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IgG4-related Disease with a Cardiac Mass Causing Cerebral Infarction
Authors:Shun Nomura  Wataru Ishii  Ryosuke Hara  Shigeki Nanasawa  Kei Nishiwaki  Mitsuru Kagoshima  Tamaki Takano  Hidetoshi Satomi  Tatsuya Usui
Affiliation:1.Division of Rheumatology, Department of Internal Medicine, Nagano Red Cross Hospital, Japan; 2.Division of Neurology, Joetsu General Hospital, Japan; 3.Division of Cardiology, Joetsu General Hospital, Japan; 4.Department of Cardiovascular Surgery, Nagano Red Cross Hospital, Japan; 5.Department of Pathology, Nagano Red Cross Hospital, Japan; 6.Department of Cardiology, Nagano Red Cross Hospital, Japan
Abstract:Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by infiltration of extensive IgG4-positive plasma cells and lymphocytes. Although IgG4-RD has been observed in almost all organs, it rarely affects the myocardium. Cardiovascular lesions of IgG4-RD appear as aortic (aortic aneurysm and aortitis) and pericardial (constrictive pericarditis) lesions as well as pseudotumors around the coronary arteries. We herein report a case of IgG4-RD with a cardiac mass in the right atrium involving a sinus node. This condition caused arrhythmia and repeated strokes. We successfully treated the patient through resection of the cardiac mass, catheter ablation and immunosuppressive therapy.
Keywords:IgG4-related disease   cardiac mass   cerebral infarction
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