Abstract

Selective IgA deficiency (sIgAD) is the most common primary immunodeficiency disease (PID), with an estimated occurrence from about 1:3000 to even 1:150, depending on population. sIgAD is diagnosed in adults and children after the 4th year of age, with immunoglobulin A level below 0.07 g/L and normal levels of IgM and IgG. Usually, the disease remains undiagnosed throughout the patient’s life, due to its frequent asymptomatic course. If symptomatic, sIgAD is connected to more frequent viral and bacterial infections of upper respiratory, urinary, and gastrointestinal tracts, as well as autoimmune and allergic diseases. Interestingly, it may also be associated with other PIDs, such as IgG subclasses deficiency or specific antibodies deficiency. Rarely sIgAD can evolve to common variable immunodeficiency disease (CVID). It should also be remembered that IgA deficiency may occur in the course of other conditions or result from their treatment. It is hypothesized that allergic diseases (e.g., eczema, rhinitis, asthma) are more common in patients diagnosed with this particular PID. Selective IgA deficiency, although usually mildly symptomatic, can be difficult for clinicians. The aim of the study is to summarize the connection between selective IgA deficiency and atopic diseases.

Highlights

  • Primary immunodeficiency diseases (PIDs) are a heterogeneous group of congenital diseases with various clinical manifestations and different models of inheritance (X-linked, AR, polygenetic), caused by the impairment or loss of at least one function of the immune system

  • Level of these immunoglobulins may be influenced by drugs that are often used in everyday practice—non-steroid anti-inflammatory drugs (NSAIDs), angiotensin convertase enzyme inhibitors (ACEI), several anti-epileptic drugs, or drugs used in rheumatology

  • A similar observation has been done in Ankara more recently, in 2017, where 45.7% of the patients diagnosed with Selective IgA deficiency (sIgAD) presented one of the following: asthma, rhinitis, eczema, atopic dermatitis, and interestingly the prevalence of allergy in a close family of this patients rose up to 43.2% [101]

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Summary

Introduction

Primary immunodeficiency diseases (PIDs) are a heterogeneous group of congenital diseases with various clinical manifestations and different models of inheritance (X-linked, AR, polygenetic), caused by the impairment or loss of at least one function of the immune system. They weaken the body’s defenses, increasing the frequency of infections as well as the risk of autoimmune and proliferative diseases, including cancers [1]. According to the recent work of Bahrami et al the mean diagnostic delay among primary immunodeficient patients was 2.05 ± 1.7 years [5] This delay is especially prominent in antibody deficiency defects and requires special attention. The suspicion of sIgAD should raise patients with recurrent infections and with other clinical manifestations

IgA—Structure
IgA—Function
Pathogenesis of IgA Deficiency
Clinical Presentation of sIgAD
Asymptomatic
Minor Infections
Autoimmunity
Allergy
Food Allergy
Asthma
Atopic Dermatitis
Allergic Rhinitis and Conjunctivitis
Severe
Complications
IgA Deficiency and CVID
Transfusion Selective IgA Deficiency
Malignancy
Atopic Diseases in Other PIDs
Findings
Conclusions
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