The Fingerland Department of Pathology, Charles University Faculty of Medicine and Faculty Hospital in Hradec Kralove, Hradec Kralove, Czech Republic.
Cardiovasc Pathol. 2011 Nov-Dec;20(6):352-60. doi: 10.1016/j.carpath.2010.09.003. Epub 2010 Oct 30.
Isolated thoracic aortitis (ITA) is diagnosed in a variable proportion of patients operated on for dilation/aneurysm of ascending aorta. The etiopathogenesis of ITA remains unclear. We studied 11 cases of ITA in order to determine the role of IgG4-mediated immune responses in its pathogenesis. The series included nine women and two men aged 52-79 years. All patients developed aortic incompetence due to dilation/aneurysm of ascending aorta. None of the patients had a history of IgG4-related disease neither did they develop features of such disease during the follow-up period. The microscopic findings included the presence of lymphoplasmacellular fibrosing infiltrate of varied intensity involving the adventitia and media of aorta. This inflammation was associated with severe medial elastic fiber defects. Obliterative phlebitis of the vasa vasorum was absent. Immunohistochemically, the inflammatory infiltrate comprised T- and B-lymphocytes as well as plasma cells. The plasma cell population was polyclonal with a predominance of IgG-producing cells. In all the cases, IgG4-producing plasma cells were detected. In five cases, the count exceeded 20 cells per high-power field. The IgG4/IgG ratio ranged from 0.07 to 0.98 (median 0.55). In six cases with the ratio >0.50, severe adventitial fibrosis was present. To the best of our knowledge, ours is the first study focused on investigating the role of IgG4-positive plasma cells in the development of ITA. Our results suggest that a subset of ITA may represent aortic manifestation of IgG4-related disease. Further research is necessary in order to clarify this issue.
孤立性胸主动脉炎（ITA）是在因升主动脉扩张/动脉瘤而行手术的患者中诊断出的，其在不同比例的患者中存在。ITA 的病因仍然不清楚。我们研究了 11 例 ITA，以确定 IgG4 介导的免疫反应在其发病机制中的作用。该系列包括 9 名女性和 2 名年龄在 52-79 岁的男性患者。所有患者均因升主动脉扩张/动脉瘤而出现主动脉功能不全。在随访期间，所有患者均无 IgG4 相关疾病的病史，也未出现此类疾病的特征。显微镜下发现，不同强度的淋巴浆细胞纤维性浸润累及主动脉的外膜和中膜。这种炎症与严重的中膜弹性纤维缺陷有关。血管外膜的闭塞性静脉炎不存在。免疫组化显示，炎症浸润包括 T 细胞和 B 细胞以及浆细胞。浆细胞群体为多克隆性，以产生 IgG 的细胞为主。在所有病例中，均检测到 IgG4 产生的浆细胞。在 5 例中，每高倍视野的细胞计数超过 20 个。IgG4/IgG 比值范围为 0.07-0.98（中位数 0.55）。在 6 例比值>0.50 的病例中，存在严重的外膜纤维化。据我们所知，我们的研究是第一个专注于研究 IgG4 阳性浆细胞在 ITA 发展中的作用的研究。我们的结果表明，一部分 ITA 可能代表 IgG4 相关疾病的主动脉表现。为了阐明这一问题，需要进一步研究。
