Cao Xiaoyu, Li Shaoxiang, Wan Jin, Yu Zhibo, Dong Gehong, Zhou Wei
Department of Rheumatology and Immunology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Clin Exp Rheumatol. 2024 Sep;42(9):1736-1743. doi: 10.55563/clinexprheumatol/61mt03. Epub 2024 Mar 26.
To explore the effectiveness of tofacitinib for immunoglobulin G4-related disease (IgG4-RD) and idiopathic retroperitoneal fibrosis (IRF), and investigate the expression of JAKs in the lesion of these diseases.
Clinical data of patients with IgG4-RD or IRF who were administered with tofacitinib monotherapy were collected. IgG4-RD responder index (IgG4-RD RI) was assessed. The expression of JAK1, JAK2, JAK3, and TYK2 were analysed with immunohistochemistry staining in three salivary glands specimens of IgG4-RD and one retroperitoneal tissue of IRF.
Two patients with IRF and two patients with IgG4-RD used tofacitinib monotherapy. Two patients with IRF achieved complete remission with diminished retroperitoneal mass and decreased CRP, as IgG4-RD RI decreased from 6 to 1 in both of them. One with IgG4-RD achieved complete remission with alleviated enlargement of pancreas and IgG4 level decreased from 13.7 g/L to 2.4 g/L, as IgG4-RD RI decreased from 12 to 1. One with IgG4-RD achieved partial response with IgG4 level decreased from 77.1g/L to 25.8g/L as IgG4-RD RI from 18 to 6. JAK1, JAK2, JAK3, and TYK2 expression were detected in biopsy tissues. The staining intensity of the JAK family on the lesion from one IRF patient was similar to those from IgG4-RD patients.
Tofacitinib is a potentially effective treatment for IgG4-RD and IRF and it is reasonable to conduct clinical trial to validate its efficacy. The JAKs were expressed in the inflammatory lesions of IgG4-RD and IRF and they may share a common pathogenesis pathway that is independent of IgG4 production.
探讨托法替布治疗免疫球蛋白G4相关性疾病（IgG4-RD）和特发性腹膜后纤维化（IRF）的有效性，并研究这些疾病病变中JAKs的表达情况。
收集接受托法替布单药治疗的IgG4-RD或IRF患者的临床资料。评估IgG4-RD反应指数（IgG4-RD RI）。采用免疫组织化学染色分析IgG4-RD的三个唾液腺标本和IRF的一个腹膜后组织中JAK1、JAK2、JAK3和TYK2的表达。
2例IRF患者和2例IgG4-RD患者接受托法替布单药治疗。2例IRF患者实现完全缓解，腹膜后肿块缩小，CRP降低，他们的IgG4-RD RI均从6降至1。1例IgG4-RD患者实现完全缓解，胰腺肿大缓解，IgG4水平从13.7 g/L降至2.4 g/L，IgG4-RD RI从12降至1。1例IgG4-RD患者实现部分缓解，IgG4水平从77.1g/L降至25.8g/L，IgG4-RD RI从18降至6。活检组织中检测到JAK1、JAK2、JAK3和TYK2表达。1例IRF患者病变处JAK家族的染色强度与IgG4-RD患者的相似。
托法替布对IgG4-RD和IRF可能是一种有效的治疗方法，进行临床试验验证其疗效是合理的。JAKs在IgG4-RD和IRF的炎性病变中表达，它们可能共享一条独立于IgG4产生的共同发病机制途径。
