scholarly journals Prevalence of IgG4-Related Disease in Japan Based on Nationwide Survey in 2009

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Kazushige Uchida ◽  
Atsushi Masamune ◽  
Tooru Shimosegawa ◽  
Kazuichi Okazaki
Keyword(s):  
Confidence Interval ◽  
Autoimmune Pancreatitis ◽  
Retroperitoneal Fibrosis ◽  
Disease Onset ◽  
Nationwide Survey ◽  
Female Ratio ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Number Of Patients ◽  
Male Female Ratio

The number of patients with autoimmune pancreatitis who visited hospitals in Japan in 2007 was approximately 2709 (95% confidence interval; range 2540–3040). Because IgG4-related disease is a new clinical entity, there are no data with regard to its prevalence. To estimate the number of patients with IgG4-related disease in Japan, we randomly selected hospitals using stratification and asked them how many patients they had with IgG4-related disease in 2009. The number of patients with Mikulicz’s disease, IgG4-related retroperitoneal fibrosis, IgG4-related renal disease, IgG4-related pulmonary disease, and IgG4-related lymphadenopathy who visited hospitals in Japan in 2009 was approximately 4304 (95% confidence interval; range 3360–5048), 272 (95% confidence interval; range 264–306), 57 (95% confidence interval; range 47–66), 354 (95% confidence interval; range 283–424), and 203 (95% confidence interval; range 187–240), respectively. The total number of patients with IgG4-related disease without autoimmune pancreatitis in Japan was approximately 5190 (95% confidence interval; range 4141–6084). The male : female ratio was 1 : 0.77, and the average of age of disease onset was 58.8 years. The total number of patients with IgG4-related disease in Japan in 2009, including autoimmune pancreatitis, was approximately 8000.

scholarly journals Disparities between IgG4-related disease with and without kidney involvement: a case-control study based on 450 patients

2020 ◽  
Author(s):  
Qiaozhu Zeng ◽  
Jingyuan Gao ◽  
Xinyu Zhang ◽  
Aichun Liu ◽  
Zhenfan Wang ◽  
...  
Keyword(s):  
Risk Factors ◽  
Kidney Disease ◽  
Retroperitoneal Fibrosis ◽  
Imaging Finding ◽  
Age At Onset ◽  
Female Ratio ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Kidney Involvement ◽  
Low Serum

Abstract Background We aimed to compare the demographic, clinical and laboratory characteristics between IgG4-related kidney disease (IgG4-RKD+) and extrarenal IgG4-related disease (IgG4-RKD-) in a large Chinese cohort, as well as describing the radiological and pathological features of IgG4-RKD+.Methods We retrospectively analyzed the medical records of 450 IgG4-RD patients at Peking University People’s Hospital from January 2004 to January 2020.Results Among the 450 IgG4-RD patients, 66 were diagnosed with IgG4-RKD+. Compared with IgG4-RKD- patients, IgG4-RKD+ patients had older age at onset and at diagnosis (59 vs 54, P=0.007; 61 vs 56, P=0.002, respectively). Male to female ratio of IgG4-RKD+ patients is significantly higher (2.5:1 vs 1.2:1, P=0.015). In the IgG4-RKD+ group, the most commonly involved organs were salivary gland (54.6%), lymph nodes (47.0%) and pancreas (40.9%). More than three organs involved (OR=6.212), retroperitoneal fibrosis involvement (RPF) (OR=12.460) and low serum C3 level (OR=0.202) were risk factors for the kidney disease in IgG4-RD patients. It was found that renal function was impaired in more than 50% of IgG4-RKD+ patients. The most common imaging finding is multiple, often bilateral, hypodense lesions, followed by ureteric obstruction and hydronephrosis related to RPF, and thickening of the renal pelvic wall. Conclusions Involvement of three or more organs, retroperitoneal fibrosis and low serum C3 level were risk factors for the kidney disease in IgG4-RD patients. These findings indicate potential differences in pathogenesis of these two phenotypes.

Epidemiologic Pictures of Kawasaki Disease in Japan: From the Nationwide Incidence Survey in 1991 and 1992

PEDIATRICS ◽  
1995 ◽  
Vol 95 (4) ◽  
pp. 475-479 ◽  
Author(s):  
Hiroshi Yanagawa ◽  
Mayumi Yashiro ◽  
Yosikazu Nakamura ◽  
Tomisaku Kawasaki ◽  
Hirohisa Kato
Keyword(s):  
Kawasaki Disease ◽  
Disease Onset ◽  
Incidence Rates ◽  
Descriptive Epidemiology ◽  
Female Ratio ◽  
Number Of Patients ◽  
Yearly Incidence ◽  
Male Female Ratio ◽  
History Of ◽  
Questionnaire Form

