IgG4-Related Sclerosing Disease Involving the Superior Vena Cava and the Atrial Septum of the Heart
A 55-year-old woman presented with frequent episodes of syncope due to sinus pauses. During ambulatory Holter monitoring, atrial fibrillation and first-degree atrioventricular nodal block were observed. Magnetic resonance imaging and CT scans showed a tumor-like mass from the superior vena cava to t...
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          | Published in | Yonsei medical journal Vol. 54; no. 5; pp. 1285 - 1288 | 
|---|---|
| Main Authors | , , , , | 
| Format | Journal Article | 
| Language | English | 
| Published | 
        Korea (South)
          Yonsei University College of Medicine
    
        01.09.2013
     연세대학교의과대학  | 
| Subjects | |
| Online Access | Get full text | 
| ISSN | 0513-5796 1976-2437 1976-2437  | 
| DOI | 10.3349/ymj.2013.54.5.1285 | 
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| Abstract | A 55-year-old woman presented with frequent episodes of syncope due to sinus pauses. During ambulatory Holter monitoring, atrial fibrillation and first-degree atrioventricular nodal block were observed. Magnetic resonance imaging and CT scans showed a tumor-like mass from the superior vena cava to the right atrial septum. Open chest cardiac biopsy was performed. The tumor was composed of proliferating IgG4-positive plasma cells and lymphocytes with surrounding sclerosis. The patient was diagnosed with IgG4-related sclerosing disease. Because of frequent sinus pauses and syncope, a permanent pacemaker was implanted. The cardiac mass was inoperable, but it did not progress during the one-year follow-up. | 
    
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| AbstractList | A 55-year-old woman presented with frequent episodes of syncope due to sinus pauses. During ambulatory Holter monitoring, atrial fibrillation and first-degree atrioventricular nodal block were observed. Magnetic resonance imaging and CT scans showed a tumor-like mass from the superior vena cava to the right atrial septum. Open chest cardiac biopsy was performed. The tumor was composed of proliferating IgG4-positive plasma cells and lymphocytes with surrounding sclerosis. The patient was diagnosed with IgG4-related sclerosing disease. Because of frequent sinus pauses and syncope, a permanent pacemaker was implanted. The cardiac mass was inoperable, but it did not progress during the one-year follow-up. A 55-year-old woman presented with frequent episodes of syncope due to sinus pauses. During ambulatory Holter monitoring, atrial fibrillation and first-degree atrioventricular nodal block were observed. Magnetic resonance imaging and CT scans showed a tumor-like mass from the superior vena cava to the right atrial septum. Open chest cardiac biopsy was performed. The tumor was composed of proliferating IgG4-positive plasma cells and lymphocytes with surrounding sclerosis. The patient was diagnosed with IgG4-related sclerosing disease. Because of frequent sinus pauses and syncope, a permanent pacemaker was implanted. The cardiac mass was inoperable, but it did not progress during the one-year follow-up.A 55-year-old woman presented with frequent episodes of syncope due to sinus pauses. During ambulatory Holter monitoring, atrial fibrillation and first-degree atrioventricular nodal block were observed. Magnetic resonance imaging and CT scans showed a tumor-like mass from the superior vena cava to the right atrial septum. Open chest cardiac biopsy was performed. The tumor was composed of proliferating IgG4-positive plasma cells and lymphocytes with surrounding sclerosis. The patient was diagnosed with IgG4-related sclerosing disease. Because of frequent sinus pauses and syncope, a permanent pacemaker was implanted. The cardiac mass was inoperable, but it did not progress during the one-year follow-up. A 55-year-old woman presented with frequent episodes of syncope due to sinus pauses. During ambulatory Holter monitoring, atrial fibrillation and first-degree atrioventricular nodal block were observed. Magnetic resonance imaging and CT scans showed a tumor-like mass from the superior vena cava to the right atrial septum. Open chest cardiac biopsy was performed. The tumor was composed of proliferating IgG4-positive plasma cells and lymphocytes with surrounding sclerosis. The patient was diagnosed with IgG4-related sclerosing disease. Because of frequent sinus pauses and syncope, a permanent pacemaker was implanted. The cardiac mass was inoperable, but it did not progress during the one-year follow-up. KCI Citation Count: 9  | 
    
| Author | Yoon, Yong-Nam Kim, Se Hoon Song, Changho Koh, Myoung Ju Joung, Boyoung  | 
    
| AuthorAffiliation | 2 Department of Pathology, Yonsei University College of Medicine, Seoul, Korea 3 Department of Cardiothoracic Surgery, Yonsei University College of Medicine, Seoul, Korea 1 Division of Cardiology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea  | 
    
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| Cites_doi | 10.2169/internalmedicine.45.1601 10.1016/j.jjcc.2011.10.005 10.1046/j.1563-258X.2002.02031.x 10.1080/00365520410008033 10.1186/1741-7015-4-23 10.1080/00365520701731263 10.1056/NEJM200103083441005 10.1148/radiol.11102250 10.1016/j.jjcc.2011.07.014 10.1159/000085277 10.1097/PAP.0b013e3181ee63ce  | 
    
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| Keywords | IgG4-related sclerosing disease sinoatrial node dysfunction pacemaker  | 
    
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| SubjectTerms | Atrial Septum - pathology Case Report Female Humans Immunoglobulin G - blood Middle Aged Pacemaker, Artificial Sclerosis - complications Sclerosis - diagnosis Sclerosis - therapy Syncope - etiology Vena Cava, Superior - pathology 의학일반  | 
    
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| Title | IgG4-Related Sclerosing Disease Involving the Superior Vena Cava and the Atrial Septum of the Heart | 
    
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