Autoinflammatory & rare systemic diseases

The development and initial validation of IgG4-related disease damage index: a consensus report from Chinese IgG4-RD Consortium

RMD OpenPublished Consensus development and validation study

Authors' conclusion

The IgG4-RD DI is a useful approach to analyse disease outcomes, and it has good operability and credibility.

Study context

Population
40 IgG4-RD cases representing four clinical scenarios, each with two time points of assessment over at least 3 years of follow-up, rated by 48 rheumatologists from 35 hospitals in China48 raters
Participants
40 cases
Outcome
Development, reliability and criterion validity of the IgG4-RD DI
Methodological detailsDelphi method, inter-rater reliability, criterion validity
  • Delphi method
  • Inter-rater reliability
  • Criterion validity

Recommendations

As reported in the abstract

Structure: cumulative index of 14 organ-system domains
39 items
Intraclass correlation coefficient (ICC) at the two time points
0.69 and 0.70
KW at the two time points
0.74 and 0.73
Sensitivity
0.86 (95% CI 0.82 to 0.88)
Specificity
0.79 (95% CI 0.76 to 0.82)
AUC
0.88 (95% CI 0.85 to 0.91)

Further findings

Inter-rater reliability in subgroups

Over

ICC in all subgroups
0.55
KW in all subgroups
0.61

Original publication

The development and initial validation of IgG4-related disease damage index: a consensus report from Chinese IgG4-RD Consortium. RMD Open. Published 8 March 2024. doi:10.1136/rmdopen-2023-003938

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