Löffler Christian, Hoffend Johannes, Rebel Martin, Waldherr Rüdiger, Uppenkamp Michael, Bergner Raoul
Medizinische Klinik A, Klinikum Ludwigshafen, Ludwigshafen, Germany.
Klinik für diagnostische und interventionelle Radiologie, Klinikum Ludwigshafen, Ludwigshafen, Germany.
Clin Rheumatol. 2016 Jan;35(1):265-70. doi: 10.1007/s10067-014-2535-0. Epub 2014 Feb 23.
IgG4-related disease (IgG4-RD) are a group of autoinflammatory diseases often presenting as tumor-like lesions because of their infiltrative or mass forming behavior. They are characterized by a typical histology consisting of storiform fibrosis, high numbers of infiltrating IgG4-positive plasma cells, obliterative phlebitis, and a moderate presence of eosinophilic cells. Serum IgG4 levels can be elevated. We present a case of a 57 year-old male patient with immobilizing lower back pain, fever, and night sweats. We diagnosed IgG4-related periaortitis using serum IgG4 levels, abdominal ultrasound, PET/CT, and histology. We successfully treated the patient with glucocorticoids (GC) and azathioprine. Periaortitis is a rare presentation of IgG4-RD and therefore noteworthy. It has to be considered in patients with a retroperitoneal mass.
IgG4相关性疾病(IgG4-RD)是一组自身炎症性疾病,因其浸润性或肿块形成行为常表现为肿瘤样病变。其特征为典型的组织学表现,包括席纹状纤维化、大量浸润的IgG4阳性浆细胞、闭塞性静脉炎以及中等数量嗜酸性细胞的存在。血清IgG4水平可能升高。我们报告一例57岁男性患者,有固定性下背痛、发热和盗汗症状。我们通过血清IgG4水平、腹部超声、PET/CT及组织学检查诊断为IgG4相关性主动脉周炎。我们用糖皮质激素(GC)和硫唑嘌呤成功治疗了该患者。主动脉周炎是IgG4-RD的一种罕见表现,因此值得关注。对于有腹膜后肿块的患者必须考虑到这一疾病。