Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts.

ABSTRACT:

Objective

IgG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to diagnostic delays. We sought to identify key IgG4-RD phenotypes.

Methods

We used two cross-sectional studies assembled by an international, multispecialty network of IgG4-RD specialists who submitted 765 cases to derive and replicate phenotypic groups. Phenotype groups of disease manifestations and key covariate distributions across the identified groups were measured using latent class analysis.

Results

In the derivation cohort (n=493), we identified four groups with distinct manifestations: Group 1 (31%), Pancreato-Hepato-Biliary disease; Group 2 (24%), Retroperitoneal Fibrosis and/or Aorti

SUBMITTER: Wallace ZS 

PROVIDER: S-EPMC6996288 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature