Autoinflammatory & rare systemic diseases

Distinct impact of malignancy and allergy on the clinical and immunological features of IgG4-related disease

Clin Exp RheumatolPublished Retrospective cohort study

Authors' conclusion

The clinical and immunological phenotypes of IgG4-RD differ among those with underlying diseases.

Study context

Population
Consecutive patients with IgG4-RD treated at Keio University Hospital, 2010–2021
Participants
123 patients
Exposure
IgG4-RD with malignancy vs allergy alone vs neither
  • Malignancyn = 18
  • Allergyn = 57
  • Idiopathicn = 48
Outcome
Clinical characteristics by group
Methodological detailssingle-centre, consecutive patients
  • Single-centre
  • Consecutive patients

Main finding

As reported in the abstract

Age (years), malignancy vs allergy vs idiopathic: 70.1 vs. 54.4 vs. 64.9
p<0.001
Male (%), malignancy vs allergy vs idiopathic: 83.3 vs. 42.1 vs. 54.2
p=0.008
Smoking habits (%), malignancy vs allergy vs idiopathic: 77.8 vs. 42.1 vs. 43.8
p=0.02
Aorta/large vessel involvement (%), malignancy vs allergy vs idiopathic: 33.3 vs. 7.0 vs. 20.8
p=0.02
Overall survival significantly poorer in malignancy group
p=0.02

Observational data: associations, not effects.

Further findings

Peripheral blood immunophenotyping (56 immune cell subsets)

Increase in CXCR5+CD2-double negative T cells and decrease in naive CD8 T cells characteristic of malignancy group

Original publication

Distinct impact of malignancy and allergy on the clinical and immunological features of IgG4-related disease. Clin Exp Rheumatol. Published 14 October 2022. doi:10.55563/clinexprheumatol/7g2na0

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