Objective. Since 1970, twelve nationwide epidemiologic surveys of Kawasaki disease (KD) have been conducted throughout Japan every two years to describe KD in Japan. By the end of 1992, a total of 116 848 cases were reported. This paper summarizes the statistical analysis of the latest survey for the 2-year period from January 1991 through December 1992. Method. A questionnaire form and diagnostic guidelines for KD were sent to all pediatric departments of hospitals with 100 or more beds throughout Japan and information was obtained on patients with KD diagnosed during the 2-year period from January 1991 through December 1992. Results. The summary of the results is: 1) the number of patients reported was 11 221 (6604 males and 4617 females; male/female ratio = 1.43) with a yearly incidence rate of 90 per 100 000 children <5 years old; 2) the monthly number of patients was higher in winter and summer, although the monthly difference was not marked; 3) age-specific incidence rates showed a unimodal peak at 1 year of age; 4) the proportion of patients with a family history of KD in a sibling was 1%; 5) the proportion of recurrent patients was 3%; 6) the proportion of patients with cardiac sequelae 1 month after disease onset was 13%; and 7) the number of patients who died was 9, which conforms to 0.08% of total patients. Conclusion. The incidence rates of KD in Japan are ten times higher than those reported in western countries and almost constant over 6 years. The descriptive epidemiology of the disease, which supports the infection theory, does not change for years.

scholarly journals Identification of serum IFN-α and IL-33 as novel biomarkers for type 1 autoimmune pancreatitis and IgG4-related disease

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Kosuke Minaga ◽  
Tomohiro Watanabe ◽  
Akane Hara ◽  
Ken Kamata ◽  
Shunsuke Omoto ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Characteristic Curve ◽  
Elevated Serum ◽  
Normal Serum ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Number Of Patients ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis

Abstract IgG4-related disease (IgG4-RD) is a multi-organ autoimmune disease characterized by elevated serum IgG4 concentration. Although serum IgG4 concentration is widely used as a biomarker for IgG4-RD and type 1 autoimmune pancreatitis (AIP), a pancreatic manifestation of IgG4-RD, a significant number of patients have normal serum IgG4 levels, even in the active phase of the disease. Recently, we reported that the development of experimental AIP and human type 1 AIP is associated with increased expression of IFN-α and IL-33 in the pancreas. In this study, we assessed the utility of serum IFN-α and IL-33 levels as biomarkers for type 1 AIP and IgG4-RD. Serum IFN-α and IL-33 concentrations in patients who met the diagnostic criteria for definite type 1 AIP and/or IgG4-RD were significantly higher than in those with chronic pancreatitis or in healthy controls. Strong correlations between serum IFN-α, IL-33, and IgG4 concentrations were observed. Diagnostic performance of serum IFN-α and IL-33 concentrations as markers of type 1 AIP and/or IgG4-RD was comparable to that of serum IgG4 concentration, as calculated by the receiver operating characteristic curve analysis. Induction of remission by prednisolone treatment markedly decreased the serum concentration of these cytokines. We conclude that serum IFN-α and IL-33 concentrations can be useful as biomarkers for type 1 AIP and IgG4-RD.

Clinical features of IgG4-related retroperitoneal fibrosis among 407 patients with IgG4-related disease: a retrospective study

Rheumatology ◽  
2020 ◽  
Author(s):  
Yanying Liu ◽  
Lijuan Zhu ◽  
Zhenfan Wang ◽  
Qiaozhu Zeng ◽  
Fei Yang ◽  
...  
Keyword(s):  
Medical Records ◽  
Retroperitoneal Fibrosis ◽  
Disease Onset ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Kidney Involvement ◽  
Peking University ◽  
Male Patients ◽  
The Common

Abstract Objectives IgG4-related disease (IgG4-RD) is recently recognized as a fibro-inflammatory condition featured by tumefactive lesions in multiple organs, and the retroperitoneum is one of the common involved sites. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-RD patients with retroperitoneum lesion (IgG4-RD RPF+) and retroperitoneum free IgG4-RD (IgG4-RD RPF−) in a large cohort. Methods We carried out a retrospective review of the medical records of 407 cases of IgG4-RD diagnosed at Peking University People’s Hospital between March 2009 and May 2019. Results Among 407 patients, 58 had retroperitoneum affected. As compared with IgG4-RD RPF− patients, IgG4-RD RPF+ patients showed older age at disease onset and diagnosis. IgG4-RD RPF+ group involved more male patients. In terms of organ involvement, IgG4-RD RPF+ group was more frequently presented with kidney involvement, while salivary gland, lacrimal gland and pancreas were more prominent in the IgG4-RD RPF− group. In addition, the CRP, ESR level and creatinine level were significantly higher in IgG4-RD RPF+ patients, and hypocomplementemia were more common in this group. Conclusion We have revealed demographic, clinical and laboratory differences between IgG4-RD RPF+ and RPF− patients, which indicated potential differences in pathogenesis and important implications for the diagnosis and management of these two phenotypes.

