Autoinflammatory & rare systemic diseases
The co-occurrence of IgG4-related disease and malignancy: insights from a prospective cohort study
Authors' conclusion
IgG4-RD patients exhibit an elevated risk of specific malignancies, particularly lymphoma, with elevated ESR and greater organ involvement identified as significant risk factors for malignancy development during follow-up period.
Study context
- Population
- Patients with IgG4-related disease (IgG4-RD) in a prospective cohort22 with malignancies
- Participants
- 278 patients
- Exposure
- Malignancy in IgG4-RD patients vs expected incidence (SIR); follow-up vs history/concurrent malignancy groups
- Outcome
- Malignancy development (tumour distribution and risk factors)
Methodological detailsstandardised incidence ratios, Bayesian statistical models
- Standardised incidence ratios
- Bayesian statistical models
Main finding
As reported in the abstract
Patients who developed malignancies
22
7.9%
Overall SIR
3.28
95% CI 1.31–5.58
Lymphoma SIR
17.63
95% CI 0–52.89
Observational data: associations, not effects.
Further findings
Lung cancer (most prevalent malignancy, 13.6%), follow-up vs history/concurrent
P = 0.008
| Outcome | Follow-up | History/concurrent | P value |
|---|---|---|---|
| Testicular cancer | — not stated in the abstract | 3.5% | P = 0.044 |
— not stated in the abstract
Testicular cancer
- Testicular cancer absent in the follow-up group; occurred exclusively in the history/concurrent group
Lymphoma SIR
- Lymphoma SIR
- 17.63 (95% CI 0-52.89)
Risk factors for malignancy development (Bayesian models) · follow-up
- Elevated ESR, estimate
- 0.12 (95% CI 0.04-0.23)
- More organ involvements, estimate
- 1.94 (95% CI 0.31-3.93)
Original publication
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