Autoinflammatory & rare systemic diseases
The external validation of the 2019 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for IgG4-related disease in a large cohort from China
Authors' conclusion
The 2019 ACR/EULAR classification criteria for IgG4-RD show outstanding specificity and good sensitivity in real-world clinical practice.
Study context
- Population
- 875 IgG4-related disease (IgG4-RD) and 302 non-IgG4-RD cases (213 mimickers and 89 patients with other diseases), cohort from China875 IgG4-RD and 302 non-IgG4-RD cases
- Comparison
- 2019 ACR/EULAR classification criteria vs 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD, against expert clinical judgment
- Outcome
- Performance (sensitivity, specificity, AUC) of the 2019 ACR/EULAR criteria for IgG4-RD
Methodological detailsexternal validation
- External validation
Performance
As reported in the abstract
Diagnostic performance of the 2019 ACR/EULAR criteria, overall cohort
| Test or model | Sensitivity | Specificity | AUC |
|---|---|---|---|
| 2019 ACR/EULAR criteria | 76.6% (95% CI: 73.8% to 79.4%) | 98.0% (96.0%-99.4%) | 0.873 (0.857-0.889) |
2019 ACR/EULAR criteria
Sensitivity76.6% (95% CI: 73.8% to 79.4%)
Specificity98.0% (96.0%-99.4%)
AUC0.873 (0.857-0.889)
Further findings
False negative vs true positive cases under the 2019 ACR/EULAR criteria
- False negatives had significantly lower serum IgG4, fewer had pathological information, and more often had uncommon organ involvement vs true positives
Cases negative by 2019 ACR/EULAR but "definite" by 2020 RCD criteria
- More involvement of uncommon organs
Original publication
The external validation of the 2019 ACR/EULAR classification criteria for IgG4-related disease in a large cohort from China. Semin Arthritis Rheum. Published 29 March 2023. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →