title: Sudden cardiac death due to coronary artery involvement by IgG4-related disease: a rare, serious complication of a rare disease.
abstract: Immunoglobulin G4-related disease (IgG4-RD) is a systemic disorder characterized by multiorgan fibrosis with IgG4-producing plasma cells, increased IgG4 serum concentration, and responsiveness to steroid therapy. Involvement of the pancreas, salivary glands, orbit, aorta, and other sites has been well documented in the literature; however, there have been limited reports of cases involving the coronary arteries. We report the case of a 53-year-old Hispanic man who was brought to the emergency center and diagnosed with sudden cardiac death. Autopsy was subsequently performed, revealing multiorgan involvement by IgG4-RD, including involvement of the coronary arteries. The inflammation and fibrosis, in combination with concomitant atherosclerotic disease, resulted in severe stenosis of the coronary arteries. Two of the coronary arteries were further occluded by thrombosis. These factors led to cardiac hypoperfusion, myocardial infarction and, ultimately, sudden cardiac death. Fatal involvement of the coronary arteries has not been previously reported, raising a new concern for a severe complication of IgG4-RD.
authors.affiliation: From the Department of Pathology & Immunology, Baylor College of Medicine, Houston, Texas (Drs Patel and Elghetany); Medical Examiner Service, Harris County Institute of Forensic Sciences, Houston, Texas (Dr Anzalone); the Department of Pathology & Laboratory Medicine, The University of Texas Health Science Center at Houston (Dr Buja); and the Department of Pathology, Texas Children's Hospital, Houston (Dr Elghetany).
authors.forename: Nimesh R
authors.initials: NR
authors.lastname: Patel
authors.forename: Mary L
authors.initials: ML
authors.lastname: Anzalone
authors.forename: L Maximilian
authors.initials: LM
authors.lastname: Buja
authors.forename: M Tarek
authors.initials: MT
authors.lastname: Elghetany
mesh_terms: D001167:Arteritis / Q000209:etiology / Q000276:immunology / Q000473:pathology; D023921:Coronary Stenosis / Q000209:etiology / Q000276:immunology / Q000473:pathology; D003331:Coronary Vessels / Q000276:immunology* / Q000473:pathology*; D016757:Death, Sudden, Cardiac / Q000209:etiology* / Q000473:pathology; D005355:Fibrosis; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications* / Q000276:immunology / Q000473:pathology; D007074:Immunoglobulin G / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D009203:Myocardial Infarction / Q000209:etiology / Q000276:immunology / Q000473:pathology; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology
chemical_list: D007074:Immunoglobulin G