Selective IgM deficiency: evaluation of 75 patients according to different diagnostic criteria.

Autor: Batista CHR; Disciplina de Imunologia Clínica, Faculdade de Medicina, Centro Universitário FMABC, Avenida Lauro Gomes, 2000, Santo Andre, SP, 09060.870, Brazil., Smanio MCM; Disciplina de Imunologia Clínica, Faculdade de Medicina, Centro Universitário FMABC, Avenida Lauro Gomes, 2000, Santo Andre, SP, 09060.870, Brazil., Poltronieri PB; Disciplina de Imunologia Clínica, Faculdade de Medicina, Centro Universitário FMABC, Avenida Lauro Gomes, 2000, Santo Andre, SP, 09060.870, Brazil., Resende LL; Disciplina de Imunologia Clínica, Faculdade de Medicina, Centro Universitário FMABC, Avenida Lauro Gomes, 2000, Santo Andre, SP, 09060.870, Brazil., Kokron CM; Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil., Barros MT; Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil., Agondi RC; Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil., Ferraroni NR; Clínica Ferraroni, Brasília, DF, Brazil., Roxo-Junior P; Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil., Ferriani MPL; Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil., Chong-Neto H; Serviço de Alergia E Imunologia, Complexo Hospital de Clínicas, Universidade Federal Do Paraná, Curitiba, Brazil., Rosario Filho N; Serviço de Alergia E Imunologia, Complexo Hospital de Clínicas, Universidade Federal Do Paraná, Curitiba, Brazil., Kamoi TO; Clínica Privada, Curitiba, Paraná, Brazil., Di Gesu R; Hospital Nossa Senhora Conceição, Porto Alegre, RS, Brazil., Goudouris E; Universidade Federal Do Rio de Janeiro, Rio de Janeiro, RJ, Brazil., Aranda CS; Federal University of São Paulo, São Paulo, SP, Brazil., Mansour E; State University of Campinas, Campinas, SP, Brazil., Henriques MT; Disciplina de Imunologia Clínica, Faculdade de Medicina, Centro Universitário FMABC, Avenida Lauro Gomes, 2000, Santo Andre, SP, 09060.870, Brazil., Bardou MLD; Disciplina de Imunologia Clínica, Faculdade de Medicina, Centro Universitário FMABC, Avenida Lauro Gomes, 2000, Santo Andre, SP, 09060.870, Brazil., Antila HG; Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil., Grumach AS; Disciplina de Imunologia Clínica, Faculdade de Medicina, Centro Universitário FMABC, Avenida Lauro Gomes, 2000, Santo Andre, SP, 09060.870, Brazil. anete.grumach@fmabc.net.
Jazyk: angličtina
Zdroj: Immunologic research [Immunol Res] 2024 Dec 16; Vol. 73 (1), pp. 15. Date of Electronic Publication: 2024 Dec 16.
DOI: 10.1007/s12026-024-09568-4
Abstrakt: Selective IgM deficiency (SIgMD) has recently been included in the inborn errors of immunity classification. SIgMD has conflicting diagnostic criteria and diverse clinical and immunological findings. We aimed to assess the clinical and laboratory profiles of patients with SIgMD and to compare the data of patients diagnosed using two inclusion criteria. This was a descriptive, retrospective, observational, collaborative study. Patients were included according to the following definitions: Group 1, IgM levels < 0.20 g/L in children and < 0.30 g/L in adults, and Group 2, serum IgM levels below 2SD and, for both, absence of associated immunological diseases or secondary causes. The protocol was approved by the Ethics Committee, and patients provided consent. In total, 75 patients were included: 37 (16 M:21F; mean age, 52.92) and 38 (13 M:25F; mean age, 53.47) in Groups 1 and 2, respectively. The most frequent clinical manifestations were allergic rhinitis (G1, 45.9%; G2, 36.8%), asthma (G1, 37.8%; G2, 28.9%), and pulmonary infections (G1, 27.03%; G2, 21.05%). Chromosomopathies (16.22%) and neoplasia (13.51%) were more frequent in G1, whereas URTI (23.68%) and skin infections (23.68%) were more common in G2. There was no difference in sex or mean age at symptom onset between both groups of patients. Regarding the clinical picture, 90.7% of the lesions were benign (68/75). Chromosomopathies may be associated with SIgMD, suggesting the need to quantify serum IgM levels in these cases. Considering the possibility of developing autoimmunity, neoplasia, and common variable immunodeficiency, it is advisable to follow up patients with SIgMD.
Competing Interests: Declarations. Ethics approval: This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of University Center FMABC on January 17, 2021 (CAAE 52536915.5.0000.0082). All the authors were consulted and consented to participate and submit to the Journal of Clinical Immunology. Consent to participate: Informed consent was obtained from all individual participants included in the study. The consent was read and explained for each participant, and all the text was previously revised by the ethical committee. Patients younger than 18 years old had a specific consent, and the parent or legal guardian also signed the consent to participate in the study. Consent for publication: The patient or in the case of patients less than 18 years old, his/her parent or legal guardian authorized the publication of the results without identifying their names. The patients were nominated by numbers to maintain their identity anonymous. Conflict of interest: No conflicts of interest related to this project. ASG belongs to the editorial board. ASG has been a consultant and/or speaker for CSL Behring, Takeda, and Catalyst. CMK has been a speaker for Takeda. PRJ has been a consultant and/or speaker for CSL Behring, Takeda, Novartis, and Sanofi. EM has been a speaker for Takeda, CSL Behring, Novartis, and Sanofi and a consultant for Takeda and CSL Behring. CSA has been a speaker for Takeda, CSL Behring, Sanofi, and Roche and a consultant for PTC, EUSA Pharma, and Sanofi.
(© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
Databáze: MEDLINE
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