الفهرس 
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Abstract
C
hronic Spontaneous Urticaria is a chronic inflammatory skin disease characterized by the appearance of wheals, angioedema or both. It’s a mast cell-driven disease triggered by infections, food, drug intolerance, activation of the coagulation cascade, genetic disposition or autoimmunity, with the latter being the most frequent underlying cause with two autoimmune endotypes, type I autoimmune chronic spontaneous urticaria driven by Immunoglobulin (Ig) E antibodies directed against autoantigens, and type IIb autoimmune chronic spontaneous urticaria MCs are activated by IgG autoantibodies directed against IgE or its receptor FcεRI. Type I is more common and the two endotypes may overlap in some patients.
Selective immunoglobulin A-deficiency (SIgAD) is the most common primary immunodeficiency. It has been defined Male or female patient greater than 4 years of age who has a serum IgA of less than 7 mg/dl (0.07 g/L) but normal serum IgG and IgM, in whom other causes of hypogammaglobulinemia have been excluded as per European Society for Immunodeficiencies (2019).
Severe IgA deficiency refers to serum levels below 7 mg/dL.
Patients with higher serum IgA level, but two standard deviations lower than the normal value, are classified as partial IgA deficiency (pIgAD).
SIgAD patients are classified into five clusters based on clinical phenotype: Asymptomatic, Minor infection, Allergy, Autoimmune, Severe.
It is widely accepted that SIgAD is associated with allergy and various atopic manifestations including allergic rhino-conjunctivitis, asthma, urticaria, food allergy and atopic dermatitis (AD). However, there is still controversy on the true prevalence of allergy and its manifestations in SIgAD, arguing whether it can be considered a comorbidity or a consequence of the disease, It is also worth mentioning that in many cases allergy is the first symptom of SIgAD, and, in some cases, it is the only symptom of disease, it is also reported that up to 25% of SIgAD patients are diagnosed during an allergology clinical assessment, furthermore, patients can develop new allergic manifestations during follow-up suggesting that periodic allergic assessment in SIgAD patients is warranted, especially when a positive family history for allergies is present. It has been estimated that allergies can be the first manifestation of SIgAD in up to 40% of cases. Therefore, the suspicion of SIgAD should heighten not only in patients with recurrent infections but also in those with atopic manifestations.
Our study is an observational cross-sectional study conducted on 104 patients attending Allergy Clinic at Ain Shams University Hospitals aged between 18-65 years of both genders, all patients were subjected to full detailed history including urticaria activity score 7 (UAS7), full clinical examination, CBC with Differential, Total IgE, IgA, IgG and IgM only in IgA deficient patients, ESR, CRP, AST/ALT, Creatinine, Uric acid, T.Bill/D.Bill. Our study aimed at detecting the prevalence of SIgAD in CSU patients through the measurement of serum IgA level in CSU patients and serum IgM & IgG only in patients with IgA deficiency, our results showed that 5 patients out of 104 cases were found to have selective IgA deficiency.
Of whom 4 patients had partial IgA deficiency and only 1 patient had severe IgA deficiency.
Gender and age played major confounding factors in our study. Especially that most of our patients were most predominantly females.
Our findings implicate that especially the combination of serum IgA and IgE levels is of relevance in the diagnostic workup of CSU patients. Notably, patients with lower IgA levels present different disease phenotypes such as recurrent respiratory tract infections, allergies, autoimmune diseases and gastrointestinal disorders and other complications. Such pathology urges patients with low IgA levels to seek early medical advice.
CONCLUSION
O
ur study focused on determining the prevalence of selective immunoglobulin A-deficiency among patients diagnosed with chronic Spontaneous Urticaria. Consistently, our results detected 5 patients out of 104 cases were found to have selective IgA deficiency with a percentage of (4.8%), 4 of whom had partial IgA deficiency and only 1 patient had severe IgA deficiency.
Our research results strongly support that IgA and IgE level assessment is essential to further diagnose subtypes of CSU. Such assessment will potentiate the early formulation of appropriate therapy to each patient.
RECOMMENDATIONS
• More multicentric studies are needed with larger sample size and matched age and gender.
• Further work on impact of age and gender on IgA and IgE levels is needed. Particularly, in determination of X-linked influence on SIgA levels.
• Family studies in patients with SIgAD may be helpful to test for genetic factors.
• The assessment of serum IgA and IgE levels is cost-effective and easy-to-perform and further work on the role of both immunoglobulins as diagnostic biomarkers is required.