Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts.
Ann Rheum Dis
; 78(3): 406-412, 2019 03.
Article
in En
| MEDLINE
| ID: mdl-30612117
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 Aortitis; Group 3 (24%), Head and Neck-Limited disease and Group 4 (22%), classic Mikulicz syndrome with systemic involvement. We replicated the identification of four phenotype groups in the replication cohort. Compared with cases in Groups 1, 2 and 4, respectively, cases in Group 3 were more likely to be female (OR 11.60 (95% CI 5.39 to 24.98), 10.35 (95% CI 4.63 to 23.15) and 9.24 (95% CI 3.53 to 24.20)) and Asian (OR 6.68 (95% CI 2.82 to 15.79), 7.43 (95% CI 2.97 to 18.56) and 6.27 (95% CI 2.27 to 17.29)). Cases in Group 4 had a higher median serum IgG4 concentration (1170 mg/dL) compared with groups 1-3 (316, 178 and 445 mg/dL, respectively, p<0.001).CONCLUSION:
We identified four distinctive IgG4-RD phenotypes according to organ involvement. Being Asian or female may predispose individuals to head and neck-limited disease. These phenotypes serve as a framework for identifying IgG4-RD and studying its aetiology and optimal treatment.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Otorhinolaryngologic Diseases
/
Aortitis
/
Retroperitoneal Fibrosis
/
Digestive System Diseases
/
Immunoglobulin G4-Related Disease
/
Mikulicz' Disease
Type of study:
Clinical_trials
/
Observational_studies
/
Prevalence_studies
/
Risk_factors_studies
Limits:
Adult
/
Female
/
Humans
/
Male
/
Middle aged
Country/Region as subject:
Asia
/
Europa
Language:
En
Journal:
Ann Rheum Dis
Year:
2019
Document type:
Article
Affiliation country: