托珠单抗单药治疗由特发性多中心Castleman病引起的IgG4相关性疾病样特征的有效性：一项基于病例的综述。

文献信息

中文摘要

背景： 特发性多中心Castleman病（iMCD）和免疫球蛋白G4相关性疾病（IgG4-RD）是不同的系统性炎症性疾病，可表现出重叠的临床表现，包括血清IgG4水平升高和IgG4阳性浆细胞的组织浸润。尽管白细胞介素-6（IL-6）抑制剂是iMCD既定的一线治疗，但对于具有IgG4-RD样特征的iMCD病例，最佳治疗策略仍未确立，特别是关于糖皮质激素是否必需。在此，我们报告一例具有IgG4-RD样特征的iMCD病例，经托珠单抗单药治疗成功。方法：一名68岁女性因复发性喉部肿瘤导致呼吸困难就诊，伴有肺实变、纵隔和腋窝淋巴结肿大、硬化性肠系膜炎和腹膜后纤维化。实验室评估显示C反应蛋白（CRP）和血清IgG4水平升高。尽管器官受累和IgG4水平升高提示IgG4-RD，且喉部和肺部标本显示IgG4阳性浆细胞浸润，但淋巴结的组织病理学检查证实了iMCD的诊断；因此，我们诊断该患者为具有IgG4-RD样特征的iMCD。托珠单抗单药治疗使CRP和IgG4水平迅速正常化，并改善了器官受累。患者在没有糖皮质激素的情况下获得了长期临床缓解。结果：我们的文献综述确定了9例先前报道的具有IgG4-RD样特征的iMCD病例。9例中有8例对初始糖皮质激素单药治疗反应不足，随后需要加用IL-6靶向药物才能获得疾病缓解。结论：结合我们文献综述的发现，我们的病例提示托珠单抗单药治疗可能是由iMCD引起的IgG4-RD样特征的潜在治疗选择。

英文摘要

Idiopathic multicentric Castleman's disease (iMCD) and immunoglobulin G4-related disease (IgG4-RD) are distinct systemic inflammatory diseases that can exhibit overlapping clinical manifestations, including elevated serum IgG4 levels and tissue infiltration of IgG4-positive plasma cells. Although interleukin-6 (IL-6) inhibitors are established first-line therapies for iMCD, optimal treatment strategies for cases of iMCD with IgG4-RD-like features remain unestablished, particularly regarding whether glucocorticoids are essential or not. Herein, we report our case of iMCD with IgG4-RD-like features successfully treated with tocilizumab monotherapy. A 68-year-old female presented with dyspnea due to a recurrent laryngeal tumor, accompanied by pulmonary consolidation, mediastinal and axillary lymphadenopathy, sclerosing mesenteritis, and retroperitoneal fibrosis. Laboratory evaluation revealed elevated C-reactive protein (CRP) and serum IgG4 levels. Although the organ involvement and elevated IgG4 levels were suggestive of IgG4-RD, and laryngeal and pulmonary specimens exhibited IgG4-positive plasma cell infiltration, histopathological examination of lymph node confirmed the diagnosis of iMCD; therefore, we diagnosed the patient with iMCD with IgG4-RD-like features. Tocilizumab monotherapy achieved rapid normalization of CRP and IgG4 levels, along with improvement of organ involvements. The patient attained long-term clinical remission without glucocorticoids. Our literature review identified 9 previously reported cases of iMCD with IgG4-RD-like features. Eight of nine cases showed an insufficient response to initial glucocorticoid monotherapy and subsequently required the addition of IL-6 targeting agents to obtain disease resolution. Together with the findings from our literature review, our case suggests that tocilizumab monotherapy may be a potential treatment option for IgG4-RD-like features caused by iMCD.