Autoinflammatory & rare systemic diseases
IgG4-related disease as a variable-vessel vasculitis: A case series of 13 patients with medium-sized coronary artery involvement
Authors' conclusion
Coronary arteritis and periarteritis are important manifestations of IgG4-RD, which should be regarded as a variable-vessel vasculitis that is among the most diverse forms of vasculitis known.
Study context
- Population
- Patients with igG4-related disease (IgG4-RD) from a large, prospective IgG4-RD cohort13 with coronary artery involvement (CAI)
- Participants
- 361 patients
- Outcome
- Coronary artery involvement (CAI) in IgG4-RD: frequency and manifestations
Methodological detailsfrom a large prospective IgG4-RD cohort
- From a large prospective IgG4-RD cohort
Main finding
As reported in the abstract
IgG4-related CAI
4%
13
- Serum IgG4, median (IQR: 510-1568 mg/dL; reference: 4-86 mg/dL)
- 955 mg/dL
- Disease duration at CAI diagnosis, median (IQR: 8.23-15.5 years)
- 11 years
- All three major coronary arteries involved
- 11 (85%)
- Wall thickening or periarterial soft tissue encasement
- 85%
- Stenosis
- 69%
- Calcification
- 69%
- Aneurysms or ectasia
- 62%
Observational data: associations, not effects.
Safety
Complications of CAI
- MI
- 5 (38%)
- Coronary artery bypass grafting required
- 2 (15%)
- Ischemic cardiomyopathy
- 2 (15%)
Original publication
IgG4-related disease as a variable-vessel vasculitis: A case series of 13 patients with medium-sized coronary artery involvement. Semin Arthritis Rheum. Published 18 February 2023. doi:10.
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