Kitahara Satoshi, Kataoka Yu, Fujino Yusuke
Department of Cardiology, Kashiwa Kousei General Hospital, 617 Shikoda, Kashiwa 277-0862, Japan.
Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Centre, 6-1, Kishibe-Shinmachi, Suita, Osaka 564-8565, Japan.
Eur Heart J Case Rep. 2025 May 28;9(6):ytaf271. doi: 10.1093/ehjcr/ytaf271. eCollection 2025 Jun.
Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated inflammatory disease that infrequently involves the coronary arteries. Given that pericoronary adipose tissue (PCAT) attenuation reflects the degree of inflammation in the coronary arteries, monitoring inflammation with PCAT may enable evaluation of disease activity in IgG4-related coronary periarteritis (CP).
A 58-year-old man with a history of IgG4-RD presented with ST-segment elevation myocardial infarction. Emergent coronary angiography revealed a severe stenotic lesion in the mid-segment of his left circumflex artery (LCX). Intravascular ultrasound (IVUS) imaging demonstrated thickening of the adventitia, and optical coherence tomography (OCT) showed the formation of vasa vasorum in the proximal segment of the LCX. Along with an elevated IgG4 level (1890 mg/dL), he was diagnosed with IgG4-related CP. Coronary computed tomography angiography (CCTA) after percutaneous coronary intervention (PCI) revealed soft tissue proliferation with elevated PCAT attenuation [PCAT attenuation = -68.4 Hounsfield units (HU)] around the proximal LCX. Following the initiation of prednisolone, the IgG4 level decreased to 239 mg/dL at 8 months post-PCI. Follow-up IVUS showed reduced adventitial thickness, and most of the previously observed vasa vasorum had disappeared on OCT. Furthermore, CCTA demonstrated a reduction in PCAT attenuation (to -81.8 HU), accompanied by a reduction in soft tissue volume.
In this case, serial PCAT analysis demonstrated resolution of inflammatory activity in response to prednisolone therapy. Serial PCAT imaging may have potential for evaluating disease activity and monitoring response to anti-inflammatory therapy in patients with IgG4-RD.
免疫球蛋白G4相关性疾病(IgG4-RD)是一种全身性免疫介导的炎症性疾病,很少累及冠状动脉。鉴于冠状动脉周围脂肪组织(PCAT)衰减反映冠状动脉炎症程度,通过PCAT监测炎症可能有助于评估IgG4相关性冠状动脉周围炎(CP)的疾病活动度。
一名有IgG4-RD病史的58岁男性因ST段抬高型心肌梗死就诊。急诊冠状动脉造影显示其左旋支动脉(LCX)中段有严重狭窄病变。血管内超声(IVUS)成像显示外膜增厚,光学相干断层扫描(OCT)显示LCX近端有滋养血管形成。结合IgG4水平升高(1890 mg/dL),他被诊断为IgG4相关性CP。经皮冠状动脉介入治疗(PCI)后行冠状动脉计算机断层扫描血管造影(CCTA)显示LCX近端周围有软组织增生,PCAT衰减升高[PCAT衰减=-68.4亨氏单位(HU)]。开始使用泼尼松龙后,PCI术后8个月时IgG4水平降至239 mg/dL。随访IVUS显示外膜厚度减小,OCT显示大部分先前观察到的滋养血管消失。此外,CCTA显示PCAT衰减降低(至-81.8 HU),同时软组织体积减小。
在本病例中,系列PCAT分析显示泼尼松龙治疗后炎症活动消退。系列PCAT成像可能具有评估IgG4-RD患者疾病活动度和监测抗炎治疗反应的潜力。