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Clinical and immunological phenotypes of selective IgM deficiency in children: Results from a multicenter study
Riccardo Castagnoli1,2, Ivan Taietti1,2, Martina Votto1,2
1Pediatric Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences, University of Pavia, Pavia, Italy.
Insights
Selective IgM deficiency (SIgMD) in children often presents with recurrent infections and allergies. Comprehensive immunological work-up and long-term follow-up are crucial for accurate diagnosis and management of SIgMD.
Area of Science:
- Immunology
- Pediatrics
- Genetics
Background:
- Selective IgM deficiency (SIgMD) is a rare primary immunodeficiency.
- Limited data exists on the clinical and immunological features of SIgMD in pediatric populations.
Purpose of the Study:
- To characterize the clinical and immunological phenotypes of pediatric patients with SIgMD.
- To evaluate SIgMD phenotypes based on different diagnostic criteria.
Main Methods:
- Multicenter study of pediatric SIgMD patients.
- Diagnosis evaluated over several months to years.
- Analysis of clinical manifestations and immunological parameters.
Main Results:
- Forty-eight pediatric patients with SIgMD were included (mean serum IgM: 33 mg/dL).
- Recurrent infections (67%) and allergies (48%) were the most common manifestations.
- Long-term follow-up showed 87% retained SIgMD diagnosis; two developed IgA reduction.
Conclusions:
- Reduced serum IgM in children warrants a complete immunological evaluation.
- Long-term follow-up is essential for understanding SIgMD evolution and guiding management.
Background:
A few studies assessed the clinical and immunological features of selective IgM deficiency (SIgMD), especially in the pediatric age. We aimed to characterize the clinical and immunological phenotypes of a cohort of pediatric patients with SIgMD according to the different diagnostic criteria available.
Methods:
In this multicenter study, we evaluated pediatric SIgMD patients diagnosed at the Pediatric Clinic in Pavia, Italy, or through the Italian Primary Immunodeficiency NETwork (IPINET) and monitored changes in their diagnosis over a time frame that ranges from several months to several years.
Results:
Forty-eight patients with SIgMD were included (mean serum IgM: 33 mg/dL). The most common clinical manifestations were recurrent infections (67%) and allergies (48%). Subgroup analysis according to SIgMD definition criteria of the European Society for Immunodeficiencies (ESID) showed no significant difference in clinical manifestations, also considering the group with additional immunological abnormalities. Sixteen patients had long-term follow-up, during which 87% preserved their SIgMD diagnosis, while two patients showed a reduction in IgA in addition to low IgM.
Conclusions:
Our data suggest that the identification of a reduction in serum IgM in children should lead to a complete immunological work-up to obtain a comprehensive clinical and immunological characterization of the patient. The follow-up of these patients is fundamental to define the disease evolution and appropriate management.
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