Yuxue Nie

Peking Union Medical College Hospital

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Linyi Peng

Peking Union Medical College Hospital

Wen Zhang

Peking Union Medical College Hospital

Jiaxin Zhou

Chinese Academy of Medical Sciences & Peking Union Medical College

Jingna Li

Peking Union Medical College Hospital

Mengtao Li

Chinese Academy of Medical Sciences & Peking Union Medical College

Yu Peng

Chongqing Medical University

Ruijie Sun

Peking Union Medical College Hospital

Hui Lu

Capital Medical University

Yunyun Fei

Chinese Academy of Medical Sciences & Peking Union Medical College

Xiaofeng Zeng

Chinese Academy of Medical Sciences & Peking Union Medical College

Cooperative Institutions

Peking Union Medical College Hospital

Chinese Academy of Medical Sciences & Peking Union Medical College

Ministry of Education of the People's Republic of China

National Clinical Research Center for Digestive Diseases

National Clinical Research

Academy of Medical Sciences

Sichuan University

Capital Medical University

Sun Yat-sen University

Southern Medical University

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Memory CD4+T cell profile is associated with unfavorable prognosis in IgG4-related disease: Risk stratification by machine-learning

Clinical Immunology (2023)

Yuxue Nie Zheng Liu Wei Cao Yu Peng Hui Lu

Immunophenotyping

Risk Stratification

IgG4-Related Disease

10.1016/j.clim.2023.109301

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Citations (3)

The potential impact of autoimmune diseases family history in IgG4-Related Disease: a case control study

Research Square (Research Square) (2022)

Rui Sun Zheng Liu Hui Lu Yu Peng Jieqiong Li

Abstract Objective: Autoimmune comorbidities may be associated with IgG4-Related Disease (IgG4-RD), here we aimed to determine the correlation of AID family history and IgG4-RD in a Chinese cohort. Methods: This case-control studyidentified 628 cases of IgG4-RD in Peking Union Medical College Hospital. Patients were classified into two groups, with AID family history group (AID-pos) and without AID family history group (AID-neg). We viewed the potential value of AID family history on IgG4-RD by comparing the differences between the two groups. In addition, Cox regression analysis estimated confidence intervals (CI) and hazard ratio (HR) for IgG4-RD risk. Results: 93 (14.8%) IgG4-RD patients had AID family history. Compared with AID-neg group, baseline data analysis revealed that AID-pos group patients had an earlier age of IgG4-RD onset (50.4 ± 14.8 vs. 54.2 ± 12.6, p =0.014*), a higher percentage of antinuclear antibody (ANA) positivity (38.9% vs 22.7%, p =0.0277*) and IgG4-related thyroiditis (10.9% vs 2.4%, p =0.001*), were prone to comorbid with other AID (16.1% vs 6.2%, p =0.0238*). Cox analysis found that younger age (HR 0.97 [95%CI 0.94-0.99], p =0.0384*) and higher proportions of baseline peripheral eosinophils (HR 1.1 [95%CI 1.02-1.2], p =0.0199*) increased the risk of unfavorable prognosis for AID-pos IgG4-RD patients. Conclusions: 14.8% of IgG4-RD patients had AID family history, with younger age of disease onset age and higher frequency of ANA positivity, indicating that IgG4-RD may share genetic background with other AID.

Medical History

10.21203/rs.3.rs-2149027/v1

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Coronary periarteritis and pericarditis are rare but distinct manifestations of heart involvement in IgG4-related disease: a retrospective cohort study

Orphanet Journal of Rare Diseases (2024)

Tianrui Hua Juan Du Xiaoxiao Guo Linyi Peng Jiaxin Zhou

The heart can be involved in immunoglobulin (Ig)-G4-related disease (IgG4-RD). This study aimed to summarize the clinical features and efficacy of treatment for IgG4-RD patients with heart involvement.

