Challenges in investigating patients with isolated decreased serum IgM: The SIMcal study

Autor: Janssen, Lisanne M A, van Hout, Roeland W N M, de Vries, Esther, Pignata, C, Cirillo, E, Arkwright, P D, Lougaris, V, Buckland, M, Garcia-Prat, M, d'Hebron, V, Soler-Palacin, P, Ouederni, M, Kralickova, P, Abolhassani, H, Hammerstrom, L, Aghamohamamdi, A, Santos-Pérez, J L, Sobh A, van de Werff Ten Bosch, J, Henriet, S, Kilic, S S, Karali, Y, Gonzalez-Granado, L I, Sediva, A
Přispěvatelé: Huisarts & Ziekenhuis, Tranzo, Scientific center for care and wellbeing, Janssen, Lisanne M A, van Hout, Roeland W N M, de Vries, Esther, Pignata, C, Cirillo, E, Arkwright, P D, Lougaris, V, Buckland, M, Garcia-Prat, M, D'Hebron, V, Soler-Palacin, P, Ouederni, M, Kralickova, P, Abolhassani, H, Hammerstrom, L, Aghamohamamdi, A, Santos-Pérez, J L, Sobh, A, van de Werff Ten, Bosch, J, Henriet, S, Kilic, S S, Karali, Y, Gonzalez-Granado, L I, Sediva, A
Rok vydání: 2019
Předmět:
Male
0301 basic medicine
Pediatrics
unclassified antibody deficiency
lnfectious Diseases and Global Health Radboud Institute for Molecular Life Sciences [Radboudumc 4]
Immunologic Deficiency Syndromes/blood
Human Immunology
0302 clinical medicine
Decreased serum IgM
T-Lymphocyte Subsets
Young adult
Child
Respiratory Tract Infections
IgM deficiency
biology
primary selective IgM deficiency
General Medicine
Middle Aged
Child
Preschool

Cohort
Variation and Distance
Female
medicine.symptom
Antibody
B-Lymphocyte Subsets/cytology
Adult
medicine.medical_specialty
Adolescent
Asymptomatic Diseases/epidemiology
Immunology
B-Lymphocyte Subsets
Respiratory Tract Infections/epidemiology
primary immunodeficiency
Asymptomatic
Young Adult
03 medical and health sciences
All institutes and research themes of the Radboud University Medical Center
medicine
Humans
In patient
Preschool
business.industry
Immunologic Deficiency Syndromes
Infant
Newborn

Infant
Newborn
Immunoglobulin M/blood
medicine.disease
Language & Communication
T-Lymphocyte Subsets/cytology
030104 developmental biology
Immunoglobulin M
Asymptomatic Diseases
Primary immunodeficiency
biology.protein
business
030215 immunology
Zdroj: The SIMcal Consortium 2019, ' Challenges in investigating patients with isolated decreased serum IgM : The SIMcal study ', Scandinavian Journal of Immunology, vol. 89, no. 6, e12763 . https://doi.org/10.1111/sji.12763
Scandinavian journal of immunology, 89(6):e12763. Wiley-Blackwell Publishing Ltd
Scandinavian Journal of Immunology, 89
Scandinavian Journal of Immunology, 89, 6
Scandinavian Journal of Immunology
ISSN: 1365-3083
0300-9475
DOI: 10.1111/sji.12763
Popis: The clinical consequences of isolated decreased serum immunoglobulin (Ig)M are not sufficiently known. Therefore, it is difficult to determine the clinical policy following such a finding. Only few reported IgM‐deficient patients fulfil the European Society for Immunodeficiencies (ESID) diagnostic criteria for selective IgM deficiency (true sIgMdef), or their diagnosis is uncertain due to insufficient laboratory data (possible sIgMdef). Decreased serum IgM is often incidentally found in asymptomatic adults. The objective of our study was to further characterize true sIgMdef and to compare the European data collected through the ESID Registry community (tertiary centres) to our previously published Dutch cohort (secondary centre). Fifteen centres (12 countries) participated with 98 patients. Patients were excluded if serum IgM was only determined once (n = 14), had normalized (n = 8), or if they also had other immunological abnormalities (n = 15). Ten patients (5 adults) completely fulfilled the ESID criteria for true sIgMdef. Age‐matched cut‐off values varied widely between centres; when using the ESID diagnostic protocol reference values, only six patients (five adults) had true sIgMdef. Because of these small numbers, further analyses were performed in patients with true or possible sIgMdef (13 adults, 48 children). Respiratory infections were commonly reported at presentation (adults 54%, children 60%). Symptomatic adults had lower serum IgM levels (mean 0.27 g/L, 95% CI 0.22‐0.31) than those without symptoms (mean 0.33 g/L, 95% CI 0.30‐0.36; P = 0.02). To be able to explore the clinical consequences of true sIgMdef, we should fully analyse and accurately describe those patients in whom a decreased serum IgM is found.
Databáze: OpenAIRE