A suspected case of coronary periarteritis due to IgG4-related disease as a cause of ischemic heart disease

Forensic Sci Med Pathol. 2014 Mar;10(1):103-8. doi: 10.1007/s12024-013-9516-5. Epub 2013 Dec 12.


IgG4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by marked infiltration of IgG4-positive (+) plasma cells into affected organs, but the concept of this disease has only recently been established. Coronary vasculitis is a rare disease that can cause sudden death, and it has recently been reported that IgG4-RD may be associated with vasculitis, including periarteritis and coronary disease. In this paper we report an autopsy case of sudden death of a man in his thirties, in which coronary periarteritis with features of IgG4-related periarteritis was detected. IgG4-RD was suspected from the presence of the following histopathological features: (1) markedly thickened adventitia and marked infiltration of the adventitia and periarterial fat by lymphocytes and plasma cells; and (2) infiltration of IgG4-positive plasma cells (ratio of IgG4+ cells to IgG4+ cells of >40 %, 50 IgG4+ plasma cells per high-power field) on immunostaining. The etiology and pathophysiology of IgG4-RD and IgG4-related periarteritis are still unclear, and further investigation of these conditions and their association with coronary lesions is needed. Careful consideration should be given to the possible presence of IgG4-RD when forensic pathologists encounter cases of sudden death accompanied by coronary periarteritis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Arteritis / diagnostic imaging
  • Arteritis / etiology*
  • Arteritis / immunology
  • Arteritis / pathology
  • Autopsy
  • Biomarkers / analysis
  • Cause of Death
  • Coronary Angiography / methods
  • Coronary Artery Disease / diagnostic imaging
  • Coronary Artery Disease / etiology*
  • Coronary Artery Disease / immunology
  • Coronary Artery Disease / pathology
  • Coronary Vessels / diagnostic imaging
  • Coronary Vessels / immunology*
  • Coronary Vessels / pathology
  • Death, Sudden, Cardiac / etiology*
  • Death, Sudden, Cardiac / pathology
  • Fatal Outcome
  • Forensic Pathology / methods
  • Humans
  • Immunoglobulin G / analysis*
  • Inflammation / complications*
  • Inflammation / immunology
  • Male
  • Tomography, X-Ray Computed


  • Biomarkers
  • Immunoglobulin G