Intracoronary IgG4-related disease as an unusual cause of sudden cardiac arrest: a case series.

Background: IgG4-related disease (IgG4-RD) is a fibro-inflammatory condition classically causing retroperitoneal fibrosis, aortitis, thyroiditis, or pancreatitis. Diagnosis includes the presence of lymphoplasmacytic infiltrate (with >40% ratio IgG4+:IgG plasma cells) and fibrosis. Cardiac involvement may include aortic, pericardial, or coronary disease. Coronary manifestations encompass obstructive intra-luminal lesions, external encasing pseudo-tumour on imaging, or lymphoplasmacytic arteritis.
Case Summary: Case 1 : A fit and healthy 50-year-old man was found deceased. His only known medical condition was treated Hashimoto's thyroiditis. Post-mortem examination demonstrated an isolated severe stenosis of the left anterior descending (LAD) coronary artery without histopathological evidence of acute myocardial infarction. Coronary plaque histopathology showed florid IgG4-positive plasma cell infiltrate throughout all layers of the artery with dense fibrous tissue connective tissue stroma, all features consistent with coronary artery IgG4-RD. Case 2 : A 48-year-old man collapsed at work. Computed tomography scan 1 week prior reported an ill-defined para-aortic retroperitoneal soft tissue density. No cardiac symptoms were reported in life. Post-mortem examination showed coronary arteritis and peri-arteritis with sclerosing peri-aortitis in the LAD. There was myocardial fibrosis of the anterior left ventricle and focal myocarditis of the right ventricle.
Discussion: IgG4-related disease presenting as sudden cardiac death without any preceding symptoms is very rare (six prior cases identified on literature review). Reported targeted successful interventions for intracoronary IgG4-RD diagnosed in life have included steroid therapy and B cell depleting therapy (i.e. rituximab). If cardiac symptoms are present in a patient with known IgG4-RD, cardiac investigations should be promptly arranged.
(© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology.)