Autoinflammatory & rare systemic diseases
The Positive Predictive Value of a Very High Serum IgG4 Concentration for the Diagnosis of IgG4-Related Disease
Authors' conclusion
The majority of cases with serum IgG4 concentrations > 5× ULN in this study had IgG4-RD.
Study context
- Population
- Cases with IgG4 concentration test results in 2 large academic healthcare systems; IgG4-related disease (IgG4-RD) by ACR/EULAR classification criteriaof 32,206 tested
- Participants
- 191 cases
- Outcome
- PPV of an IgG4 concentration > 5× ULN for IgG4-RD
Methodological detailsretrospective, data warehouses of 2 large academic healthcare systems
- Retrospective
- Data warehouses of 2 large academic healthcare systems
Performance
As reported in the abstract
PPV for IgG4-RD
| Test or model | PPV |
|---|---|
| IgG4 >5× upper limit of normal (ULN) | 75.4% (95% CI 68.7-81.3) |
IgG4 >5× upper limit of normal (ULN)
PPV75.4% (95% CI 68.7-81.3)
Further findings
Elevated IgG4 among all tested cases
- Elevated IgG4 concentrations
- 3039 (9.4%)
- IgG4 > 5× ULN
- 191 (0.6%)
Other conditions in non-IgG4-RD cases
- Elevated IgG4 observed among patients with malignancies, autoimmune diseases, and infections
Original publication
The Positive Predictive Value of a Very High Serum IgG4 Concentration for the Diagnosis of IgG4-Related Disease. J Rheumatol. Published 1 November 2022. doi:10.
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