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

Elizabeth D Paratz; Laura Ross; Dominica Zentner; Natalie Morgan; Heinrich Bouwer; Matthew Lynch; Sarah Parsons; Andre La Gerche

doi:10.1093/ehjcr/ytac050

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

Eur Heart J Case Rep. 2022 Feb 3;6(2):ytac050. doi: 10.1093/ehjcr/ytac050. eCollection 2022 Feb.

Authors

Elizabeth D Paratz^{1

2

3}, Laura Ross^{3

4

5}, Dominica Zentner^{6

7}, Natalie Morgan⁸, Heinrich Bouwer^{8

9}, Matthew Lynch^{8

9}, Sarah Parsons^{8

9}, Andre La Gerche^{1

2

3}

Affiliations

¹ Department of Cardiology, Baker Heart and Diabetes Institute, 75 Commercial Road, Prahran, VIC 3181, Australia.
² Department of Cardiology, Alfred Hospital, 55 Commercial Road, Prahran, VIC 3181, Australia.
³ Department of Cardiology, St Vincent's Hospital Melbourne, 41 Victoria Pde, Fitzroy. VIC 3065, Australia.
⁴ Department of Rheumatology, St Vincent's Hospital Melbourne, 41 Victoria Pde, Fitzroy, VIC 3065, Australia.
⁵ Department of Medicine, The University of Melbourne at St Vincent's, 41 Victoria Pde, Fitzroy, VIC 3065, Australia.
⁶ Department of Cardiology, Royal Melbourne Hospital, 300 Grattan Street, Parkville, VIC 3050, Australia.
⁷ Department of Cardiology, Royal Melbourne Hospital Clinical School, Faculty of Medicine, Dentistry and Health Sciences, University of Melbourne, Melbourne, VIC 3000, Australia.
⁸ Victorian Institute of Forensic Medicine, 65 Kavanagh Street, Southbank, VIC 3006, Australia.
⁹ Department of Forensic Medicine, Monash University, 65 Kavanagh Street, Southbank, VIC 3006, Australia.

Abstract

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.

Keywords: Case report; Coronary artery disease; IgG4-related disease; Post-mortem; Sudden cardiac death.

Publication types

Case Reports