Efficacy and safety of low dose Mycophenolate mofetil treatment for immunoglobulin G4-related disease: a randomized clinical trial

Fei, Yunyun; Peng, Yu; Zhang, Panpan; Xia, Zhengyuan; Linyi, Peng; Zhou, Jiaxin; Li, Zhang; Zhang, Shangzhu; Liu, Jinjing; Wu, Di; Liu, Yamin; Liu, Xiaowei; Xue, Huadan; Zhang, Xuan; Zeng, Xiaofeng; Zhang, Fengchun; Zhao, Yan; Zhang, Wen

doi:10.1093/rheumatology/key227

Cited by 100 publications

(78 citation statements)

References 20 publications

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“…Therapy included two stages: the GC tapering period and the GC maintenance period. The standard induction dosage of oral prednisone was 0.6–1.0 mg kg −1 day −1 in the first month and was gradually decreased by 5 mg per 1 or 2 weeks to the maintenance dosage after patients achieved disease remission; this was defined as the GC tapering period .…”

Section: Methodsmentioning

confidence: 99%

“…Clinical relapse was defined as recurrence or worsening of clinical symptoms or imaging findings with or without re‐elevation of IgG4 concentration . Patients with only elevated serum IgG4 levels were not considered as having relapsed .…”

Section: Methodsmentioning

confidence: 99%

“…IgG4‐related disease (IgG4‐RD) is a newly recognized chronic fibro‐inflammatory disease of unknown aetiology, which is characterized by IgG4‐positive plasma cell infiltration of the affected organs or tissues, swelling and sclerosis, with or without elevated serum IgG4 concentration . The disease commonly affects the lacrimal glands, periorbital tissues, salivary glands, pancreas, biliary tree, lymph nodes, retroperitoneal tissues, lung, kidney and skin .…”

Section: Introductionmentioning

confidence: 99%

“…IgG4‐RD can follow two clinical courses, deterioration/relapse and remission, with an overall 24–63% chance of relapse . Almost all patients respond well to initial glucocorticoid (GC) therapy, with amelioration of symptoms and serum IgG4 concentration .…”

Section: Introductionmentioning

confidence: 99%

“…Almost all patients respond well to initial glucocorticoid (GC) therapy, with amelioration of symptoms and serum IgG4 concentration . However, some patients might present with recurrence whilst receiving tapered or maintenance dosages . To date, some studies have reported relapse predictors in IgG4‐RD patients with Mikulicz's disease, autoimmune pancreatitis and other specific types of IgG4‐RD, but few studies have reported reliable relapse predictors in patients with full‐spectrum IgG4‐RD.…”

Section: Introductionmentioning

confidence: 99%

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Clinical outcomes and predictive relapse factors of IgG4‐related disease following treatment: a long‐term cohort study

Peng

Zhang

et al. 2019

J Intern Med

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Clinical outcomes and predictive relapse factors of IgG4related disease following treatment: a long-term cohort study. J Intern Med 2019; 286: 542-552.Objectives. The aim of this study was to investigate the predictive factors for relapse of IgG4-related disease (IgG4-RD) and observe the long-term clinical outcomes in patients with IgG4-RD.Methods. We included in the present analysis 122 patients who were newly diagnosed with IgG4-RD, treated with glucocorticoid (GC) monotherapy or GC and immunosuppressant combination therapy, and followed for at least 3 years. Clinical relapse, response and side effects were recorded.Results. The cumulative relapse rates of patients in this study were 10.66%, 22.95% and 27.87% at 12, 24 and 36 months, respectively. Complete drug withdrawal was an independent risk factor for disease relapse. Higher serum IgG4 concentrations, involvement of more organs, higher IgG4 RI scores and elevation of eosinophils at baseline were closely associated with disease relapse. Re-elevation of serum IgG4 concentrations and low GC maintenance dosage during the follow-up period were significantly associated with clinical relapse. The GC dosage should be more than 6.25 mg day À1 as monotherapy during the maintenance stage; moreover, combining with immunosuppressants can reduce the GC dosage. Adding GC or immunosuppressants for patients with re-elevation of serum IgG4 levels could prevent later disease relapse. No serious complications were noted during long-term follow-up.Conclusions. The combination of GC with immunosuppressants was more effective than GC monotherapy during the steroid tapering and maintenance stages. Higher serum IgG4 levels, involvement of more organs, higher IgG4 RI scores, history of allergy, eosinophil elevation at baseline, re-elevation of serum IgG4 levels and lower GC maintenance dosage at follow-up might be predictive of relapse.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinical outcomes and predictive relapse factors of IgG4‐related disease following treatment: a long‐term cohort study

Peng

Zhang

et al. 2019

J Intern Med

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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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IgG4-Related Tubulointerstitial Kidney Disease

Buglioni

Sethi

Cornell

2022

Tubulointerstitial Nephritis

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Efficacy and safety of low dose Mycophenolate mofetil treatment for immunoglobulin G4-related disease: a randomized clinical trial

Abstract: ClinicalTrials.gov, www.clinicaltrials.gov, NCT02458196.

Cited by 100 publications

References 20 publications

Clinical outcomes and predictive relapse factors of IgG4‐related disease following treatment: a long‐term cohort study

Clinical outcomes and predictive relapse factors of IgG4‐related disease following treatment: a long‐term cohort study

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

IgG4-Related Tubulointerstitial Kidney Disease

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