Clinical characteristics of immunoglobulin G4–related disease: a prospective study of 118 Chinese patients

Lin, Weihong; Lu, Sha; Chen, Hua; Wu, Qingjun; Fei, Yunyun; Li, Mengtao; Zhang, Xuan; Tian, Xinping; Zheng, Wenjie; Leng, Xiaomei; Xu, Dong; Wang, Qian; Shen, Min; Wang, Li; Li, Jing; Wu, Di; Zhao, Lidan; Wu, Caimei; Yang, Yun; Peng, Linyi; Zhou, Jiaxin; Wang, Yu; Sha, Yue; Huang, Xueying; Jiao, Yang; Zhang, Xuejun; Shi, Qun; Li, Ping; Zhang, Shulan; Hu, Chao; Deng, Chuiwen; Li, Yongzhe; Zhang, Shangzhu; Liu, Jinjing; Su, Jinmei; Hou, Yong; Ying, Jiang; You, Xin; Zhang, Haiting; Yan, Linyi; Zhang, Wen; Zhao, Yan; Zeng, Xiaofeng; Zhang, Fengchun; Lipsky, Peter E.

doi:10.1093/rheumatology/kev203

Cited by 199 publications

(203 citation statements)

References 35 publications

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“…Over 70% of patients in Queen Mary Hospital had positive histopathological confirmation, in comparison to 54.2% in a large Mainland Chinese cohort. 19 Although this proportion increased to 81.7% in the combined analysis, this could be an overestimation of real clinical practice with potential publication and selection bias from literature review. Similar to other reported populations, HBP system, salivary gland, lymph node, and eye were the most common organ systems involved in both Queen Mary Hospital and the combined analysis.…”

Section: Discussionmentioning

confidence: 84%

“…After combination of our patients with all other published cases of IgG4-RD from Hong Kong, we analysed 104 cases comprising predominantly older men (mean age, 62 ± 13 years; male-to-female ratio=3:1), which is consistent with other reports. [19][20][21][22] Over 95% of patients had serum IgG4 level of >135 mg/dL and an IgG4:IgG ratio of >8%. Although these cut-offs are often quoted in the diagnostic criteria for IgG4-RD, 4,23 it is important to note that elevated serum IgG4 levels can be seen in a variety of other conditions such as malignancies, infections, or autoimmune disorders.…”

Section: Discussionmentioning

confidence: 99%

“…A summary of the demographics, clinical features, and treatment modalities in comparison to other cohorts is shown in Table 1. [8][9][10][11][12][13][14][15][16][17][18][19][20][21] The most common organ systems involved were HBP system (40.4%), salivary gland (33.7%), lymph node (29.8%), and eye (19.2%). A Venn diagram of these most common involved systems from the combined cohort is shown in the Figure. …”

Section: Demographics Clinical Features and Treatment Modalitiesmentioning

confidence: 99%

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Immunoglobulin G4–related disease in Hong Kong: clinical features, treatment practices, and its association with multisystem disease

et al. 2017

Hong Kong Med J

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Section: Discussionmentioning

confidence: 84%

Section: Discussionmentioning

confidence: 99%

Section: Demographics Clinical Features and Treatment Modalitiesmentioning

confidence: 99%

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Immunoglobulin G4–related disease in Hong Kong: clinical features, treatment practices, and its association with multisystem disease

et al. 2017

Hong Kong Med J

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“…But in our study, there was no significant correlation between IgG4 and the number of organ involvement (r=0.220, P=0.430). Moreover, serum IgE levels and eosinophilia were commonly observed (8,20,27). The elevated serum IgE levels and eosinophilia were observed in 55% and 20%, respectively.…”

Section: Discussionmentioning

confidence: 98%

Pulmonary function tests findings and their diagnostic value in patients with IgG4-related disease

