Challenges in investigating patients with isolated decreased serum IgM

The SIMcal study.

L.M.A. Janssen, R.W.N.M. van Wout, E. de Vries*

*Corresponding author for this work

Research output: Contribution to journalArticleScientificpeer-review

Abstract

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.
Original languageEnglish
Article number12763
JournalScandinavian journal of immunology
Volume89
Issue number6
DOIs
Publication statusPublished - 2019

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Keywords

  • ADULT PATIENTS
  • DELETION
  • FEATURES
  • FUNCTIONAL ASSESSMENT
  • IMMUNOGLOBULIN-M DEFICIENCY
  • PRIMARY IMMUNODEFICIENCY
  • SUBSETS
  • VALUES
  • primary immunodeficiency
  • primary selective IgM deficiency
  • unclassified antibody deficiency

Cite this

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title = "Challenges in investigating patients with isolated decreased serum IgM: The SIMcal study.",
abstract = "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.",
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author = "L.M.A. Janssen and {van Wout}, R.W.N.M. and {de Vries}, E.",
year = "2019",
doi = "10.1111/sji.12763",
language = "English",
volume = "89",
journal = "Scandinavian journal of immunology",
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Challenges in investigating patients with isolated decreased serum IgM : The SIMcal study. / Janssen, L.M.A.; van Wout, R.W.N.M.; de Vries, E.

In: Scandinavian journal of immunology, Vol. 89, No. 6, 12763, 2019.

Research output: Contribution to journalArticleScientificpeer-review

TY - JOUR

T1 - Challenges in investigating patients with isolated decreased serum IgM

T2 - The SIMcal study.

AU - Janssen, L.M.A.

AU - van Wout, R.W.N.M.

AU - de Vries, E.

PY - 2019

Y1 - 2019

N2 - 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.

AB - 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.

KW - ADULT PATIENTS

KW - DELETION

KW - FEATURES

KW - FUNCTIONAL ASSESSMENT

KW - IMMUNOGLOBULIN-M DEFICIENCY

KW - PRIMARY IMMUNODEFICIENCY

KW - SUBSETS

KW - VALUES

KW - primary immunodeficiency

KW - primary selective IgM deficiency

KW - unclassified antibody deficiency

U2 - 10.1111/sji.12763

DO - 10.1111/sji.12763

M3 - Article

VL - 89

JO - Scandinavian journal of immunology

JF - Scandinavian journal of immunology

SN - 1365-3083

IS - 6

M1 - 12763

ER -