Two cases of refractory IgG4-related disease successfully treated with tocilizumab

Ong, Jeremy; Brown, S.; Gilbertson, Michael; Grigoriadis, George; Simpson, Ian; Vilcassim, Shahla; Löw, Michael; Fedele, Pasquale; Motorna, Olga; Roberts, Lynden; Gingold, Michael; Gregory, Gareth P.

doi:10.1007/s00277-022-04787-x

Cited by 3 publications

(3 citation statements)

References 8 publications

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“…Hence, Khan et al indicated that JAK was a promising therapeutic target in IgG4-RD (8). In two patients, tocilizumab, an IL-6 receptor antibody, was reported to control active IgG4-RD refractory to RTX (10). Here, we reported four patients with IgG4-RD or IRF who improved remarkably after tofacitinib monotherapy.…”

Section: Introductionmentioning

confidence: 69%

Effectiveness of tofacitinib monotherapy for patients with IgG4-RD or idiopathic retroperitoneal fibrosis

Cao,

Li,

Wan

et al. 2023

Clinical and Experimental Rheumatology

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ObjectiveTo 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. Methods Clinical data of patients withIgG4-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. ResultsTwo 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. ConclusionTofacitinib 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.

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Section: Introductionmentioning

confidence: 69%

Effectiveness of tofacitinib monotherapy for patients with IgG4-RD or idiopathic retroperitoneal fibrosis

Cao,

Li,

Wan

et al. 2023

Clinical and Experimental Rheumatology

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“…The blockage of Janus kinase 1/2 inhibitors on IL‐6/IL‐6 receptor signal implies its therapeutic potential 76 . Tocilizumab, a monoclonal antibody targeting the IL‐6 receptor, has shown good curative effects and tolerance in IgG4‐RD 77,78 . Other feasible therapeutic strategies are emerging for the treatment of IgG4‐RD.…”

Section: B‐cell Targeted Therapymentioning

confidence: 99%

“… 76 Tocilizumab, a monoclonal antibody targeting the IL‐6 receptor, has shown good curative effects and tolerance in IgG4‐RD. 77 , 78 Other feasible therapeutic strategies are emerging for the treatment of IgG4‐RD.…”

Section: B‐cell Targeted Therapymentioning

confidence: 99%

The spectrum of B cells in the pathogenesis, diagnosis and therapeutic applications of immunoglobulin G4‐related disease

Hao,

Sun,

Liu

2023

Clin & Trans Imm

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Immunoglobulin G4 (IgG4)‐related disease is a chronic fibroinflammatory disease mediated by immune disorders. Given the challenging clinical diagnosis and treatment, knowledge of the pathogenesis of IgG4‐related disease is important. The typical elevation of serum IgG4 concentrations and infiltration of IgG4‐positive plasma cells in the involved tissues indicate the involvement of B lymphocytes in the pathogenesis of IgG4‐related disease. Mass production of autoantibodies reflects abnormal activation of B cells, which causes tissue damage. Circulating plasmablasts are recently discovered markers that correlate with serum IgG4 concentration, the extent of organ involvement and disease activity. B‐cell depletion therapy is an emerging curative strategy that can significantly alleviate clinical manifestations and achieve remission in patients with IgG4‐related disease. These findings highlight the potential role of B cells in IgG4‐related disease. In this review, we discuss the pathogenic impact of B lymphocytes on IgG4‐related disease and describe novel therapies targeting B cells.

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Therapeutic Uses of Rituximab and Clinical Features in Immunoglobulin G4-Related Disease: A Systematic Review

Patel,

Saxena,

Patel

et al. 2023

Cureus

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This research presents a systematic review focusing on rituximab's therapeutic applications in immunoglobulin G4 (IgG4)-related disease (IgG4-RD), a rare condition characterized by immune-mediated systemic inflammation and tissue fibrosis, as well as the clinical features of IgG4-RD. While the disease commonly affects organs such as the bile ducts, lymph nodes, retroperitoneum, pancreas, and salivary glands, it can potentially involve other organs. This intricacy often leads to diagnostic challenges due to clinical overlaps with cancer, infections, and other autoimmune disorders. The diagnosis of IgG4-RD necessitates a comprehensive approach involving laboratory tests, imaging studies, and clinical assessments. Symptoms can vary, ranging from lymphadenopathy to jaundice, affecting multiple organs. Although elevated blood IgG4 levels and findings of tissue involvement and fibrosis on imaging can be suggestive, they lack the specificity for a definitive diagnosis. Early diagnosis is crucial for initiating corticosteroids and immunosuppressive to prevent further damage from IgG4-RD. This study highlights the therapeutic role of rituximab in managing this condition. Adhering to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) criteria, the research identifies and evaluates relevant literature across various electronic databases, including PubMed, ScienceDirect, and Google Scholar. This review includes 14 selected publications, comprising three systematic reviews, three observational studies, four narrative reviews, and four case reports. The study design ensures a comprehensive evaluation of rituximab's potential efficacy in treating IgG4-RD and its associated clinical characteristics. Based on this study, it can be concluded that IgG4-RD can potentially be treated with rituximab, particularly in cases of relapse and maintaining remission.

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Two cases of refractory IgG4-related disease successfully treated with tocilizumab

Cited by 3 publications

References 8 publications

Effectiveness of tofacitinib monotherapy for patients with IgG4-RD or idiopathic retroperitoneal fibrosis

Effectiveness of tofacitinib monotherapy for patients with IgG4-RD or idiopathic retroperitoneal fibrosis

The spectrum of B cells in the pathogenesis, diagnosis and therapeutic applications of immunoglobulin G4‐related disease

Therapeutic Uses of Rituximab and Clinical Features in Immunoglobulin G4-Related Disease: A Systematic Review

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