Effective glucocorticoid treatment improves outcomes for IgG4-related cardiac involvement

Cardiac involvement in immunoglobulin G4-related disease (IgG4-RD) is rare but significant, according to a study.

A recent retrospective study, analyzed 42 patients with heart involvement from a broader cohort of 1075 IgG4-RD cases, collected between 2010 and 2022.

The study found that coronary periarteritis and pericarditis were the most common cardiac manifestations, with incidences of 1.2% and 3.1%, respectively. Valvular disease potentially linked to IgG4-RD was detected in 2 patients, though no cases of myocardial involvement were identified. The patients often presented with asymptomatic conditions, making diagnosis reliant on advanced imaging techniques such as coronary artery CT.

Mass-like and diffuse wall-thickening lesions were the most frequently observed types of coronary periarteritis, while pericarditis commonly appeared as pericardial effusion, localized thickening, calcification, or mass. Treatment with glucocorticoids and immunosuppressants led to significant clinical improvement, with all patients achieving radiological remission and a reduction in IgG4-RD responder index scores. However, 2 patients with coronary periarteritis experienced relapses during the maintenance phase.

Reference
Hua T, Du J, Guo X, et al. Coronary periarteritis and pericarditis are rare but distinct manifestations of heart involvement in IgG4-related disease: a retrospective cohort study. Orphanet J Rare Dis. 2024;19(1):266. doi: 10.1186/s13023-024-03266-y. PMID: 39010060; PMCID: PMC11247878.