Giant Tumorous Legions Surrounding the Right Coronary Artery Associated with Immunoglobulin-G4-Related Systemic Disease

Abstract

Immunoglobulin G4 (IgG4)-related systemic disease was first recognized as a clinicopathological entity about 10 years ago, and since then, it has attracted growing attention. It is an autoimmune disease which affects multiple organs including the pancreas, bile duct, salivary glands and retroperitoneum. Further, it was recently reported that it can be manifested as periarteritis, often as inflammatory abdominal aortic aneurysm. We describe the case of a 75-year-old man with autoimmune pancreatitis and parotitis who presented with angina. The serum concentration of IgG4 was significantly increased at 2,510 mg/dl. Coronary angiography showed multiple stenotic lesions and pronounced dilatation of the right coronary artery. Cardiac computed tomography disclosed increased wall thickness of the coronary arteries and focal tumorous lesions surrounding the right coronary artery. Treatment with steroids proved only marginally effective and he underwent surgical resection of the aneurysm and coronary artery bypass grafting. The diagnosis of IgG4-related systemic disease was confirmed by histological examination of the resected mass, which showed a massive infiltration of IgG4-positive plasma cells. This case emphasizes the importance of considering the diagnosis in any patient with abnormally increased wall thickness or ectatic lesions in the coronary arteries.

Immunoglobulin G4-Related Cardiovascular Diseases

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] DelgadoGarcia and colleagues presented a case of tumorous formation encasing the left anterior descending artery. 1) Tumor resection and coronary bypass grafting relieved the myocardial ischemia.…”

Sudden Cardiac Death Due to Coronary Artery Involvement by IgG4-Related Disease: A Rare, Serious Complication of a Rare Disease

“…This appears to be the first reported case of a patient with IgG4-related coronary periarteritis and active coronary ischemia. Subsequently, Ikutomi et al 15 reported the case of a patient with IgG4-RD who presented with angina and was found to have stenotic lesions of the left anterior descending and left circumflex arteries with dilatation of the RCA. This patient also had increased wall thickness of the coronary arteries with tumorous lesions surrounding the RCA.…”

IgG4-Related Cardiovascular Disorders

“…IgG4-related IAAAs are estimated to be 5% of surgical AAA and 50% of total IAAA. 66) IgG4-related inflammatory aneurysms are also reported in other large to medium-sized arteries, such as the aortic arch, 18) thoracic aorta, 67) coronary artery, 26) and other arteries. 68) Solitary aneurysm is a relatively common lesion in IgG4 related disease.…”

“…5) In 2001, Type 1 AIP with elevated serum IgG4 level and abundant IgG4-positive lymphoplasmacyte infiltration in the pancreas was reported and the concept of IgG4-related disease was proposed. 6) Then, it was gradually discovered that IgG4-related disease involves inflammation and sclerosing in many other organs, 7) especially exocrine organs: 8) bile ducts, 9) lacrimal and salivary glands, 10) retroperitoneum, 11) urinary organs, [12][13][14] pituitary gland, 15) prostate, 16) arteries, [17][18][19][20] veins, [21][22][23] heart, [24][25][26] lung, 25,27,28) eye, 24,29) mesenterium, 30) mammary gland, 31) central nervous system, 32) esophagus, 33) stomach, 34) liver, 35) gastrointestinal tract, 36) thyroid, 37,38) nose, 39) blood, 40,41) skin, 42) and pseudotumors in various organs 9,14,25,[43][44]…”