Autoinflammatory & rare systemic diseases

Potential impact of autoimmune diseases family history in IgG4-related disease: a retrospective cohort study

RMD OpenPublished Retrospective cohort study

Authors' conclusion

14.8% of IgG4-RD patients had AID family history, with younger age of disease onset age and higher frequency of ANA positivity in AID-positive group, indicating that IgG4-RD may share genetic background with other AID.

Study context

Population
Patients with IgG4-related Disease (IgG4-RD), Peking Union Medical College Hospital, China
Participants
628 patients
Exposure
IgG4-RD with autoimmune diseases (AID) family history vs without AID family history
  • AID-positiven = 93
Outcome
Prevalence of AID family history and its correlation with IgG4-RD features
Methodological detailssingle-centre, Cox regression
  • Single-centre
  • Cox regression

Main finding

As reported in the abstract

AID family history

AID-positive

14.8%

93

Observational data: associations, not effects.

Further findings

ANA positivity · baseline

P = 0.0277*

Riedel thyroiditis · baseline

P = 0.001*

Comorbid other AID · baseline

P = 0.0238*

Risk of unfavourable prognosis in AID-positive IgG4-RD (Cox analysis)

HR (95% CI)P value
Younger age0.97 (0.94–0.99)P = 0.0384*
Higher baseline peripheral eosinophils1.1 (1.02–1.2)P = 0.0199*
OutcomeAID-positiveAID-negativeP value
Age of IgG4-RD onset · baselineEarlier onset in AID-positive50.4 ± 14.854.2 ± 12.6P = 0.014*

Original publication

Potential impact of autoimmune diseases family history in IgG4-related disease: a retrospective cohort study. RMD Open. Published 9 February 2023. doi:10.1136/rmdopen-2022-002865

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