IgG4-related Disease with a Cardiac Mass

A 69-year-old man with palpitations and decreased blood pressure was referred. Echocardiography showed a mass in the right atrium and cardiac septum. The serum IgG4 level was 1,450 mg/dL. A biopsy of the cardiac mass showed fibrosis with inflammatory cells and increased IgG4-positive plasma cells an...

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Bibliographic Details
Published in:Internal Medicine 2020/05/01, Vol.59(9), pp.1203-1209
Main Authors: Matsumura, Ikuko, Mitsui, Takeki, Tahara, Kenichi, Shimizu, Hiroaki, Yanagisawa, Kunio, Ishizaki, Takuma, Koiso, Hiromi, Takizawa, Makiko, Yokohama, Akihiko, Saitoh, Takayuki, Hirato, Junko, Murakami, Hirokazu, Handa, Hiroshi, Tsukamoto, Norifumi
Format: Article
Language:English
Subjects:
Atrium
Biopsy
Blood pressure
cardiac mass
Case Report
Coronary artery disease
Echocardiography
Fibrosis
Flow cytometry
Heart diseases
IgG4-related disease
Immunoglobulin G
Inflammation
Internal medicine
Lymphocytes
Lymphoma
non-Hodgkin lymphoma
Plasma cells
Polymerase chain reaction
Septum
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Summary:A 69-year-old man with palpitations and decreased blood pressure was referred. Echocardiography showed a mass in the right atrium and cardiac septum. The serum IgG4 level was 1,450 mg/dL. A biopsy of the cardiac mass showed fibrosis with inflammatory cells and increased IgG4-positive plasma cells and lymphocytes. Flow cytometry and polymerase chain reaction of the immunoglobulin heavy chain did not demonstrate monoclonality. He was diagnosed with IgG4-related disease (IgG4-RD). IgG4-RD with a cardiac mass is rare and it is difficult to distinguish it from malignant lymphoma by a pathological examination alone. We therefore performed a biopsy and analyzed the clonality in order to make an accurate diagnosis of IgG4-RD.
ISSN:0918-2918
1349-7235
DOI:10.2169/internalmedicine.4054-19