Background:Coronary periarteritis is one of the clinical manifestations of IgG4-related disease. It can cause serious conditions such as angina and ruptured aneurysms. Therefore, it is important to recognize the clinical and radiological characteristics, which was little known.Objectives:We report four patients with IgG4-related coronary periarteritis with a systematic literature review.Methods:We identified four patients with IgG4-related coronary periarteritis at the St. Luke’s International Hospital in Tokyo, Japan from 2014 to 2020. A systematic literature review was conducted for English articles on IgG4-related coronary periarteritis cases with a full text or abstract available. We summarized patient demographics, IgG and IgG4 titers, the site and morphological type of coronary lesion, and other organ involvements.Results:Our 4 cases and 38 cases identified by the literature review were assessed. Coronary artery lesions were detected by a coronary CT in all but two cases. Wall thickening was the most common type of the lesion. Moreover, there were 32 (76.1%) patients with other organ involvements. The commonest other lesion was peri-aortitis in 21 (50.0%) patients. In cases with peri-aortitis, IgG and IgG4 titers were significantly higher than those without peri-aortitis (IgG4; 1540 [705.0, 2570.0] vs 246.0 [160.0, 536.3]; p = 0.001, IgG; 3596.5 [2838.3, 4260.0] vs 1779.0 [1288.3, 1992.8]; p =0.040). In addition, 15 (71.4%) patients of them had three or more IgG4 related organ involvements.Conclusion:Coronary CT was a useful imaging modality for the diagnosis of IgG4-related coronary periarteritis, and wall thickening was the most common lesion. Moreover, about half cases coexisted with peri-aortitis. Peri-aortitis and other organ involvements should be screened in those with higher IgG and IgG4.Table 1.Characteristics of our cases and the literature review cases.RCA: right coronary artery, LAD: left anterior descending artery, LCx: left circumflex arteryDisclosure of Interests:None declared