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

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

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Clues To Mortality Trends And Their Related Factors In IgG4-Related Disease: A Japanese Single-Center Retrospective Study

10.21203/rs.3.rs-1063841/v2 ◽

2021 ◽

Author(s):

Hiroyuki Kawahara ◽

Ichiro Mizushima ◽

Shunsuke Tsuge ◽

Seung Shin ◽

Takahiro Yoshinobu ◽

...

Keyword(s):

Cox Regression ◽

Organ Involvement ◽

Regression Analyses ◽

Mortality Trends ◽

Related Factors ◽

Crude Mortality Rate ◽

Crude Mortality ◽

Long Term Prognosis

Abstract Background: Few observations on the long-term prognosis have been conducted in immunoglobulin G4-related disease (IgG4-RD) patients with various organ involvement, not limited to autoimmune pancreatitis. Especially, mortality and its related factors in patients with IgG4-RD with various organ involvement are not well known. This study aimed to clarify mortality trends and its related factors in IgG4-RD with various organ involvement.Methods: We retrospectively reviewed the medical records of patients with IgG4-RD at a single center in Japan. 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 and the median follow-up from diagnosis was 47 months (IQR 19-96). Ten patients (5.6%) in our cohort died during the follow-up period. 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 would have been expected to occur within the follow-up period, resulting in an 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), eGFR <45 mL/min/1.73m2 at diagnosis (vs. ≥45, HR 8.48, 95% CI 2.42-29.79), 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.Conclusions: Our findings suggested that IgG4-RD does not significantly affect long-term patient survival. On the other hand, multi-organ involvement and renal dysfunction as well as malignancy might be associated with higher mortality trends in IgG4-RD. Early detection and appropriate management of risk factors may improve the long-term prognosis of IgG4-RD.

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Clues to Mortality Trends and Their Related Factors in IgG4-related Disease: a Japanese Single-center Retrospective Study

10.21203/rs.3.rs-1063841/v1 ◽

2021 ◽

Author(s):

Hiroyuki Kawahara ◽

Ichiro Mizushima ◽

Shunsuke Tsuge ◽

Seung Shin ◽

Takahiro Yoshinobu ◽

...

Keyword(s):

Cox Regression ◽

Organ Involvement ◽

Regression Analyses ◽

Mortality Trends ◽

Related Factors ◽

Crude Mortality Rate ◽

Crude Mortality ◽

Long Term Prognosis

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IgG4-aortitis among thoracic aortic aneurysms

Heart ◽

10.1136/heartjnl-2018-314499 ◽

2019 ◽

Vol 105 (20) ◽

pp. 1583-1589 ◽

Cited By ~ 3

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.

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The prevalence, mortality, and associated risk factors for developing COVID-19 in hip fracture patients: a systematic review and meta-analysis

Bone and Joint Research ◽

10.1302/2046-3758.912.bjr-2020-0473.r1 ◽

2020 ◽

Vol 9 (12) ◽

pp. 873-883

Author(s):

Nicholas D. Clement ◽

Nathan Ng ◽

Cameron J. Simpson ◽

Robyn F. L. Patton ◽

Andrew James Hall ◽

...

Keyword(s):

