Clinical Rheumatology

, Volume 35, Issue 11, pp 2857–2864 | Cite as

Myocardial ischemia as presenting manifestation of IgG4-related disease: a case-based review

  • Guillermo Delgado-García
  • Sergio Sánchez-Salazar
  • Erick Rendón-Ramírez
  • Mario Castro-Medina
  • Bárbara Sáenz-Ibarra
  • Álvaro Barboza-Quintana
  • María Azalea Loredo-Alanis
  • David Hernández-Barajas
  • Dionicio Galarza-Delgado
Case Based Review


Coronary involvement in IgG4-related disease (IgG4-RD) has been scarcely reported, and myocardial ischemia as its presenting feature is even rarer. Here, we describe an additional case with novel and relevant observations. The patient was a previously healthy, middle-aged woman who presented to the clinic with new-onset typical angina. One tumefactive lesion encasing the left anterior descending artery was found during her workup. The most common underlying malignancies with secondary cardiac involvement were rationally ruled out. Symptoms persisted despite medical treatment, and she was therefore referred to surgery. Tumor excision was successfully performed, and she received coronary bypass grafting. IgG4-related coronary arteritis with pseudotumor formation was subsequently diagnosed following the comprehensive diagnostic criteria. This condition was clinically classified as active and circulating plasmablasts were found to be increased (5480/mL), even when these were determined 38 days after surgery. A PET/CT revealed an additional hypermetabolic lymph node. She was therefore treated with rituximab as induction therapy (two 1000 mg doses, administered 15 days apart). Three months later, her disease remained clinically inactive. Circulating plasmablasts were repeated and these had dropped to 0/mL. We thereafter review the current and pertinent literature on the topic, emphasizing the previous cases with similar presenting features (n = 7). We lastly suggest that IgG4-RD should be part of the differential diagnosis of any patient with tumefactive lesions surrounding the coronary arteries, since it can initially presented as sudden cardiac death.


Coronary arteritis Coronary artery bypass IgG4-related disease Plasmablast Rituximab 



The authors are pleased to acknowledge Prof. Yoh Zen (Kobe University Hospital, Japan) for his thought-provoking remarks.

Compliance with ethical standards




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Copyright information

© International League of Associations for Rheumatology (ILAR) 2016

Authors and Affiliations

  • Guillermo Delgado-García
    • 1
  • Sergio Sánchez-Salazar
    • 2
  • Erick Rendón-Ramírez
    • 2
  • Mario Castro-Medina
    • 3
  • Bárbara Sáenz-Ibarra
    • 4
  • Álvaro Barboza-Quintana
    • 4
  • María Azalea Loredo-Alanis
    • 5
  • David Hernández-Barajas
    • 6
  • Dionicio Galarza-Delgado
    • 7
  1. 1.Department of Internal Medicine, University HospitalAutonomous University of Nuevo LeónMonterreyMéxico
  2. 2.Division of Pulmonology and Intensive Care Unit, University HospitalAutonomous University of Nuevo LeónMonterreyMéxico
  3. 3.Division of Cardiovascular Surgery, University HospitalAutonomous University of Nuevo LeónMonterreyMéxico
  4. 4.Division of Anatomic Pathology, University HospitalAutonomous University of Nuevo LeónMonterreyMéxico
  5. 5.Division of Anatomic Pathology, National Medical Center “20 de Noviembre”Institute for Social Security and Services for State Workers (ISSSTE)Mexico CityMéxico
  6. 6.Division of Oncology, University HospitalAutonomous University of Nuevo LeónMonterreyMéxico
  7. 7.Division of Rheumatology, University HospitalAutonomous University of Nuevo LeónMonterreyMéxico

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