Zen Yoh, Onodera Manabu, Inoue Dai, Kitao Azusa, Matsui Osamu, Nohara Takahiro, Namiki Mikio, Kasashima Satomi, Kawashima Atsuhiro, Matsumoto Yasushi, Katayanagi Kazuyoshi, Murata Tetsuya, Ishizawa Shin, Hosaka Noriko, Kuriki Ken, Nakanuma Yasuni
Institute of Liver Studies, King's College Hospital, Denmark Hill, London SE5 9RS, UK.
Am J Surg Pathol. 2009 Dec;33(12):1833-9. doi: 10.1097/pas.0b013e3181b72882.
The possible involvement of immunoglobulin G4 (IgG4) in the pathogenesis of idiopathic sclerosing lesions has been suggested. In this study, a clinicopathologic analysis was performed to reveal characteristics of retroperitoneal fibrosis relating to IgG4. The study involved 17 patients with retroperitoneal fibrosis. Immunohistochemistry revealed numerous IgG4-positive plasma cell infiltrates in 10 cases (IgG4-related), but only a few positive cells in 7 cases (non-IgG4-related). All patients with IgG4-related retroperitoneal fibrosis were male, whereas all except 1 with unrelated lesions were female. Histologically, eosinophilic infiltration (>5 cells per high-power field) and obliterative phlebitis were commonly observed in IgG4-related lesions. Serologically, serum IgG and IgG4 concentrations were significantly higher in the IgG4-related cases, with the IgG4 concentrations all over 135 mg/dL (the upper limit of the normal range). Steroid therapy was performed in 13 cases, and was effective irrespective of IgG4. Three patients had recurrence during the follow up. Five of 10 IgG4-related cases had sclerosing lesions at other sites. The only tests that reliably distinguish the 2 groups were serum IgG4 levels or IgG4/IgG ratio in the plasma cells in a tissue biopsy. The only major clinical difference was the striking male predominance in IgG4-related cases. In conclusion, this study revealed that retroperitoneal fibrosis could be classified as IgG4-related or not. This distinction seems important to help better characterize the biology/pathogenesis of both groups and better predict the possibility of other IgG4-related processes at other anatomic sites.
免疫球蛋白G4（IgG4）可能参与特发性硬化性病变的发病机制，这一点已被提出。在本研究中，进行了临床病理分析以揭示与IgG4相关的腹膜后纤维化的特征。该研究纳入了17例腹膜后纤维化患者。免疫组织化学显示，10例（IgG4相关）有大量IgG4阳性浆细胞浸润，而7例（非IgG4相关）仅有少数阳性细胞。所有IgG4相关的腹膜后纤维化患者均为男性，而除1例无关病变患者外，其余均为女性。组织学上，IgG4相关病变中常见嗜酸性粒细胞浸润（每高倍视野>5个细胞）和闭塞性静脉炎。血清学方面，IgG4相关病例的血清IgG和IgG4浓度显著更高，IgG4浓度均超过135mg/dL（正常范围上限）。13例患者接受了类固醇治疗，无论IgG4情况如何均有效。3例患者在随访期间复发。10例IgG4相关病例中有5例在其他部位有硬化性病变。唯一能可靠区分这两组的检查是组织活检中浆细胞的血清IgG4水平或IgG4/IgG比值。唯一主要的临床差异是IgG4相关病例中男性明显占优势。总之，本研究表明腹膜后纤维化可分为IgG4相关或非IgG4相关。这种区分对于更好地描述两组的生物学/发病机制以及更好地预测其他解剖部位发生其他IgG4相关病变的可能性似乎很重要。