Systematic Review ◽

Hip Fracture ◽

Mortality Rate ◽

Meta Analysis ◽

Crude Mortality Rate ◽

Crude Mortality ◽

Increased Risk ◽

Associated Risk Factors ◽

Reported Data

Aims The aims of this meta-analysis were to assess: 1) the prevalence of coronavirus disease 2019 (COVID-19) in hip fracture patients; 2) the associated mortality rate and risk associated with COVID-19; 3) the patient demographics associated with COVID-19; 4) time of diagnosis; and 5) length of follow-up after diagnosis of COVID-19. Methods Searches of PubMed, Medline, and Google Scholar were performed in October 2020 in line with the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) statement. Search terms included “hip”, “fracture”, and “COVID-19”. The criteria for inclusion were published clinical articles reporting the mortality rate associated with COVID-19 in hip fracture patients. In total, 53 articles were identified and following full text screening 28 articles satisfied the inclusion criteria. Results A total of 28 studies reported the mortality of COVID-19-positive patients, of which 21 studies reported the prevalence of COVID-19-positive patients and compared the mortality rate to COVID-19-negative patients. The prevalence of COVID-19 was 13% (95% confidence interval (CI) 11% to 16%) and was associated with a crude mortality rate of 35% (95% CI 32% to 39%), which was a significantly increased risk compared to those patients without COVID-19 (odds ratio (OR) 7.11, 95% CI 5.04 to 10.04; p < 0.001). COVID-19-positive patients were more likely to be male (OR 1.51, 95% CI 1.16 to 1.96; p = 0.002). The duration of follow-up was reported in 20 (71.4%) studies. A total of 17 studies reported whether a patient presented with COVID-19 (n = 108 patients, 35.1%) or developed COVID-19 following admission (n = 200, 64.9%), of which six studies reported a mean time to diagnosis of post-admission COVID-19 at 15 days (2 to 25). Conclusion The prevalence of COVID-19 was 13%, of which approximately one-third of patients were diagnosed on admission, and was associated with male sex. COVID-19-positive patients had a crude mortality rate of 35%, being seven times greater than those without COVID-19. Due to the heterogenicity of the reported data minimum reporting standards of outcomes associated with COVID-19 are suggested. Cite this article: Bone Joint Res 2020;9(12):873–883.

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

Journal of Clinical Medicine ◽

10.3390/jcm10061329 ◽

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.

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Factors affecting the course of body and kidney growth in infants with urolithiasis: A critical long-term evaluation

Archivio Italiano di Urologia e Andrologia ◽

10.4081/aiua.2016.4.249 ◽

2016 ◽

Vol 88 (4) ◽

pp. 249 ◽

Cited By ~ 4

Author(s):

Kemal Sarica ◽

Fatma Narter ◽

Kubilay Sabuncu ◽

Ahmet Akca ◽

Utku Can ◽

...

Keyword(s):

Breast Feeding ◽

Clinical Course ◽

Stone Disease ◽

The Body ◽

Renal Growth ◽

Related Factors ◽

Growth Status ◽

Long Term Follow Up

Objective: To investigate the possible effects of dietary, patient and stone related factors on the clinical course of the stone disease as well as the body and renal growth status of the infants. Patients and Methods: A total of 50 children with an history of stone disease during infancy period were studied. Patient (anatomical abnormalities, urinary tract infection - UTI, associated morbidities), stone (obstruction, UTI and required interventions) and lastly dietary (duration of sole breast feeding, formula feeding) related factors which may affect the clinical course of the disease were all evaluated for their effects on the body and renal growth during long-term follow-up. Results: Mean age of the children was 2.40 ± 2.65 years. Our findings demonstrated that infants receiving longer period of breast feeding without formula addition seemed to have a higher rate of normal growth percentile values when compared with the other children. Again, higher frequency of UTI and stone attacks affected the growth status of the infants in a remarkable manner than the other cases. Our findings also demonstrated that thorough a close follow-up and appropriately taken measures; the possible growth retardation as well as renal growth problems could be avoided in children beginning to suffer from stone disease during infancy period. Conclusions: Duration of breast feeding, frequency of UTI, number of stone attacks and stone removal procedures are crucial factors for the clinical course of stone disease in infants that may affect the body as well as kidney growth during long-term follow-up.

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Using Satellite Images and Deep Learning to Identify Associations Between County-Level Mortality and Residential Neighborhood Features Proximal to Schools: A Cross-Sectional Study

Frontiers in Public Health ◽

10.3389/fpubh.2021.766707 ◽

2021 ◽

Vol 9 ◽

Author(s):

Joshua J. Levy ◽

Rebecca M. Lebeaux ◽

Anne G. Hoen ◽

Brock C. Christensen ◽

Louis J. Vaickus ◽

...

