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Constrictive pericarditis as first presentation of IgG4-related disease: Pitfalls and pearls

Timothy N Kwan1, Gemma Kwan2, John Yiannikas1

  • 1Department of Cardiology, Concord Hospital, The University of Sydney, Concord, NSW, Australia.

Modern Rheumatology Case Reports
|December 26, 2024
PubMed

Insights

Immunoglobulin G4-related disease (IgG4-RD) can present with cardiac issues like pericardial disease. Delayed diagnosis due to under-recognition of these cardiac signs risks patient safety and necessitates timely identification.

Area of Science:

  • Cardiology
  • Rheumatology
  • Immunology

Background:

  • Immunoglobulin G4-related disease (IgG4-RD) is a multisystem inflammatory condition with diverse clinical presentations.
  • Cardiac involvement in IgG4-RD is recognized but often overlooked and not included in diagnostic criteria.
  • Pericardial disease is a common cardiac manifestation of IgG4-RD.

Purpose of the Study:

  • To highlight pericardial disease as an initial presentation of IgG4-related disease.
  • To emphasize the diagnostic challenges posed by under-recognized cardiac manifestations of IgG4-RD.
  • To underscore the importance of considering IgG4-RD in patients with unexplained pericardial disease.

Main Methods:

  • Case report detailing a patient with IgG4-related disease presenting with pericardial disease.
  • Review of literature regarding cardiac manifestations of IgG4-RD.
  • Discussion of diagnostic delays and potential risks.

Main Results:

  • The case illustrates pericardial disease as the primary manifestation leading to delayed IgG4-RD diagnosis.
  • Diagnostic delay exposed the patient to risks associated with invasive procedures like pericardiectomy.
  • Early recognition of cardiac IgG4-RD could prevent misdiagnosis and unnecessary interventions.

Conclusions:

  • Cardiac manifestations, particularly pericardial disease, are crucial presentations of IgG4-related disease.
  • Increased awareness and inclusion of cardiac findings in diagnostic criteria for IgG4-RD are warranted.
  • Timely diagnosis of IgG4-RD is essential to avoid complications and guide appropriate management.

Keywords:
IgG4-related diseaseautoimmunitypericarditis

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