et al. 2017

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Background: IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder that can affect most organs. To date, there have been no detailed assessments of pulmonary function in patients with IgG4-RD. In this study, we investigated pulmonary function in IgG4-RD patients and evaluated the value of pulmonary function tests (PFTs) in diagnosing IgG4-related respiratory disease (IgG4-RRD).Methods: This was a retrospective study of 17 patients with IgG4-RD. The patients were divided into two groups: IgG4-RRD group and IgG4-related disease extrapulmonary involvement (IgG4-RDEI) group. The PFT results were compared between the two groups.Results: All patients in the IgG4-RRD group had pulmonary dysfunction. Five of 8 (62.5%) patients in the IgG4-RDEI group had pulmonary dysfunction, despite having normal thoracic computed tomography scans and no respiratory symptoms. Patients in both groups showed restrictive ventilatory dysfunction and abnormal diffusing capacity, and two patients in the IgG4-RRD group had obstructive ventilatory dysfunction. The incidence of diffusing capacity of the lung for carbon monoxide per liter of alveolar volume (D L CO/V A ) decrease were significantly higher in the IgG4-RRD group than in the IgG4-RDEI group (P=0.029). D L CO/V A were significantly higher in the IgG4-RDEI than in the IgG4-RRD group (P=0.044), but otherwise, there were no significant differences. We report the first finding of a negative correlation between pulmonary diffusing capacity and total serum concentrations of IgG and IgG subclasses (IgG4, IgG3 and IgG2).Conclusions: D L CO/V A plays an important role for detecting lung involvement in IgG4-RD patients. The patient with high serum IgG may be more prone to respiratory involvement. (5), in 2011, two separate names were adopted for the respiratory tract lesions of IgG4-RD: 'IgG4-related lung disease' and 'IgG4-related pleural disease' (6). In the latest published diagnostic guide in 2016, they are collectively as 'IgG4-related respiratory disease (IgG4-RRD)' (7). IgG4-RRD can involve only the lung or additionally affect other organs. Although many medical conditions have been attributed to IgG4-RD, relatively little is known about IgG4-RRD and it remains a rare condition. The epidemiology of IgG4-RRD is poorly understood, mainly because of challenges in recognizing the disease and in differentiating it from the many disorders it mimics. Only about 50% of patients with IgG4-RRD exhibit respiratory symptoms such as cough, dyspnea on exertion and chest pain, while the remaining 50% display only radiographic abnormalities (8).Thoracic involvement in IgG4-RD can involve the parenchyma, airways, pleura, and mediastinum and can affect the patient's lung function. Currently, pulmonary function tests (PFTs) are not routine examinations for IgG4-RD. Since PFTs are a sensitive method for detecting diffuse parenchymal lung disease, we considered that they might be helpful in diagnosing IgG4-RRD. To date, there have been few detailed reports of pulmonary function as...

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“…[1][2][3] Due to the short duration of understanding of the disease, there is no uniform standard or guideline for treatment of IgG4-RD. It is widely accepted that patients with IgG4-RD are generally sensitive to glucocorticoid, with organ swelling reduced or disappearing after treatment, along with the decreasing of serum IgG4 levels.…”

Section: Introductionmentioning

confidence: 99%

Efficacy between high and medium doses of glucocorticoid therapy in remission induction of IgG4‐related diseases: a preliminary randomized controlled trial

Chang

Chen

et al. 2017

Int J of Rheum Dis

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Aim: In order to evaluate the efficacy and safety of high versus medium doses of glucocorticoid therapy in remission induction of immunoglobulin G4-related disease (IgG4-RD), we set up a randomized controlled study.Method: Newly diagnosed IgG4-RD patients were randomly assigned to two groups: high doses of prednisone (0.8-1.0 mg/kg/day) and medium doses (0.5-0.6 mg/kg/day). Patients were assessed at weeks 0, 4, 12 and 24. The primary outcome was the remission rate at week 24. The secondary endpoints included IgG4-RD responder index (IgG4-RD RI), IgG and IgG4 levels.Results: Twenty cases in each group finished the study. At week 24, the total response rates were 95% and 80% in high and medium dose groups, respectively. There was no significant difference between the two groups. IgG4-RD RI reduced from 14.9 to 3.0 in the high dose group, while in the medium dose group it was from 13.1 to 3.2. At week 24, the average level of IgG4 reduced from 1576 to 324 mg/dL and from 1445 to 684 mg/dL in the two groups, respectively. Relapsed patients had higher baseline IgG4-RD RI. There was no severe adverse effect shown in both groups. Conclusion:The effect of remission induction was similar in high and medium glucocorticoid groups. However, patients with more organs involved and higher IgG4-RD RI score at baseline might get more benefit with high dose glucocorticoid for remission induction.

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Clinical characteristics of immunoglobulin G4–related disease: a prospective study of 118 Chinese patients

Cited by 199 publications

References 35 publications

Immunoglobulin G4–related disease in Hong Kong: clinical features, treatment practices, and its association with multisystem disease

Immunoglobulin G4–related disease in Hong Kong: clinical features, treatment practices, and its association with multisystem disease

Pulmonary function tests findings and their diagnostic value in patients with IgG4-related disease

Efficacy between high and medium doses of glucocorticoid therapy in remission induction of IgG4‐related diseases: a preliminary randomized controlled trial

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