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Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disorder characterized by elevated serum IgG4 levels and the enlargement and fibrosis of organs. As a rare manifestation, coronary arteries can be affected by IgG4-RD as coronary periarteritis, leading to serious complications such as stenosis or aneurysm. Although coronary periarteritis poses a life-threatening condition, optimal treatment strategies remain unclear due to its extreme rarity.

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Presented case study deals with the sudden death of a 47 years old male, shortly after a mountain bike race after reported nausea and chest pain followed by loss of consciousness and resuscitation. Cardiopulmonary resuscitation was unsuccessful. An autopsy was enacted due to the sudden death in a seemingly healthy person.

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