Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: a retrospective study of 127 Chinese patients
Abstract Background and aims Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition. The aim of the present study was to characterize the clinical features and therapeutic response of patients with IgG4-RD and identify risk factors for disease relapse. Methods We co...
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| Format: | Article |
| Language: | English |
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BMC
2022-08-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13023-022-02404-8 |
| _version_ | 1828444475035222016 |
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| author | Wen An Zhen Wu Min Li Haitian Yu Xinyan Zhao Xiaoming Wang Yu Wang Qianyi Wang Weijia Duan Yuanyuan Kong Hong Ma Xiaojuan Ou Hong You Yanying Liu Peng Li Ting Duan Jidong Jia |
| author_facet | Wen An Zhen Wu Min Li Haitian Yu Xinyan Zhao Xiaoming Wang Yu Wang Qianyi Wang Weijia Duan Yuanyuan Kong Hong Ma Xiaojuan Ou Hong You Yanying Liu Peng Li Ting Duan Jidong Jia |
| author_sort | Wen An |
| collection | DOAJ |
| description | Abstract Background and aims Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition. The aim of the present study was to characterize the clinical features and therapeutic response of patients with IgG4-RD and identify risk factors for disease relapse. Methods We collected baseline data of eligible patients with IgG4-RD and analyzed clinical features by interview and review of medical records. The patients who received glucocorticoids (GC) therapy with at least 3 months follow-up were used to characterize the therapeutic response and identify risk factors for relapse. Result Totally 127 IgG4-RD patients, including 92 males and 35 females, were enrolled in the present study. The median age of onset was 63.0 years, ranging from 23 to 86. The pancreas, bile duct and lymph nodes were the most frequently involved organs. The serum IgG4 level was elevated in 94.5% of the patients and was correlated with the number of organs involved. Patients classified as head and neck limited group were more likely to be female. Compared to Mikulicz syndrome and systemic involvement group, pancreato-hepatobiliary group had higher aminotransferase, alkaline phosphatase, gamma-glutamyl transpeptidase, bilirubin and lower IgG4 level. Mikulicz syndrome and systemic involvement group had the highest IgG4-RD RI score, IgG level. Among 92 patients who received medical therapy with at least 3 months follow-up, 76 received GC alone or in combination with immunomodulator (IM) and 16 patients did not take GC. 74 out of the 76 patients (97.3%) achieved remission, with 59 of them remained in remission and 15 of them relapsed. Whereas 16 patients did not take GC, among them, 6 patients achieved remission with one relapsed. On multivariate analysis, higher initial score of ACR/EULAR IgG4-RD Classification Criteria and GC withdrawal were independent predictors for relapse. Conclusion Four phenotypes of IgG4-RD showed different demographic and serological features. GC + IM therapy was safe and effective and might protect patients from relapse. The independent risk factors of relapse were GC withdrawal and higher score of ACR/EULAR IgG4-RD Classification Criteria. |
| first_indexed | 2024-12-10T21:44:01Z |
| format | Article |
| id | doaj.art-c2f382c4ebdb41fd91bdae8e37fbabe6 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-10T21:44:01Z |
| publishDate | 2022-08-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-c2f382c4ebdb41fd91bdae8e37fbabe62022-12-22T01:32:25ZengBMCOrphanet Journal of Rare Diseases1750-11722022-08-0117111010.1186/s13023-022-02404-8Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: a retrospective study of 127 Chinese patientsWen An0Zhen Wu1Min Li2Haitian Yu3Xinyan Zhao4Xiaoming Wang5Yu Wang6Qianyi Wang7Weijia Duan8Yuanyuan Kong9Hong Ma10Xiaojuan Ou11Hong You12Yanying Liu13Peng Li14Ting Duan15Jidong Jia16Liver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesLiver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesCenter for Clinical Epidemiology and EBM, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesLiver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesLiver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesLiver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesLiver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesLiver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesLiver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesCenter for Clinical Epidemiology and EBM, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesLiver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesLiver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesLiver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesDepartment of Rheumatology, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesDepartment of Gastroenterology, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesDepartment of Rheumatology, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesLiver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Cirrhosis, National Clinical Research Center for Digestive DiseasesAbstract Background and aims Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition. The aim of the present study was to characterize the clinical features and therapeutic response of patients with IgG4-RD and identify risk factors for disease relapse. Methods We collected baseline data of eligible patients with IgG4-RD and analyzed clinical features by interview and review of medical records. The patients who received glucocorticoids (GC) therapy with at least 3 months follow-up were used to characterize the therapeutic response and identify risk factors for relapse. Result Totally 127 IgG4-RD patients, including 92 males and 35 females, were enrolled in the present study. The median age of onset was 63.0 years, ranging from 23 to 86. The pancreas, bile duct and lymph nodes were the most frequently involved organs. The serum IgG4 level was elevated in 94.5% of the patients and was correlated with the number of organs involved. Patients classified as head and neck limited group were more likely to be female. Compared to Mikulicz syndrome and systemic involvement group, pancreato-hepatobiliary group had higher aminotransferase, alkaline phosphatase, gamma-glutamyl transpeptidase, bilirubin and lower IgG4 level. Mikulicz syndrome and systemic involvement group had the highest IgG4-RD RI score, IgG level. Among 92 patients who received medical therapy with at least 3 months follow-up, 76 received GC alone or in combination with immunomodulator (IM) and 16 patients did not take GC. 74 out of the 76 patients (97.3%) achieved remission, with 59 of them remained in remission and 15 of them relapsed. Whereas 16 patients did not take GC, among them, 6 patients achieved remission with one relapsed. On multivariate analysis, higher initial score of ACR/EULAR IgG4-RD Classification Criteria and GC withdrawal were independent predictors for relapse. Conclusion Four phenotypes of IgG4-RD showed different demographic and serological features. GC + IM therapy was safe and effective and might protect patients from relapse. The independent risk factors of relapse were GC withdrawal and higher score of ACR/EULAR IgG4-RD Classification Criteria.https://doi.org/10.1186/s13023-022-02404-8IgG4-related diseaseClinical phenotypeTherapeutic responseRelapsePredictive factors |
| spellingShingle | Wen An Zhen Wu Min Li Haitian Yu Xinyan Zhao Xiaoming Wang Yu Wang Qianyi Wang Weijia Duan Yuanyuan Kong Hong Ma Xiaojuan Ou Hong You Yanying Liu Peng Li Ting Duan Jidong Jia Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: a retrospective study of 127 Chinese patients Orphanet Journal of Rare Diseases IgG4-related disease Clinical phenotype Therapeutic response Relapse Predictive factors |
| title | Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: a retrospective study of 127 Chinese patients |
| title_full | Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: a retrospective study of 127 Chinese patients |
| title_fullStr | Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: a retrospective study of 127 Chinese patients |
| title_full_unstemmed | Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: a retrospective study of 127 Chinese patients |
| title_short | Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: a retrospective study of 127 Chinese patients |
| title_sort | clinical characteristics and therapeutic response of immunoglobulin g4 related disease a retrospective study of 127 chinese patients |
| topic | IgG4-related disease Clinical phenotype Therapeutic response Relapse Predictive factors |
| url | https://doi.org/10.1186/s13023-022-02404-8 |
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