Nationwide Survey on Kawasaki Disease in Japan

PEDIATRICS ◽  
1987 ◽  
Vol 80 (1) ◽  
pp. 58-62 ◽  
Author(s):  
Hiroshi Yanagawa ◽  
Tomisaku Kawasaki ◽  
Itsuzo Shigematsu
Keyword(s):  
Statistical Analysis ◽  
Kawasaki Disease ◽  
Incidence Rate ◽  
Disease Onset ◽  
Nationwide Survey ◽  
Cumulative Number ◽  
Female Ratio ◽  
Number Of Patients ◽  
Male To Female ◽  
Sibling Cases

Results of the statistical analysis of a nationwide survey of patients with Kawasaki disease diagnosed within a 2-year 6-month period, from July 1982 to December 1984, are as follows. (1) The cumulative number of patients reported by the end of 1984 was 63,399 (36,891 boys and 26,508 girls; male to female ratio 1.4). (2) There were two epidemic years, 1979 and 1982, in which the numbers of patients were more than twofold that of the previous years. (3) A curve plotted for age-specific incidence rate showed a unimodal peak at age 1 year. (4) Steroid therapy was used for 6.3% of the patients, aspirin for 89.8%, antibiotics for 57.6%, and γ-globulin for 11.4%. (5) The proportion of sibling cases was 1.4% and that of recurrent cases was 3.9% of all cases reported. (6) The incidence of cardiac sequelae in 1 month after disease onset was 17.2%.

scholarly journals A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110169
Author(s):  
Kim Rouven Liedtke ◽  
Christoph Käding ◽  
Paula Döring ◽  
Sander Bekeschus ◽  
Anne Susann Glitsch
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Inflammatory Diseases ◽  
Histological Change ◽  
Iliac Vessel ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Cystic Tumors ◽  
Retroperitoneal Cyst ◽  
Retroperitoneal Lymphangioma ◽  
Severe Fibrosis

Several chronic inflammatory diseases have been found to be a subtype of IgG4-related disease, all of which have a typical clinical and histological change, which is based in particular on an overexpression of IgG4 and subsequent fibrosis. At least a part of the retroperitoneal fibrosis, which was originally classified as idiopathic, seems to be assigned to IgG4-related disease. Lymphangiomas are benign, cystic tumors that rarely occur in adults. However, there is no firm association with IgG4-related disease described in the literature to date. This report is about a patient suffering from acute renal failure due to a giant retroperitoneal cyst. Surgical resection remains incomplete in the iliac vessel area due to severe fibrosis and histology revealed features of both lymphangioma and IgG4+ fibrosis. The case description is followed by a brief overview of IgG4-related disease and a consideration of whether lymphangiomas might be assigned to this topic.

scholarly journals Disparities between IgG4-related kidney disease and extrarenal IgG4-related disease in a case–control study based on 450 patients

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Qiaozhu Zeng ◽  
Jingyuan Gao ◽  
Xinyu Zhang ◽  
Aichun Liu ◽  
Zhenfan Wang ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Clinical Laboratory ◽  
Imaging Finding ◽  
Age At Onset ◽  
Female Ratio ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Peking University ◽  
Chinese Cohort ◽  
Male To Female

AbstractWe aimed to compare the demographic, clinical and laboratory characteristics between IgG4-related kidney disease (IgG4-RKD+) and extrarenal IgG4-related disease (IgG4-RKD−) in a large Chinese cohort, as well as describing the radiological and pathological features of IgG4-RKD+. We retrospectively analyzed the medical records of 470 IgG4-related disease (IgG4-RD) patients at Peking University People’s Hospital from January 2004 to January 2020. The demographic, clinical, laboratory, radiological and pathological characteristics between IgG4-RKD+ and IgG4-RKD− were compared. Twenty IgG4-RD patients who had definite etiology of renal impairment including diabetes, hypertension and etc. were excluded. Among the remained 450 IgG4-RD patients, 53 were diagnosed with IgG4-RKD+ . IgG4-RKD+ patients had older age at onset and at diagnosis. Male to female ratio of IgG4-RKD+ patients is significantly higher. In the IgG4-RKD+ group, the most commonly involved organs were salivary gland, lymph nodes and pancreas. It was found that renal function was impaired in approximately 40% of IgG4-RKD+ patients. The most common imaging finding is multiple, often bilateral, hypodense lesions. Male sex, more than three organs involved, and low serum C3 level were risk factors for IgG4-RKD+ in IgG4-RD patients. These findings indicate potential differences in pathogenesis of these two phenotypes.

Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease

2011 ◽  
Vol 46 (3) ◽  
pp. 277-288 ◽  
Author(s):  
Kazuichi Okazaki ◽  
Kazushige Uchida ◽  
Masanori Koyabu ◽  
Hideaki Miyoshi ◽  
Makoto Takaoka
Keyword(s):  
Autoimmune Pancreatitis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Recent Advances
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