Keyword(s):

United States ◽

Deep Learning ◽

Mortality Rate ◽

Satellite Images ◽

The United States ◽

Mortality Rates ◽

Image Features ◽

County Level ◽

Crude Mortality Rate ◽

Crude Mortality

What is the relationship between mortality and satellite images as elucidated through the use of Convolutional Neural Networks?Background: Following a century of increase, life expectancy in the United States has stagnated and begun to decline in recent decades. Using satellite images and street view images, prior work has demonstrated associations of the built environment with income, education, access to care, and health factors such as obesity. However, assessment of learned image feature relationships with variation in crude mortality rate across the United States has been lacking.Objective: We sought to investigate if county-level mortality rates in the U.S. could be predicted from satellite images.Methods: Satellite images of neighborhoods surrounding schools were extracted with the Google Static Maps application programming interface for 430 counties representing ~68.9% of the US population. A convolutional neural network was trained using crude mortality rates for each county in 2015 to predict mortality. Learned image features were interpreted using Shapley Additive Feature Explanations, clustered, and compared to mortality and its associated covariate predictors.Results: Predicted mortality from satellite images in a held-out test set of counties was strongly correlated to the true crude mortality rate (Pearson r = 0.72). Direct prediction of mortality using a deep learning model across a cross-section of 430 U.S. counties identified key features in the environment (e.g., sidewalks, driveways, and hiking trails) associated with lower mortality. Learned image features were clustered, and we identified 10 clusters that were associated with education, income, geographical region, race, and age.Conclusions: The application of deep learning techniques to remotely-sensed features of the built environment can serve as a useful predictor of mortality in the United States. Although we identified features that were largely associated with demographic information, future modeling approaches that directly identify image features associated with health-related outcomes have the potential to inform targeted public health interventions.

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Analysis of death causes of residents in poverty-stricken Areas in 2020: take Liangshan Yi Autonomous Prefecture in China as an example

BMC Public Health ◽

10.1186/s12889-022-12504-6 ◽

2022 ◽

Vol 22 (1) ◽

Author(s):

Rujun Liao ◽

Lin Hu ◽

Qiang Liao ◽

Tianyu Zhu ◽

Haiqun Yang ◽

...

Keyword(s):

Mortality Rate ◽

Life Expectancy ◽

Malignant Tumors ◽

Heart Diseases ◽

Health Policies ◽

Years Of Life Lost ◽

Crude Mortality Rate ◽

Crude Mortality ◽

Standardized Mortality Rate ◽

Death Causes

Abstract Background Continuous surveillance of death can measure health status of the population, reflect social development of a region, thus promote health service development in the region and improve the health level of local residents. Liangshan Yi Autonomous Prefecture was a poverty-stricken region in Sichuan province, China. While at the end of 2020, as the announcement of its last seven former severely impoverished counties had shaken off poverty, Liangshan declared victory against poverty. Since it is well known that the mortality and cause of death structure will undergo some undesirable changes as the economy develops, this study aimed to reveal the distribution of deaths, as well as analyze the latest mortality and death causes distribution characteristics in Liangshan in 2020, so as to provide references for the decision-making on health policies and the distribution of health resources in global poverty-stricken areas. Methods Liangshan carried out the investigation on underreporting deaths among population in its 11 counties in 2018, and combined with the partially available data from underreporting deaths investigation data in 2020 and the field experience, we have estimated the underreporting rates of death in 2020 using capture-recapture (CRC) method. The crude mortality rate, age-standardized mortality rate, proportion and rank of the death causes, potential years of life lost (PYLL), average years of life lost (AYLL), potential years of life lost rate (PYLLR), standardized potential years of life lost (SPYLL), premature mortality from non-communicable diseases (premature NCD mortality), life expectancy and cause-eliminated life expectancy were estimated and corrected. Results In 2020, Liangshan reported a total of 16,850 deaths, with a crude mortality rate of 608.75/100,000 and an age-standardized mortality rate of 633.50/100,000. Male mortality was higher than female mortality, while 0-year-old mortality of men was lower than women’s. The former severely impoverished counties’ age-standardized mortality and 0-year-old mortality were higher than those of the non-impoverished counties. The main cause of death spectrum was noncommunicable diseases (NCDs), and the premature NCD mortality of four major NCDs were 14.26% for the overall population, 19.16% for men and 9.27% for women. In the overall population, the top five death causes were heart diseases (112.07/100,000), respiratory diseases (105.85/100,000), cerebrovascular diseases (87.03/100,000), malignant tumors (73.92/100,000) and injury (43.89/100,000). Injury (64,216.78 person years), malignant tumors (41,478.33 person years) and heart diseases (29,647.83 person years) had the greatest burden on residents in Liangshan, and at the same time, the burden of most death causes on men were greater than those on women. The life expectancy was 76.25 years for overall population, 72.92 years for men and 80.17 years for women, respectively, all higher than the global level (73.3, 70.8 and 75.9 years). Conclusions Taking Liangshan in China as an example, this study analyzed the latest death situation in poverty-stricken areas, and proposed suggestions on the formulation of health policies in other poverty-stricken areas both at home and abroad.

