scholarly journals Case Follow-Up: Development of Primary Membranous Glomerulonephritis in a Patient with IgG4 Related Disease

2017 ◽  
Vol 03 (01) ◽  
Author(s):  
Philip Clerc ◽  
Julie Davenport ◽  
Matthew Carroll
Keyword(s):  
Membranous Glomerulonephritis ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab

2021 ◽  
Vol 10 (6) ◽  
pp. 1329
Author(s):  
Johanna Backhus ◽  
Christian Neumann ◽  
Lukas Perkhofer ◽  
Lucas A Schulte ◽  
Benjamin Mayer ◽  
...  
Keyword(s):  
Disease Activity ◽  
Clinical Activity ◽  
Inflammatory Disorder ◽  
International Consensus ◽  
Steroid Pulse ◽  
Related Disease ◽  
Igg4 Related Disease

Objectives: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears a promising steroid-sparing therapy, although long-term data are lacking. We aimed to bridge this gap with a cohort of IgG4-RD patients treated with RTX and to assess the potential value of the Responder Index (RI) as a discriminatory score for disease activity. Methods: We retrospectively evaluated 46 patients from a tertiary referral centre who were diagnosed with IgG4-RD and/or type 1 AIP according to the International Consensus Diagnostic Criteria or Unifying-AIP criteria between June 2006 and August 2019. Results: Patients resembled previous cohorts in terms of characteristics, diagnosis, and therapeutic response. Thirteen of the 46 patients with IgG4-RD/type 1 AIP were treated with RTX pulse therapy due to relapse, adverse reactions to steroids, or high-risk constellations predicting a severe course of disease with multi-organ involvement. Median follow-up after diagnosis was 52 months for all subjects, and 71 months in IgG4-RD patients treated with RTX. While patients in the RTX group showed no significant response to an initial steroid pulse, clinical activity as measured by the RI significantly decreased in the short-term after RTX induction. Within 16 months, 61% of patients relapsed in the RTX group but responded well to re-induction. Clinical and laboratory parameters improved equally in response to RTX. Conclusion: RTX therapy in patients with IgG4-RD is an effective and safe treatment to induce treatment response and possible long-term remission. Repeated RTX administration after 6–9 months may be of value in reducing the risk of relapse. The RI appears to be a reasonable index to assess disease activity and to identify patients with IgG4-related disease who may benefit from B-cell-depleting therapy.

scholarly journals SAT0525 EFFICACY AND SAFETY OF MZR FOR IgG4-RELATED DISEASE.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1219-1220
Author(s):  
S. Kawaai ◽  
S. Fukui ◽  
T. Nakai ◽  
G. Kidoguchi ◽  
H. Ozawa ◽  
...  
Keyword(s):  
Adverse Events ◽  
Retention Rate ◽  
Case Reports ◽  
Efficacy And Safety ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Number Of Patients ◽  
Serum Igg4 ◽  
Over Time

Background:IgG4-Related Disease (IgG4RD) is known to cause multiple organ lesions with infiltration of IgG4-positive plasma cells, and patients often have relapses with tapering treatments despite an initial good response to glucocorticoids therapy. Mizoribine (MZR) is an immunosuppressant working as an inhibitor of purine synthesis, which mechanism of action is similar to mycophenolate mofetil. Data regarding the efficacy and safety of MZR on IgG4RD is limited although some previous case reports1showed effectiveness for IgG4RD.Objectives:This study aims to assess the efficacy and safety of MZR in patients with IgG4RD.Methods:We retrospectively reviewed charts of IgG4RD patients who used MZR between January 2004 and December 2019 at Immuno-Rheumatology Center in St. Luke’s International Hospital, Tokyo, Japan. We investigated basic demographics, involved organs, results of blood tests including IgG and IgG4 titer, and medications used including glucocorticoid and other immunosuppressants (IS). We followed IgG4 titer, dose of glucocorticoid, flare of disease and retention of MZR at the beginning, 6 and 12months after starting MZR. We compared changes in PSL (prednisolone) doses and IgG4 titers over time using Friedman test with Bonferroni correction. We also checked adverse events during follow up.Results:Twenty-two patients with IgG4RD who used MZR were included. Median age was 62 years old, and 15 (68.2%) patients are male. Lacrimal and salivary glands, pancreatitis and retroperitoneal fibrosis were common lesions. All patients were initially treated with glucocorticoids. Flare was observed in 5 (22.7 %) patients before initiation of MZR. The number of patients who continued MZR without flare are 19 (86.4 %) at 6 months, and 14 (73.7 %) at 12 months. IgG4 titer significantly declined at 6 and 12 months from baseline although significant consecutive decrease in PSL dose (Figure 1, 2). Liver dysfunctions are commonest adverse events (n=16, 72.7%) but mild (grade1; n=15, 68.2%) and most cases are apparently due to other reasons. Serious infection (SI) occurred in 3 (13.6%) patients in total follow up, however no SI were observed during 1 year after MZR treatment.Conclusion:MZR can be safely used in patients of IgG4RD with high retention rate, and seemed to have steroid-sparing effect. Prospective comparative studies are needed.References:[1]Nanke Y, Kobashigawa T, Yago T, Kamatani N, Kotake S. A case of Mikulicz’s disease, IgG4-related plasmacytic syndrome, successfully treated by corticosteroid and mizoribine, and then by mizoribine alone. Intern Med 49: 1449-1453, 2010.Table 1.Patient characteristics    Table 2.Disease and treatment status before and after initiation of MZR    Figure 1.Serum IgG4 level changesFigure 2.Changes in the PSL dose over timeDisclosure of Interests:None declared

