Department of Cardiology, Osaka Medical College, Takatsuki-shi, Daigaku-machi 2-7, Osaka 569-8686, Japan.
J Cardiol. 2012 Mar;59(2):132-8. doi: 10.1016/j.jjcc.2011.10.005. Epub 2011 Dec 3.
The cardiovascular system may be involved as a target organ of multifocal fibrosclerosis, which may manifest as idiopathic retroperitoneal fibrosis, inflammatory aortic aneurysm, inflammatory periarteritis, and inflammatory pericarditis. These pathological conditions can sometimes occur concomitantly. Idiopathic retroperitoneal fibrosis and inflammatory abdominal aortic aneurysm are both characterized by the presence of fibro-inflammatory tissue around the abdominal aorta expanding into the surrounding retroperitoneal structures, and together they may be termed 'chronic periaortitis'. Cardiovascular fibrosclerosis has become non-uncommonly encountered condition since imaging modalities have made its diagnosis more feasible. In addition, recent studies have demonstrated that a certain fraction, but not all, of cardiovascular fibrosclerosis may have a link with immunoglobulin-G4 (IgG4)-related sclerosing disease (IgG4-SD). IgG4-SD is histologically characterized by dense fibrosclerosis and infiltration of lymphocytes and IgG4-positive plasma cells, and these histopathologic findings seem to be essentially similar regardless of the organs involved. In this mini review, we summarize what is known so far about multifocal fibrosclerosis of the cardiovascular system and its association with IgG4-SD, and what remains to be clarified in future investigations.
心血管系统可能是多灶性纤维性硬化症的靶器官，其表现为特发性腹膜后纤维化、炎性主动脉瘤、炎性动脉周围炎和炎性心包炎。这些病理情况有时可同时发生。特发性腹膜后纤维化和炎性腹主动脉瘤的特征均为纤维炎症组织围绕腹主动脉扩张至周围腹膜后结构，因此两者可被称为“慢性大动脉炎”。由于影像学使诊断变得更加可行，心血管纤维化已成为不常见的情况。此外，最近的研究表明，心血管纤维化的一定比例（但非全部）可能与免疫球蛋白 G4（IgG4）相关的硬化性疾病（IgG4-SD）有关。IgG4-SD 的组织学特征为致密纤维化和淋巴细胞浸润以及 IgG4 阳性浆细胞浸润，无论受累器官如何，这些组织病理学发现似乎基本相似。在这篇小型综述中，我们总结了目前已知的关于心血管系统多灶性纤维性硬化症及其与 IgG4-SD 的关联，以及未来研究中需要阐明的问题。
