Overview
Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect various organs, including the coronary arteries 1. IgG4-RD involving the coronary arteries, also known as IgG4-related coronary arteritis, is a rare manifestation of this systemic disease 1.Diagnosis
Diagnosis of IgG4-related coronary arteritis is typically made based on a combination of clinical, serological, imaging, and histopathological findings 1.
Key features may include thickening of the coronary artery wall, luminal stenosis, and characteristic histopathological findings of lymphoplasmacytic infiltration with IgG4-positive plasma cells 1.
Imaging modalities such as computed tomography angiography (CTA) or magnetic resonance angiography (MRA) can help identify coronary artery abnormalities 1.
Elevated serum IgG4 levels are often observed but are not diagnostic on their own 1.Management
Management of IgG4-related coronary arteritis is primarily guided by the principles of IgG4-RD treatment, which often involves immunosuppressive therapy 1.
Glucocorticoids are typically the first-line treatment, with the goal of inducing remission and reducing inflammation 1.
Glucocorticoid-sparing agents, such as rituximab or azathioprine, may be considered for patients who do not respond adequately to glucocorticoids or for long-term maintenance therapy to prevent relapse 1.
The specific treatment strategy should be individualized based on the extent and severity of coronary involvement and the presence of other organ manifestations 1.Key Recommendations
Glucocorticoid-sparing immunosuppressive agents should be considered in the management of large vessel vasculitis, including conditions that may involve coronary arteries 1. (Evidence: Moderate)
Imaging should be used to identify large vessel involvement in conditions like IgG4-related coronary arteritis 1. (Evidence: Moderate)
Nonglucocorticoid immunosuppressive agents should be used in conjunction with glucocorticoids as initial therapy for large vessel vasculitis 1. (Evidence: Moderate)References
1 Maz M, Chung SA, Abril A, Langford CA, Gorelik M, Guyatt G et al.. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis & rheumatology (Hoboken, N.J.) 2021. link