scholarly journals Membranous glomerulonephritis is a manifestation of IgG4-related disease

2013 ◽  
Vol 83 (3) ◽  
pp. 455-462 ◽  
Author(s):  
Mariam P. Alexander ◽  
Christopher P. Larsen ◽  
Ian W. Gibson ◽  
Samih H. Nasr ◽  
Sanjeev Sethi ◽  
...  
Keyword(s):  
Membranous Glomerulonephritis ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals Lung nodules and IgG4 related disease: a single-center based experience

2020 ◽  
Author(s):  
Yan Xie ◽  
Anji Xiong ◽  
Tony Marion ◽  
Yi Liu
Keyword(s):  
Lung Diseases ◽  
Immunosuppressive Agents ◽  
Ground Glass Opacity ◽  
Lung Nodules ◽  
Bronchial Wall ◽  
West China Hospital ◽  
West China ◽  
Related Disease ◽  
Igg4 Related Disease

Abstract Background and objective: This study was undertaken in an attempt to characterize the frequency and clinical features of lung nodules in IgG4 related disease (IgG4-RD) patients as an insight for help with the diagnosis of lung nodules.Methods: A retrospective study was carried out in West China Hospital, Sichuan University from January 2012 to December 2018, 89 patients with definite IgG4-RD were enrolled.Results: Fifty of 89 patients with definite IgG4-RD had radiologically confirmed lung nodules, 6 of whom were diagnosed with definite IgG4 related lung disease. Lung nodules detected in more than 40 patients were small and solid, always with regular margins. Multiple (41/50) and bilateral (34/50) distributions was also a major characteristic of these lung nodules. Lobulation and speculation were simultaneously detected in 3 patients, including 2 patients combined with pleural indentation. Calcification of nodules was detected in only one patient. Thirty-seven patients also had additional radiological abnormalities of lungs, including ground-glass opacity (21/50), thickening of pleura (9/50), thickening of interlobular septa (4/50), thickening of bronchial wall (3/50), pleural effusion (4/50), mass (3/50), interstitial changes (5/50), and mediastinal or hilar lymphadenopathy (32/50). Most patients (44/50) were treated with glucocorticoids alone or combined with immunosuppressive agents. Sixteen patients received a re-examination by chest computed tomography (CT) scan after treatment, 10 of whom showed a decrease in the size and/or the number of nodules.Conclusions: The incidence of lung nodules in IgG4-RD patients can be high. For an IgG4-RD patient with lung nodules, the possibility that the lung nodules related to IgG4-RLD is high. It is hard to differentiate IgG4 related lung nodules from other lung diseases, in particular, lung cancer. Radiological characteristics and positive responses to glucocorticoids and immunosuppressive agents can help with the differential diagnosis. For these patients, regular follow-up is also important.

scholarly journals AB1244 PROGNOSTIC FACTORS IN IGG4-RELATED DISEASE: A LONG-TERM MONOCENTRIC CHINESE COHORT STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1913.2-1914
Author(s):  
Z. Ji ◽  
L. Ma ◽  
L. Zhang ◽  
L. Ma ◽  
D. Liu ◽  
...  
Keyword(s):  
Cohort Study ◽  
Predictive Factors ◽  
Maintenance Period ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Tnf Α ◽  
Chinese Cohort ◽  
Relapsed Disease