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QOLP-08. THE LANDSCAPE OF EPILEPSY ASSOCIATED WITH DIFFUSELY INFILTRATIVE GLIOMAS

Neuro-Oncology ◽

10.1093/neuonc/noab196.729 ◽

2021 ◽

Vol 23 (Supplement_6) ◽

pp. vi184-vi184

Author(s):

Michael Drumm ◽

Jessica Templer ◽

Omar Bushara ◽

Dusten Unruh ◽

Jordain Walshon ◽

...

Keyword(s):

Tumor Progression ◽

Similar Pattern ◽

Cox Regression ◽

Idh Mutation ◽

Prior Work ◽

Regression Analyses ◽

Course Of Disease ◽

Kaplan Meier ◽

Extent Of Surgical Resection

Abstract Seizures are among the most prevalent co-morbidities associated with glioma, and pose a serious threat to patients. Our prior work showed that IDH mutation (IDHmut) was associated with much greater seizure frequency at the time of initial glioma diagnosis. However, less is known about the variables that contribute to seizure risk throughout the course of disease. We therefore collected data from 247 patients with grade 2–4 glioma, and determined seizure risk using Kaplan-Meier survival probabilities and multivariable cox regression analyses. Median follow-up of IDH wildtype (IDHwt) and IDHmut glioma patients was 15 months and 36 months, respectively. Incidence of pre-operative seizures for IDHwt and IDHmut patients was 75/168 (45%) and 60/79 (76%), and incidence of post-operative seizures was 70/168 (42%) and 43/79 (54%), respectively. Patients who had a pre-operative seizure had a shorter time to their first post-operative seizure than patients who never had a pre-operative seizure in both IDHwt (P< 0.0001) and IDHmut (P= 0.039) cohorts. Among IDHmut glioma patients, those with subtotal resections developed post-operative seizures faster (median time to first seizure= 9.9 months) than those with gross-total resections (median not reached) (P= 0.0005), but a similar pattern was not observed in IDHwt glioma patients (P= 0.20). Those with IDHmut astrocytomas more quickly developed post-operative seizures (median= 11.1 months), compared to those with IDHwt astrocytomas (24.9 months) or IDHmut oligodendrogliomas (median not reached) (P= 0.033). Tumor progression closely followed post-operative seizures in patients with IDHwt gliomas when either their first post-operative seizure occurred longer than 6 months following resection, or when their post-operative seizures worsened in quality. These data suggest the best predictors of post-operative seizures are as follows: the presence of pre-operative seizures; extent of surgical resection; IDHmut status. These data will help clinicians better manage glioma patients by identifying those at greatest risk of seizures.

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

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.1983 ◽

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

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