Yonsei Med J.  2013 Sep;54(5):1285-1288. 10.3349/ymj.2013.54.5.1285.

IgG4-Related Sclerosing Disease Involving the Superior Vena Cava and the Atrial Septum of the Heart

Affiliations
  • 1Division of Cardiology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
  • 2Department of Pathology, Yonsei University College of Medicine, Seoul, Korea. paxco@yuhs.ac
  • 3Department of Cardiothoracic Surgery, Yonsei University College of Medicine, Seoul, Korea.

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.

Keyword

IgG4-related sclerosing disease; sinoatrial node dysfunction; pacemaker

MeSH Terms

Atrial Septum/*pathology
Female
Humans
Immunoglobulin G/*blood
Middle Aged
Pacemaker, Artificial
Sclerosis/complications/diagnosis/therapy
Syncope/etiology
Vena Cava, Superior/*pathology
Immunoglobulin G

Figure

  • Fig. 1 (A) ECG taken on admission. First-degree AV block was observed with a PR interval of 240 ms. (B) Recovery phase during an exercise test. (C) Note the six-second sinus pause that was associated with dizziness. ECG, electrocardiogram; AV, atrioventricular.

  • Fig. 2 (Aa) Heart MRI showing a low-density mass lesion from the SVC and RA junction (left panel) to the right atrium of the heart (right panel). (Ab and Ac) Positron emission tomography-computed tomography showing increased FDG uptake at the SVC-RA junction and interartrial septum. (B) Heart multidetector computed tomography taken one year after diagnosis. No remarkable change of the mass. SVC, superior venacava; RA, right atrium; FDG, fluorodeoxyglucose.

  • Fig. 3 (A) Low-power view showing multifocal vague lymphoid follicle formations (H-E ×40). (B) High-power view showing dense extensive lymphoplasmacytic infiltrations (H-E ×200). (C) IgG4 immunohistochemical staining shows many positive plasma cells (IgG4, ×100). (D and E) More than 50 IgG4-positive plasma cells are observed (D, IgG4 ×400). The ratio of IgG4/IgG positive cells is greater than 40% (E, IgG ×400). (F) Trichrome staining does not show markedly abundant collagen deposition. (G and H) The immunohistochemical staining for CD20 and CD3 reveals a lot of T cell infiltration, mainly in paracortical area.


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