Background:Through initial response to treatment with GC, the patients with IgG4-related disease (IgG4-RD) exhibited high relapse rate after reduction or withdrawal of GC treatment, indicated the unsatisfactory prognosis for IgG4-RD. It is of clinical significance to develop new informative risk factors for refractory and relapsed disease.Objectives:To evaluate the prognosis of IgG4-RD and identify predictive factors for treatment resistance and disease relapse in a Chinese cohort.Methods:102 patients newly diagnosed with IgG4-RD were followed for 6-111 months. Clinical data were compared between patients whose disease went into remission and those who suffered refractory or relapsed disease. Predictive factors for refractory and relapsed disease were calculated by univariate analysis.Results:Among the 78 patients who received medical treatment with regular follow-up, 55 (59.8%) patients sustained clinical remission, and 23 (25%) patients suffered refractory or relapsed disease. The mortality and incidence of malignancy were both 4.35% during follow-up. Serum TNF-α ≥ 13 pg/ml, sIL-2R ≥ 1010 pg/ml, TC < 3.55 mmol/L, LDL < 2.0 mmol/L, IgG > 20.2 g/L, GC withdrawal, and treatment without immunosuppressor (IM) during the maintenance period (OR 3.23) were predictive factors for refractory and relapsed IgG4-RD. The combination of GC and IM treatment was protective (OR 0.338) against refractory and relapsed IgG4-RD.Conclusion:Serum TNF-α, sIL-2R, LDL, TC, IgG, GC withdrawal, and treatment without IM during the maintenance period were predictive factors for refractory and relapsed IgG4-RD. Treatment with GC and IM may protect against refractory and relapsed IgG4-RD.References:[1]Takahashi H, Yamamoto M, Suzuki C, Naishiro Y, Shinomura Y, Imai K. The birthday of a new syndrome: IgG4-related diseases constitute a clinical entity. Autoimmun Rev. 2010, 9: 591-4.[2]Lian L, Wang C, Tian JL. IgG4-related retroperitoneal fibrosis: a newly characterized disease. Int J Rheum Dis. 2016, 19: 1049-55.[3]Li PH, Ko KL, Ho CT, Lau LL, Tsang RK, Cheung TT, et al. Immunoglobulin G4-related disease in Hong Kong: clinical features, treatment practices, and its association with multisystem disease. Hong Kong Med J. 2017, 23: 446-53.[4]Culver EL, Sadler R, Bateman AC, Makuch M, Cargill T, Ferry B, et al. Increases in IgE, Eosinophils, and Mast Cells Can be Used in Diagnosis and to Predict Relapse of IgG4-Related Disease. Clin Gastroenterol Hepatol. 2017, 15: 1444-52.[5]Inoue D, Yoshida K, Yoneda N, Ozaki K, Matsubara T, Nagai K, et al. IgG4-related disease: dataset of 235 consecutive patients. Medicine (Baltimore). 2015, 94: e680.[6]Brito-Zeron P, Kostov B, Bosch X, Acar-Denizli N, Ramos-Casals M, Stone JH. Therapeutic approach to IgG4-related disease: A systematic review. Medicine (Baltimore). 2016, 95: e4002.[7]Peng Y, Li JQ, Zhang PP, Zhang X, Peng LY, Chen H, et al. Clinical outcomes and predictive relapse factors of IgG4-related disease following treatment: a long-term cohort study. J Intern Med. 2019.Acknowledgments:This work was supported by the National Natural Science Foundation of China (NSFC 81601398; NSFC 81771730); the Animal Research Project of Shanghai Science and Technology Commission (grant number 17140902000); and Shanghai Pujiang Rheumatologists Training Program (SPROG201801)Disclosure of Interests:None declared

scholarly journals Improved clinical outcomes of tocilizumab versus cyclophosphamide for IgG4-related disease: insights from a prospective IgG4-related disease registry

2021 ◽  
Vol 12 ◽  
pp. 204062232110287
Author(s):  
Ji Zongfei ◽  
Zhang Lijuan ◽  
Sun Ying ◽  
Liu Dongmei ◽  
Wu Sifan ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Statistical Significance ◽  
Group Versus ◽  
Complete Response ◽  
Vertebral Compression Fractures ◽  
C Reactive Protein ◽  
Reactive Protein ◽  
Related Disease ◽  
Igg4 Related Disease

