deficiency

2007 ◽  
Vol 39 (4) ◽  
pp. 430-431 ◽  
Author(s):  
Emanuela Castigli ◽  
Stephen Wilson ◽  
Lilit Garibyan ◽  
Rima Rachid ◽  
Francisco Bonilla ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Iga Deficiency ◽  
Selective Iga Deficiency ◽  
Coding Variants ◽  
Common Variable

The Association Between Immunodeficiency and the Development of Autoimmune Disease

1996 ◽  
Vol 10 (1) ◽  
pp. 57-61 ◽  
Author(s):  
J.W. Sleasman
Keyword(s):  
Immune System ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Iga Deficiency ◽  
Selective Iga Deficiency ◽  
Increased Risk ◽  
High Incidence ◽  
Autoimmune Cytopenias ◽  
The Complement System ◽  
Common Variable

There is a paradoxical relationship between immunodeficiency diseases and autoimmunity. While not all individuals with immunodeficiency develop autoimmunity, nor are all individuals with autoimmunity immunodeficient, defects within certain components of the immune system carry a high risk for the development of autoimmune disease. Inherited deficiencies of the complement system have a high incidence of systemic lupus erythematosus (SLE), glomerulonephritis, and vasculitis. Carrier mothers of children with chronic granulomatous disease, an X-linked defect of phagocytosis, often develop discoid lupus. Several antibody deficiencies are associated with autoimmune disease. Autoimmune cytopenias are commonly observed in individuals with selective IgA deficiency and common variable immune deficiency. Polyarticular arthritis can be seen in children with X-linked agammaglobulinemia. Combined cellular and antibody deficiencies, such as Wiskott-Aldrich syndrome, carry an increased risk for juvenile rheumatoid arthritis and autoimmune hemolytic anemia. Several hypothetical mechanisms have been proposed to explain the associations between autoimmunity and immunodeficiency. Immunologic defects may result in a failure to exclude microbial antigens, resulting in chronic immunologic activation and autoimmune symptoms. There may be shared genetic factors, such as common HLA alleles, which predispose an individual to both autoimmunity and immunodeficiency. Defects within one component of the immune system may alter the way a pathogen induces an immune response and lead to an inflammatory response directed at self-antigens. An understanding of the immunologic defects that contribute to the development of autoimmunity will provide an insight into the pathogenesis of the autoimmune process.

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