Selektif İmmünoglobulin A Eksikliği Olan Çocuklarda Klinik Bulgular, Alerjik Komorbiditeler ve Otoimmün Hastalıklar: Beş Yıllık Tek Merkezli Çalışma

Öz

Selektif immünoglobulin A eksikliği (SIgAD), en sık görülen baskın antikor eksikliği ve en sık karşılaşılan doğuştan bağışıklık sistemi bozukluklarından biridir. Klinik seyri oldukça heterojen olup tekrarlayan enfeksiyonlar, alerjik hastalıklar ve otoimmün hastalıkları içermektedir. Bu çalışmada SIgAD tanılı çocuk hastaların demografik, klinik ve laboratuvar özelliklerinin değerlendirilmesi amaçlandı. Mayıs 2021-Mayıs 2026 tarihleri arasında üçüncü basamak bir merkezde serum immünoglobulin A (IgA) düzeyi ölçülen 15.815 çocuk hastanın kayıtları retrospektif olarak incelendi. Hastalar Avrupa İmmün Yetmezlikler Derneği (ESID) tanı kriterlerine göre değerlendirildi ve tüm kriterleri karşılayan 47 hasta çalışmaya dahil edildi. Hastaların medyan tanı yaşı 8 yıl (çeyrekler arası aralık [IQR]: 6–12 yıl) olup %53,2'si erkekti. Tekrarlayan enfeksiyonlar hastaların %66,0'ında, alerjik hastalıklar %42,6'sında ve otoimmün hastalıklar %29,8'inde saptandı. En sık alerjik hastalık alerjik rinit, en sık otoimmün hastalık ise çölyak hastalığı idi. Alerjik duyarlanma açısından değerlendirilen hastaların %75,0'inde inhalan alerjen duyarlanması saptanmış olup en sık polen duyarlanması görüldü. Tüm hastalarda serum IgG ve IgM düzeyleri normal sınırlarda iken, lenfosit alt grup analizlerinde anlamlı bir anormallik izlenmedi. Bulgularımız, tekrarlayan enfeksiyonların yanı sıra alerjik hastalıklar ve otoimmün hastalıkların da çocukluk çağı SIgAD klinik spektrumunun önemli bileşenleri olduğunu göstermektedir. Bu nedenle SIgAD tanılı çocukların eşlik eden komorbiditeler açısından kapsamlı değerlendirilmesi ve uzun dönem multidisipliner izlenmesi önem taşımaktadır.

Anahtar Kelimeler

• selektif immunoglobulin A eksikliği
• çocuk
• alerjik rinit
• otoimmün hastalıklar
• çölyak

Destekleyen Kurum

Bu çalışma için herhangi bir finansal destek alınmamıştır.

Etik Beyan

Bu çalışma Afyonkarahisar Sağlık Bilimleri Üniversitesi Girişimsel Olmayan Klinik Araştırmalar Etik Kurulu tarafından onaylanmıştır (Karar No: 2026/9, Tarih: 08.05.2026). Çalışma retrospektif tasarıma sahip olduğundan hastalardan ayrıca aydınlatılmış onam alınması gerekli görülmemiştir.

Clinical Features, Allergic Comorbidities, and Autoimmune Diseases in Children with Selective Immunoglobulin A Deficiency: A Five-Year Single-Center Study

Öz

Selective immunoglobulin A deficiency (SIgAD) is the most common predominantly antibody deficiency and one of the most frequently encountered inborn errors of immunity. This study evaluated the demographic, clinical, and laboratory characteristics of children with SIgAD. Medical records of 15,815 children who underwent serum immunoglobulin A (IgA) measurement between May 2021 and May 2026 at a tertiary referral center were retrospectively reviewed. According to the European Society for Immunodeficiencies (ESID) diagnostic criteria, 47 children were included. The median age at diagnosis was 8 years (interquartile range [IQR], 6–12 years), and 53.2% were male. Recurrent infections were identified in 66.0% of patients, while allergic diseases and autoimmune diseases were present in 42.6% and 29.8%, respectively. Allergic rhinitis was the most common allergic disease, whereas celiac disease was the most frequent autoimmune disease. Among the 16 patients who underwent allergy testing, inhalant allergen sensitization was detected in 12 (75.0%), with pollen representing the predominant allergen. Serum IgG and IgM concentrations were normal in all patients, and lymphocyte subset analysis revealed no consistent abnormalities among the evaluated cases. These findings indicate that recurrent infections, allergic diseases, and autoimmune diseases are common manifestations in children with SIgAD. Comprehensive clinical assessment and long-term multidisciplinary follow-up remain important for the recognition and management of associated comorbidities in this tertiary-center cohort.

Anahtar Kelimeler

• selective immunoglobulin A deficiency
• child
• allergic rhinitis
• autoimmune diseases
• celiac disease

Destekleyen Kurum

The authors declared that this study received no financial support

Etik Beyan

The study was approved by the Afyonkarahisar Health Sciences University Non-Interventional Clinical Research Ethics Committee (Decision No:2026/9 Date:08.05.2026).

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