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25.05.2019 | Original Article | Ausgabe 5/2019

Journal of Clinical Immunology 5/2019

Clinical and Laboratory Features of 184 Italian Pediatric Patients Affected with Selective IgA Deficiency (SIgAD): a Longitudinal Single-Center Study

Zeitschrift:
Journal of Clinical Immunology > Ausgabe 5/2019
Autoren:
Vassilios Lougaris, Annamaria Sorlini, Chiara Monfredini, Giulia Ingrasciotta, Andrea Caravaggio, Tiziana Lorenzini, Manuela Baronio, Marco Cattalini, Antonella Meini, Laura Ruggeri, Annamaria Salpietro, Alba Pilotta, Livia Grazzani, Elena Prandi, Barbara Felappi, Giulio Gualdi, Antonella Fabiano, Maurizio Fuoti, Alberto Ravelli, Vincenzo Villanacci, Annarosa Soresina, Raffaele Badolato, Alessandro Plebani
Wichtige Hinweise

Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1007/​s10875-019-00647-y) contains supplementary material, which is available to authorized users.

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Abstract

Purpose

Selective IgA deficiency (SIgAD) is the most common humoral primary immunodeficiency. Long-term follow-up data in large cohort of pediatric patients are scarce.

Methods

We report on a single-center cohort of 184 pediatric patients affected with selective IgA deficiency and describe the characteristics at diagnosis and during follow-up.

Results

Respiratory infections were the most common clinical finding leading to the initial diagnosis (62%). Positive family history for antibody deficiencies (selective IgA deficiency, common variable immunodeficiency) led to SIgAD diagnosis in 16% of cases. During follow-up, while the incidence of respiratory infections was not particularly high, gastrointestinal symptoms were reported in 27% of patients. Allergic manifestations were found in 23% at diagnosis and an additional 16% of patients during follow-up, leading to a prevalence of atopy of 39% among SIgAD patients. Autoimmune manifestations, excluding celiac disease, were found in 9% of affected patients during follow-up. Celiac disease was found in a high prevalence (14%). Increase of serum IgA levels to partial deficiency (9%) and normal serum levels for age (4%) was observed during follow-up. A small percentage of patients (2%) progressed to common variable immunodeficiency (CVID).

Conclusions

In conclusion, this is the first study to describe a large single-center pediatric cohort of patients affected with SIgAD, revealing that overall most patients do well with regard to infections. Many develop CD, at a rate much higher than the general population. A few normalize their IgA levels. A few progress to CVID. Thus, careful follow-up is suggested to diagnose and treat potential complications earlier for avoiding potential morbidities.

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