An International Multispecialty Validation Study of the IgG4-Related Disease Responder Index.

ABSTRACT:

Objective

IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ, leading to organ dysfunction and failure. The IgG4-RD Responder Index (RI) was developed to help investigators assess the efficacy of treatment in a structured manner. The aim of this study was to validate the RI in a multinational investigation.

Methods

The RI guides investigators through assessments of disease activity and damage in 25 domains, incorporating higher weights for disease manifestations that require urgent treatment or that worsen despite treatment. After a training exercise, investigators reviewed 12 written IgG4-RD vignettes based on real patients. Investigators calculated both an RI score as well as a physician's global assessment (PhGA) score for each vignette

SUBMITTER: Wallace ZS 

PROVIDER: S-EPMC6098740 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature