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

Lougaris, Vassilios; Sorlini, Annamaria; Monfredini, Chiara; Ingrasciotta, Giulia; Caravaggio, Andrea; Lorenzini, Tiziana; Baronio, Manuela; Cattalini, Marco; Meini, Antonella; Ruggeri, Lucia; Salpietro, Annamaria; Pilotta, Alba; Grazzani, Livia; Prandi, Elena; Felappi, Barbara; Gualdi, Giulio; Fabiano, Antonella; Fuoti, Maurizio; Ravelli, A; Villanacci, Vincenzo; Soresina, Annarosa; Badolato, Raffaele; Plebani, Alessandro

doi:10.1007/s10875-019-00647-y

Cited by 26 publications

(19 citation statements)

References 20 publications

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“…When looking instead at cohorts of patients with SIgAD, a higher prevalence of celiac disease may found. In Italy, 184 pediatric patients who had come to clinical attention with SIgAD were characterized; 14% had celiac disease [ 94 ]. It is unclear why celiac disease is associated with SIgAD; secretory IgA can bind to proteins such as gliadin and tissue transglutaminase but the absence of IgA may lead to abnormal processing of these proteins [ 25 ].…”

Section: Clinical Manifestations and Managementmentioning

confidence: 99%

The clinical implications of selective IgA deficiency

Swain

Selmi

Gershwin

et al. 2019

Journal of Translational Autoimmunity

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Selective IgA deficiency (SIgAD) is the most common primary immunodeficiency but does not always result in clinical disease. This may in part be due to the definition based on serum IgA, while most IgA is secreted at mucosal surfaces, not amenable to measurement. Clinical complications include increased risk of sinopulmonary infections with bacteria and viruses, gastrointestinal infections with a predilection for Giardia lamblia , a myriad of autoimmune diseases including systemic lupus erythematosus, hyper- and hypo-thyroidism, Type 1 diabetes, celiac disease, and rarely, malignancy. SIgAD must be differentiated from IgA deficiency that may be seen with IgG2 or IgG4 deficiency, specific antibody deficiency, or as an early manifestation prior to a diagnosis of common variable immunodeficiency. Secondary IgA deficiency is increasingly recognized and may be due to medications such as anti-epileptics, or antibiotics with disruption of the microbiome which can influence IgA levels, infections or malignancies. Patients with SIgAD should be monitored at regular intervals and educated to be aware of particular complications. There is a rare chance of development of anti-IgA IgE antibodies in patients with complete deficiency, which can result in anaphylaxis if blood products with IgA are administered. Prophylactic antibiotics may be indicated in some cases, and very rarely, supplemental IgG infusions.

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Section: Clinical Manifestations and Managementmentioning

confidence: 99%

The clinical implications of selective IgA deficiency

Swain

Selmi

Gershwin

et al. 2019

Journal of Translational Autoimmunity

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“…Some chromosomal disorders, including chromosome 18 abnormalities, trisomy 21, and 22q11.2 microdeletion are associated with SIAD ( 119 – 123 ). In addition, familial clustering may occur ( 76 , 124 – 126 ). SIAD may be transient in children, or sometimes, it may evolve to CVID ( 76 , 127 , 128 ).…”

Section: Introductionmentioning

confidence: 99%

Update on Infections in Primary Antibody Deficiencies

Demirdağ

Gupta

2021

Front. Immunol.

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Bacterial respiratory tract infections are the hallmark of primary antibody deficiencies (PADs). Because they are also among the most common infections in healthy individuals, PADs are usually overlooked in these patients. Careful evaluation of the history, including frequency, chronicity, and presence of other infections, would help suspect PADs. This review will focus on infections in relatively common PADs, discussing diagnostic challenges, and some management strategies to prevent infections.

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“…It is defined as serum IgA levels <0.07 g/L with normal serum levels of IgM and IgG in individuals of 4 years old 1,2 . Affected individuals may be asymptomatic and screened out during physical examination 3 …”

Section: Introductionmentioning

confidence: 99%

The epidemiology and clinical feature of selective immunoglobulin a deficiency of Zhejiang Province in China

Zhang

Kong

et al. 2020

Clinical Laboratory Analysis

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Background Selective immunoglobulin A deficiency (SIgAD) is the most common primary antibody deficiency disease and frequently reported in the Western countries. However, large‐scale epidemiologic studies on SIgAD in China are still lacking. Methods The clinical information of 555 180 subjects (age >4 years) including the outpatient, inpatient, and healthy subjects who had ordered serum immunoglobulin A, G, M in 9 hospitals of Zhejiang Province in China was collected. The SIgAD individuals were defined as IgA level <0.07 g/L with normal levels of serum IgG and IgM, whose age should be over 4 years, and any other secondary diseases causing SIgAD were also excluded. Then, the geographical and prevalence distribution of SIgAD individuals in Zhejiang Province and patients' clinical characteristics at the time of diagnosis were also reviewed. Result Among these 555 180 subjects who had ordered the immunoglobulin evaluation, the prevalence of SIgAD was 109/555180 (0.02%). The ratio of male to female of these SIgAD individuals was 1:1.37, which also included 87 adults (≥18 years) and 22 children (18 > age >4 years). For adults, the common clinical features were infections (43/87, 49.43%), autoimmune disorders (31/87, 35.63%), allergic cases (5/87, 5.75%), and tumor cases (4/87, 4.60%). Additionally, infectious diseases (20/22, 90.91%), autoimmune disorders (4/22, 18.18%), and allergic cases (1/22, 4.55%) were found in 22 children. Conclusion We first describe a large cohort of SIgAD individuals of Zhejiang Province in China. The incidence was 0.020%. The common clinical features were infection, autoimmune disorders, tumor, and allergy, and the infection rate was higher in children than the adults.

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

Cited by 26 publications

References 20 publications

The clinical implications of selective IgA deficiency

The clinical implications of selective IgA deficiency

Update on Infections in Primary Antibody Deficiencies

The epidemiology and clinical feature of selective immunoglobulin a deficiency of Zhejiang Province in China

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