Objective: To compare the clinical outcomes of patients with active immunoglobulin G (IgG) 4 related disease (IgG4-RD) receiving tocilizumab versus those receiving cyclophosphamide (CYC). Methods: This IgG4-RD registry study was a prospective cohort study conducted among patients with active IgG4-RD hospitalized at Zhongshan Hospital, Fudan University. Patients who were treated with tocilizumab or CYC along with glucocorticoids (GCs) were enrolled. All participants were followed up at the hospital clinic at 3 and 6 months after discharge. Primary clinical outcomes were measured via the IgG4-RD responder index (RI), complete response (CR), and partial response (PR), as well as side effects. Results: From January 2015 to June 2020, 29 patients enrolled. Fourteen and 15 patients were treated with tocilizumab and CYC, respectively. At the 6-month follow-up, disease activity parameters including erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), IgG4, and IgG4-RD RI, decreased significantly in both groups. At 6 months, tocilizumab demonstrated its superiority, with 50% of patients achieving CR in the Tocilizumab group versus 20% in the CYC group. However, no statistical significance was identified ( p = 0.128). The GC dosage at 6 months was significantly lower in the tocilizumab group than in the CYC group [10 (9.4–15) mg/d versus 15 (15–15) mg/d, p = 0.025]. In the CYC group, two patients experienced lumbar vertebral compression fractures related to GCs. Other patients in both groups showed mild adverse effects. Conclusions: Tocilizumab could be a better steroid-sparing agent, with a comparable curative effect and tolerance, than CYC, in the treatment of IgG4-RD.

scholarly journals POS1348 MORTALITY AND ITS RELATED FACTORS IN PATIENTS WITH IGG4-RELATED DISEASE: A JAPANESE SINGLE-CENTER STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 956-957
Author(s):  
I. Mizushima ◽  
H. Kawahara ◽  
T. Yoshinobu ◽  
S. Shin ◽  
R. Hoshiba ◽  
...  
Keyword(s):  
Mortality Rate ◽  
Cox Regression ◽  
Clinical Course ◽  
Regression Analyses ◽  
Related Factors ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Crude Mortality Rate ◽  
Crude Mortality

Background:In recent years, IgG4-related disease (IgG4-RD) has become a widely recognized disorder. However, mortality and its related factors in this disease are not well known.Objectives:This study aimed to clarify mortality and its related factors in patients with IgG4-RD.Methods:We retrospectively reviewed the medical records of patients with IgG4-RD diagnosed by experts based on fulfillment of the Japanese comprehensive diagnostic criteria and/or the 2019 ACR/EULAR classification criteria for IgG4-RD at a single center in Japan. Using the collected data, we calculated the crude mortality rate and the standardized mortality ratio (SMR) using national Japan mortality statistics and investigated the cause of death. We performed Cox regression analyses to assess mortality-related factors.Results:A total of 179 patients with IgG4-RD were included: 124 were male (69.3%); the median age was 68 years (interquartile range [IQR] 60-75 years); and the median follow-up from diagnosis was 47 months (IQR 17-84). Ten patients (5.6%) in our cohort died during the follow-up period. Five died of malignancy, one of respiratory failure, two of infectious pneumonia, one of sudden cardiac event, and one of suspected aortic aneurysmal rupture. The crude mortality rate was 11.1 per 1,000 person-years. According to national Japan mortality statistics, 11.6 age- and sex-matched deaths were expected to occur within the follow-up period, resulting in a SMR of 0.86 (95% confidence interval [CI] 0.41-1.59). Univariate Cox regression analyses indicated that the number of affected organs at diagnosis (hazard ratio [HR] 1.45, 95% CI 1.02-2.05), serum creatinine levels at diagnosis (HR 1.82, 95% CI 1.06-3.12), and the presence of malignancy during the clinical course (HR 3.93, 95% CI 1.10-14.02) had a significant impact on the time to death, whereas the other factors including age at diagnosis and serum C-reactive protein and IgG4 levels at diagnosis did not.Conclusion:Our findings suggest that the mortality rate of patients with IgG4-RD does not significantly differ from that of the Japanese general population. Multi-organ involvement and renal dysfunction at diagnosis as well as malignancy during the clinical course may be associated with higher mortality. An appropriate clinical evaluation for the early detection of these risk factors is required at first diagnosis and during long-term follow-up.Disclosure of Interests:None declared

IgG4-aortitis among thoracic aortic aneurysms

Heart ◽  
2019 ◽  
Vol 105 (20) ◽  
pp. 1583-1589 ◽  
Author(s):  
Carlos Nicolás Pérez-García ◽  
Carmen Olmos ◽  
David Vivas ◽  
Carlos Ferrera ◽  
Daniel García-Arribas ◽  
...  
Keyword(s):  
Giant Cell ◽  
Fdg Pet ◽  
Clinical Course ◽  
Aortic Aneurysms ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Pet Ct ◽  
Disease Extension ◽  
Fdg Pet Ct

