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

Abstract

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.

IgG4-related Cardiovascular Disease from the Aorta to the Coronary Arteries: Multidetector CT and PET/CT

“…Smaller aortic vessels such as the coronary arteries and pericardial involvement also affect the prognosis. Although myocardial ischemia due to coronary artery involvement can be fatal, only a few case series have been reported (13)(14)(15)(16)(17). Because IgG4related disease usually shows a marked response to corticosteroid therapy, radiologists should be familiar with the imaging findings of IgG4related CVD to avoid a delay in the diagnosis and unnecessary surgical interventions (18).…”

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. Treacy and colleagues reported three autopsy cases in which firm white whorled masses, ranging in diameter from 9 to 25 mm, surrounded both the right and left coronary arteries.…”

The Utility of FDG PET/CT in IgG4-Related Disease with a Focus on Coronary Artery Involvement

“…Interestingly, although our numbers are small, we had a relatively large proportion (three out of 12) of coronary artery involvement. We are adding a small case series to the growing body of evidence of coronary involvement in IgG4-RD [4][5][6][7][8][9].…”