Selective IgM Deficiency: Evidence, Controversies, and Gaps

Author:

Taietti Ivan12ORCID,Votto Martina12,De Filippo Maria12,Naso Matteo12ORCID,Montagna Lorenza1,Montagna Daniela12ORCID,Licari Amelia12ORCID,Marseglia Gian Luigi12,Castagnoli Riccardo12ORCID

Affiliation:

1. Pediatric Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences, University of Pavia, 27100 Pavia, Italy

2. Pediatric Clinic, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy

Abstract

Selective Immunoglobulin M deficiency (SIgMD) has been recently included in the inborn errors of immunity (IEI) classification by the International Union of Immunological Societies Expert Committee. The understanding of SIgMD is still extremely limited, especially so in cases of SIgMD in the pediatric population. The epidemiology of SIgMD in the pediatric population is still unknown. The pathogenesis of SIgMD remains elusive, and thus far no genetic nor molecular basis has been clearly established as a definitive cause of this primary immunodeficiency. Recurrent respiratory infections represent the main clinical manifestations in children, followed by allergic and autoimmune diseases. No conclusive data on the correct therapeutic management of SIgMD are available. Although, for most SIgMD patients, Ig replacement therapy is not required, it may be recommended for patients with significantly associated antibody deficiency and recurrent or severe infections. Prophylactic antibiotics and the prompt treatment of febrile illness are crucial. There is insufficient evidence on the prognosis of this condition. Therefore, further studies are required to define the disease trajectories and to increase our understanding of the molecular mechanisms underlying SIgMD in order to facilitate a better clinical, immunological, and prognostic characterization of the condition and develop tailored therapeutic management strategies.

Publisher

MDPI AG

Subject

Clinical Biochemistry

Reference94 articles.

1. International Union of Immunological Societies: 2017 Primary Immunodeficiency Diseases Committee Report on Inborn Errors of Immunity;Picard;J. Clin. Immunol.,2018

2. Human Inborn Errors of Immunity: 2022 Update on the Classification from the International Union of Immunological Societies Expert Committee;Tangye;J. Clin. Immunol.,2022

3. The 2022 Update of IUIS Phenotypical Classification for Human Inborn Errors of Immunity;Bousfiha;J. Clin. Immunol.,2022

4. (2023, March 25). ESID—European Society for Immunodeficiencies. Available online: https://esid.org/Working-Parties/Registry-Working-Party/Diagnosis-criteria.

5. Selective IgM Deficiency—An Underestimated Primary Immunodeficiency;Gupta;Front. Immunol.,2017

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