Autoinflammatory & rare systemic diseases

Increased prevalence of malignancies in patients with IgG4-related disease: implications for clinical care

Rheumatology (Oxford)Published Retrospective cohort study

Main finding · Cancer before or after IgG4-RD diagnosisResult — go to the resultStandardized incidence ratio 2.54

Authors' conclusion

The prevalence of cancer in patients with IgG4-RD is increased compared with the Italian population and mechanistically suggests a possible paraneoplastic association.

Study context

Population
Patients with IgG4-related disease (IgG4-RD) (Italy)
Participants
210 patients
Exposure
IgG4-RD vs Italian general population
  • IgG4-RDn = 210
Outcome
Prevalence of malignancies in IgG4-RD
Methodological detailscomparison with general population registry (WHO Global Cancer Observatory), standardised incidence ratio
  • Comparison with general population registry (WHO Global Cancer Observatory)
  • Standardised incidence ratio

Main finding

Cancer before or after IgG4-RD diagnosis

Standardized incidence ratio

2.54

P = 0.007

Events by group

IgG4-RD

18%

37 of 210

As reported in the abstract

Observational data: associations, not effects.

Further findings

Malignancy Standardized Incidence Ratio (SIR) vs general Italian population, by sex (subgroup)

SIRP value
Males2.78P = 0.005
Females1.15P > 0.05

Cancer by IgG4-RD phenotype

Solid and haematologic tumours more frequent in pancreato-biliary IgG4-RD

Timing of malignancy relative to IgG4-RD diagnosis

Malignancies diagnosed before IgG4-RD
32
Malignancies diagnosed after IgG4-RD
16
Interval from IgG4-RD to cancer shorter than from cancer to IgG4-RD
Most tumours after IgG4-RD developed within 36 months of IgG4-RD diagnosis

Original publication

Increased prevalence of malignancies in patients with IgG4-related disease: implications for clinical care. Rheumatology (Oxford). Published 2 May 2024. doi:10.1093/rheumatology/keae243

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