Allergic and Autoimmune Features in Children with Selective IgA Deficiency

Abstract

Background: Selective immunoglobulin A deficiency (SIgAD) represents the most frequently encountered form of primary immunodeficiency and is defined by markedly reduced circulating IgA concentrations despite normal IgG and IgM levels. This study aimed to characterize the clinical spectrum and laboratory profile of pediatric patients with SIgAD, with particular emphasis on allergic comorbidities and autoimmune manifestations. Methods: This retrospective observational study was conducted among children aged 4–18 years who met the European Society for Immunodeficiencies (ESID) diagnostic criteria for SIgAD between April 1, 2023, and December 1, 2025. Information on demographic variables, clinical features, and laboratory measurements was collected from electronic health records and analyzed using descriptive statistics. Results: Twenty-six pediatric patients were included (61.5% male). The median ages at symptom onset and diagnosis were 4.50 years (IQR, 3.0–6.0) and 6.97 years (IQR, 5.1–9.5), respectively. Recurrent infections affected 76.9% of patients and represented the predominant clinical manifestation. Allergic diseases were present in 73.1% of patients, most frequently asthma and allergic rhinitis; 42.3% showed aeroallergen sensitization, predominantly to pollens. Among the patients who underwent autoantibody screening, positivity was detected in approximately two-thirds; however, only two were diagnosed with autoimmune diseases (thyroiditis and vitiligo). Antibiotic prophylaxis was initiated in 7.7% of patients due to recurrent infections. Conclusion: Although recurrent infections are the most common presentation in SIgAD, serum IgA evaluation should also be considered in patients with allergic or autoimmune conditions. Regular follow-up is necessary to monitor allergic manifestations, immunologic status, and the potential development of autoimmune diseases.

Keywords

• selective IgA deficiency
• allergic diseases
• autoimmunity
• recurrent infections
• pediatric

Selektif IgA Eksikliği Olan Çocuklarda Alerjik ve Otoimmün Özellikler

Abstract

Amaç: Selektif immünoglobulin A eksikliği (SIgAD), en sık görülen primer immün yetmezlik olup serum IgA düzeylerinin belirgin derecede düşük olması, buna karşın IgG ve IgM düzeylerinin normal olmasıyla tanımlanır. Bu çalışmada, SIgAD tanılı pediatrik hastaların klinik spektrumu ve laboratuvar bulgularının, özellikle alerjik komorbiditeler ve otoimmün bulgulara odaklanılarak değerlendirilmesi amaçlanmıştır. Yöntemler: Bu retrospektif gözlemsel çalışma, 1 Nisan 2023 ile 1 Aralık 2025 tarihleri arasında European Society for Immunodeficiencies (ESID) tanı kriterlerini karşılayan 4–18 yaş arası çocuklarda gerçekleştirildi. Demografik veriler, klinik özellikler ve laboratuvar ölçümleri elektronik tıbbi kayıtlarından elde edilmiş ve tanımlayıcı istatistiksel yöntemler kullanılarak analiz edildi. Bulgular: Çalışmaya 26 pediatrik hasta dahil edildi (%61,5 erkek). Semptom başlangıç yaşı ortancası 4,50 yıl (IQR, 3,0– 6,0), tanı yaşı ortancası ise 6,97 yıl (IQR, 5,1–9,5) olarak saptandı. Hastaların %76,9’unda rekürren enfeksiyonlar görülmüş olup, en sık klinik başvuru nedeniydi. Hastaların %73,1’inde en az bir alerjik hastalık (en sık astım ve alerjik rinit) saptanmış, %42,3’ünde ise en az bir aeroalerjen duyarlılığı gösterilmiş olup polen duyarlılığı ön plandaydı. Otoantikor taraması yapılan hastaların yaklaşık üçte ikisinde pozitiflik saptanmıştı; ancak yalnızca iki hastaya otoimmün hastalık (tiroidit ve vitiligo) tanısı konuldu. Tekrarlayan enfeksiyonlar nedeniyle hastaların %7,7’sine antibiyotik profilaksisi başlandığı tespit edildi. Sonuç: Tekrarlayan enfeksiyonlar SIgAD’de en sık klinik başvuru nedeni olmakla birlikte, alerjik veya otoimmün bulgularla başvuran hastalarda da serum IgA düzeyinin değerlendirilmesi düşünülmelidir. Alerjik bulguların, immünolojik durumun ve olası otoimmün hastalık gelişiminin izlenebilmesi için düzenli takip gereklidir.

Keywords

• selektif IgA eksikliği
• alerjik hastalıklar
• otoimmünite
• tekrarlayan enfeksiyonlar
• çocukluk çağı.

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