IgG4-Related Disease

10.1186/s13023-024-03266-y

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Citations (1)

Additional file 3 of TSLP promoting B cell proliferation and polarizing follicular helper T cell as a therapeutic target in IgG4-related disease

Figshare (2022)

Hui Lu Xunyao Wu Yu Peng Ruijie Sun Yuxue Nie

Additional file 3: Table S2. List of differential expression genes of TSLP activated B cells and B cells from IgG4-RD.

10.6084/m9.figshare.21069456

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Withdrawal of immunosuppressants and low-dose steroids in patients with stable IgG4-RD (WInS IgG4-RD): an investigator-initiated, multicentre, open-label, randomised controlled trial

Annals of the Rheumatic Diseases (2024)

Linyi Peng Yuxue Nie Jiaxin Zhou Lijun Wu Xiaomei Chen

Objectives

IgG4-related disease (IgG4-RD) is an immune-mediated, fibroinflammatory disease. Induction treatment with glucocorticoid (GC) is usually effective, but its tendency of relapse makes the strategy for maintenance treatment a challenge. The WInS IgG4-RD (withdraw immunosuppressants (IMs) and steroid in stable IgG4-RD) trial tested whether discontinuation of GC and IM was feasible in stable IgG4-RD.

Methods

The WInS IgG4-RD trial was a multicentre, open-label, randomised controlled trial. Patients with IgG4-RD receiving GC+IM as maintenance treatment with clinically quiescent disease for at least 12 months were randomised (1:1:1) into three groups: group 1: withdraw GC+IM; group 2: withdraw GC but maintain IM; group 3: maintain GC+IM. The primary outcome was the relapse rate of disease within 18 months. The secondary outcomes included the changes of IgG4-RD Responder Index (RI), Physician's Global Assessment (PGA), serum IgG4 and IgG, as well as adverse events.

Results

One hundred and forty-six patients were randomised, with 48 patients in group 1, 49 patients in group 2 and group 3, respectively. Within the 18-month follow-up period, disease relapse occurred in 25 out of 48 (52.1%) patients in group 1 vs 7 out of 49 (14.2%) in group 2 and 6 out of 49 (12.2%) in group 3 (p<0.001). The changes in RI and PGA were significantly higher in group 1 than in group 2 (p<0.001) or group 3 (p<0.001).

Conclusions

The maintenance of IMs, with or without low-dose GC, was found to be superior to withdraw GC+IM in preventing relapse for long-time stable IgG4-RD.

Trial registration number

NCT04124861.

Discontinuation

IgG4-Related Disease

Maintenance therapy

10.1136/ard-2023-224487

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Citations (5)

Recent advances in diagnosis, treatment, and prognosis of IgG4-related autoimmune pancreatitis: a narrative review

Journal of Pancreatology (2022)

Xin He Boyuan Sun Yuxue Nie Wen Zhang

Immunoglobulin G4 (IgG4)-related autoimmune pancreatitis (AIP), also known as “type 1 AIP,” is a rare, chronic, and fibroinflammatory disease manifested as obstructive jaundice and enlargement of the pancreas, usually accompanied by extra-pancreatic organ involvement. The understanding of IgG4-related AIP is gradually deepening. In this review, we summarized the basic concepts, common clinical manifestations, and new progress of the disease including diagnostic, therapeutic strategies, and prognosis mainly based on published case reports, cohort studies, meta-analyses, and guidelines in the past 5 years. Issues such as diagnostic markers, risk factors for relapse, and more effective treatment still need to be further studied.

Autoimmune Pancreatitis

IgG4-Related Disease

Narrative review

Obstructive jaundice

10.1097/jp9.0000000000000112

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Citations (1)

The efficacy and safety of leflunomide therapy in lupus nephritis by repeat kidney biopsy

Rheumatology International (2009)

F. S. Zhang Yuxue Nie Xiaoming Jin Honglian Yu Y. N. Li

Leflunomide

10.1007/s00296-009-0861-3