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IgG4-related Disease with a Cardiac Mass

Ikuko Matsumura1, Takeki Mitsui1, Kenichi Tahara1

  • 1Department of Hematology, Gunma University Graduate School of Medicine, Japan.

Internal Medicine (Tokyo, Japan)
|January 21, 2020
PubMed

Insights

This study details a rare case of IgG4-related disease (IgG4-RD) presenting as a cardiac mass. Diagnosis required biopsy and clonality analysis to differentiate from lymphoma.

Area of Science:

  • Cardiology
  • Immunology
  • Pathology

Background:

  • Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition.
  • Cardiac involvement in IgG4-RD is uncommon and can mimic other cardiac pathologies.

Observation:

  • A 69-year-old male presented with palpitations and hypotension.
  • Echocardiography revealed a cardiac mass in the right atrium and septum.
  • Elevated serum IgG4 levels (1,450 mg/dL) were noted.

Findings:

  • Cardiac mass biopsy showed fibrosis, inflammation, and increased IgG4-positive plasma cells and lymphocytes.
  • Flow cytometry and PCR for immunoglobulin heavy chain did not show monoclonality.
  • Diagnosis of IgG4-related disease was confirmed.

Implications:

  • IgG4-RD cardiac masses are rare and challenging to distinguish from malignant lymphoma pathologically.
  • Biopsy and clonality analysis are crucial for accurate diagnosis of IgG4-RD in cardiac lesions.
  • This case highlights the importance of considering IgG4-RD in cardiac masses with elevated IgG4 levels.

Keywords:
IgG4-related diseasecardiac massnon-Hodgkin lymphoma

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