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

Abstract

Immunoglobulin G4–related disease (IgG4-RD) is a systemic disorder characterized by multiorgan fibrosis with IgG4-producing plasma cells, increased IgG4 serum concentration, and responsiveness to steroid therapy. Involvement of the pancreas, salivary glands, orbit, aorta, and other sites has been well documented in the literature; however, there have been limited reports of cases involving the coronary arteries. We report the case of a 53-year-old Hispanic man who was brought to the emergency center and diagnosed with sudden cardiac death. Autopsy was subsequently performed, revealing multiorgan involvement by IgG4-RD, including involvement of the coronary arteries. The inflammation and fibrosis, in combination with concomitant atherosclerotic disease, resulted in severe stenosis of the coronary arteries. Two of the coronary arteries were further occluded by thrombosis. These factors led to cardiac hypoperfusion, myocardial infarction and, ultimately, sudden cardiac death. Fatal involvement of the coronary arteries has not been previously reported, raising a new concern for a severe complication of IgG4-RD.

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.…”

In Reply

“…Coronary artery involvement of IgG4-related disease has been demonstrated in several previous case reports: coronary artery luminal narrowing, presumably of an atherosclerotic nature, may occur at the site of a coronary artery surrounded by thickened or tumefactive adventitia with IgG4-positve lymphocytic infiltration, 2 as in the case described by Patel et al 1 On the other hand, several case reports have demonstrated only mild coronary artery luminal narrowing, regardless of the marked adventitial IgG4-positive cell infiltration. 3,4 The condition that was documented by Patel et al might be termed IgG4-related coronary artery disease; on the other hand, it should be noted that there have been no specific criteria for diagnosing IgG4-related coronary artery disease or IgG4-related cardiac disease until now.…”

“…In Reply.-We thank Drs Ishizaka, Tanigawa, and Suzuki for their interest in our recent article describing a case of sudden cardiac death in a man with concomitant coronary artery disease and IgG4-related disease (IgG4-RD). 1 The authors raise several concerns, and we thank them for the opportunity to further explain our findings. First, the authors of the letter state, ''The condition that was documented by Patel et al might be termed IgG4-related coronary artery disease.''…”

“…According to a previous report by Tanigawa et al 2 and the report by Patel et al, 1 there is no doubt that the cardiovascular system is a target of the multiorgan involvement of IgG4-related disease, and IgG4-related coronary artery disease may lead to a fatal outcome. On the other hand, considering that IgG4 may counteract the overactivated inflammatory process and that the severe IgG4-related pericoronary phenomenon is not necessarily accompanied by coronary artery stenosis or occlusion, we may need to further assess whether IgG4-related pathological conditions enhanced or, in reverse, ameliorated the coronary artery luminal stenosis and occlusion observed in their case.…”

“…In terms of the role of IgG4-positive lymphocytes in coronary atherogenesis, it would be of relevance to determine whether the thickened intima was positive for infiltration of such cells in the case of Patel et al 1 In general, B and T cells may reside in adventitial regions, but T cells (but not B cells), as well as monocytes/macrophages, infiltrate the inflamed intimal lesions.…”