ObjectiveThe incidence of aortitis in patients with thoracic aortic diseases is not well established. The aim of this study was to analyse the frequency and clinical course of patients with aortitis in a surgical series.Methods320 consecutive patients with ascending aorta/aortic arch aneurysm or acute aortic syndrome who underwent surgery from 2012 to 2017 in a single tertiary referral hospital were retrospectively reviewed. Epidemiological data, clinical course and variables related to diagnosis, treatment and follow-up were collected from patients with histologically proven aortitis.ResultsFrom 320 examined aortic samples, 279 (87.2%) thoracic aneurysms and 41 acute aortic syndromes (12.8%), 9 (2.8%) were aortitis: 3 cases of Takayasu’s arteritis, 3 of IgG4-related aortitis, 2 of giant cell, and 1 classified as idiopathic. Median age at surgery was 53.4 (51–69.2) years and six cases were female. Seven patients presented with non-specific symptoms and the diagnosis was made at pathology. Surgery was elective in eight patients and emergent in one case of IgG4-related aortitis. 18F-fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) was performed for disease extension study and as a monitoring technique during the follow-up of five patients, with just one case performed presurgically. All the patients with IgG4-related disease showed extrathoracic aortic involvement. There were no deaths, neither in-hospital nor during the 1.7 years of median follow-up.ConclusionsIn surgically treated thoracic aorta pathology, the frequency of aortitis is low; IgG4-related disease is among the most common aetiologies with a frequency similar to other types of aortitis, such as Takayasu’s and giant cell arteritis, and clinical manifestations are non-specific making presurgical diagnosis difficult. 18F-FDG PET/CT allows a better assessment of disease extension and therapeutic response. Surgery can be successfully performed and corticosteroid therapy ensures a good mid-term follow-up.

scholarly journals Lung nodules and IgG4 related disease: a single-center based experience

2020 ◽  
Author(s):  
Yan Xie ◽  
Anji Xiong ◽  
Tony Marion ◽  
Yi Liu
Keyword(s):  
Lung Diseases ◽  
Immunosuppressive Agents ◽  
Ground Glass Opacity ◽  
Lung Nodules ◽  
Bronchial Wall ◽  
West China Hospital ◽  
West China ◽  
Related Disease ◽  
Igg4 Related Disease

Abstract Background: This study was undertaken in an attempt to characterize the frequency and clinical features of lung nodules in IgG4 related disease (IgG4-RD) patients as an insight for help with the diagnosis of lung nodules.Methods: A retrospective study was carried out in West China Hospital, Sichuan University from January 2012 to December 2018, 89 patients with definite IgG4-RD were enrolled.Results: Fifty of 89 patients with definite IgG4-RD had radiologically confirmed lung nodules, 6 of whom were diagnosed with definite IgG4 related lung disease. Lung nodules detected in more than 40 patients were small and solid, always with regular margins. Multiple (41/50) and bilateral (34/50) distributions was also a major characteristic of these lung nodules. Lobulation and speculation were simultaneously detected in 3 patients, including 2 patients combined with pleural indentation. Calcification of nodules was detected in only one patient. Thirty-seven patients also had additional radiological abnormalities of lungs, including ground-glass opacity (21/50), thickening of pleura (9/50), thickening of interlobular septa (4/50), thickening of bronchial wall (3/50), pleural effusion (4/50), mass (3/50), interstitial changes (5/50), and mediastinal or hilar lymphadenopathy (32/50). Most patients (44/50) were treated with glucocorticoids alone or combined with immunosuppressive agents. Sixteen patients received a re-examination by chest computed tomography (CT) scan after treatment, 10 of whom showed a decrease in the size and/or the number of nodules.Conclusions: The incidence of lung nodules in IgG4-RD patients can be high. For an IgG4-RD patient with lung nodules, the possibility that the lung nodules related to IgG4-RLD is high. It is hard to differentiate IgG4 related lung nodules from other lung diseases, in particular, lung cancer. Radiological characteristics and positive responses to glucocorticoids and immunosuppressive agents can help with the differential diagnosis. For these patients, regular follow-up is also important.

Bilateral Orbital IgG4-Related Disease with Systemic and Corneal Involvement Showing an Excellent Response to Steroid and Rituximab Therapy: Report of a Case with 11 Years Follow-Up

Orbit ◽  
2015 ◽  
Vol 34 (5) ◽  
pp. 299-301 ◽  
Author(s):  
Agnes I. Berta ◽  
Abbas Agaimy ◽  
Joachim M. Braun ◽  
Bernhard Manger ◽  
Friedrich E. Kruse ◽  
...  
Keyword(s):  
Excellent Response ◽  
Related Disease ◽  
Igg4 Related Disease
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