Autoinflammatory & rare systemic diseases
Differential sensitivity of the 2020 revised comprehensive diagnostic criteria and the 2019 ACR/EULAR classification criteria across IgG4-related disease phenotypes: results from a Norwegian cohort
Authors' conclusion
The results from this study indicate that IgG4-RD patients having the "Retroperitoneum and Aorta" phenotype less often fulfill diagnostic criteria and classification criteria than patients with other IgG4-RD phenotypes.
Study context
- Population
- Adult patients diagnosed with IgG4-related disease (IgG4-RD) after comprehensive clinical assessment at Oslo University Hospital, Norway
- Participants
- 79 patients
- Exposure
- Fulfilment of 2020 RCD vs 2019 ACR/EULAR classification criteria across four IgG4-RD phenotypes
- Outcome
- Sensitivity (fulfilment) of the 2020 RCD and the 2019 ACR/EULAR classification criteria across IgG4-RD phenotypes
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
| Group | Value | RCD fulfilled (lowest across phenotypes) | 2019 ACR/EULAR criteria met (lowest across phenotypes) |
|---|---|---|---|
| Retroperitoneum and Aorta | 66.7% | 66.7% | 27.8% |
Retroperitoneum and Aorta
Value66.7%
RCD fulfilled (lowest across phenotypes)66.7%
2019 ACR/EULAR criteria met (lowest across phenotypes)27.8%
- Phenotype groups: P < 0.001 — lowest in Retroperitoneum and Aorta
- Phenotype groups: P < 0.001 — lowest in Retroperitoneum and Aorta
- RCD fulfilled, total cohort
- 7 2/79 (91.1%)
- 2019 ACR/EULAR classification criteria met, total cohort
- 57 (72.2%)
Observational data: associations, not effects.
Further findings
Phenotype distribution
| Outcome | Pancreato-Hepato-Biliary | Retroperitoneum and Aorta | Head and Neck-limited | Mikulicz' and Systemic | P value |
|---|---|---|---|---|---|
| Fulfilment of the 2019 ACR/EULAR classification criteriaLowest across phenotype groups | — not stated in the abstract | 27.8% | — not stated in the abstract | — not stated in the abstract | P < 0.001 |
— not stated in the abstract
- Fulfilment of the 2019 ACR/EULAR classification criteria — Met 2019 ACR/EULAR criteria, whole cohort
- 57 (72.2%)
Original publication
Differential sensitivity of the 2020 revised comprehensive diagnostic criteria and the 2019 ACR/EULAR classification criteria across IgG4-related disease phenotypes: results from a Norwegian cohort. Arthritis Res Ther. Published 5 September 2023. doi:10.
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