scholarly journals A Case of IgG4-related Sclerosing Cholangitis with a Normal Serum IgG4 Level: Report of a Case

2012 ◽  
Vol 79 (5) ◽  
pp. 367-372 ◽  
Author(s):  
Satoshi Mizutani ◽  
Hideyuki Suzuki ◽  
Hiroshi Yoshida ◽  
Yasuo Arima ◽  
Yasuhiko Kitayama ◽  
...  
Keyword(s):  
Sclerosing Cholangitis ◽  
Normal Serum ◽  
Serum Igg4 Level ◽  
Serum Igg4 ◽  
Igg4 Level

scholarly journals Clinical Characteristics of 76 Patients with IgG4-Related Hypophysitis: A Systematic Literature Review

2019 ◽  
Vol 2019 ◽  
pp. 1-10
Author(s):  
Yujuan Li ◽  
Hua Gao ◽  
Zhen Li ◽  
Xinxin Zhang ◽  
Yizhi Ding ◽  
...  
Keyword(s):  
Clinical Features ◽  
Case Reports ◽  
Disease Onset ◽  
Elevated Serum ◽  
Median Number ◽  
Normal Serum ◽  
Hormone Deficiency ◽  
Serum Igg4 Level ◽  
Serum Igg4 ◽  
Igg4 Level

Background. IgG4-related hypophysitis (IgG4-RH) is a rare disease, and its prevalence remains unclear. In recent years, an increasing number of cases have been reported because of the increasing recognition of this disease. We aimed to summarize case reports of IgG4-RH and outline the clinical features and outcomes. Methods. We performed PubMed search of articles using the search terms “hypophysitis [AND] IgG4.” Consequently, only 54 English articles (76 cases) met Leporati’s diagnostic criteria. Results. Of the 76 cases, the ratio of men to women was 1.5 : 1, and the age at diagnosis was 54.1 ± 17.8 years. The median IgG4 concentration was 405.0 mg/dl. Anterior hypopituitarism, isolated central diabetes insipidus, and panhypopituitarism were observed in 14 (18.4%), 12 (15.8%), and 44 (57.9%) cases, respectively. The sequence of anterior hormone deficiency was as follows: gonadotropin (68.4%), ACTH (63.2%), TSH (59.2%), GH (48.7%), and prolactin (42.1%). The median number of involved organs was 1.5, and the lung (18.4%), retroperitoneum (17.1%), kidney (15.8%), submandibular glands (14.5%), and pancreas (13.2%) were the common involved organs. Elevated IgG4 concentration and normal IgG4 level were in 42 (76.4%) and 13 (23.6%) cases, respectively. Patients with elevated serum IgG4 concentration were older (60.9 ± 14.3 vs 45.6 ± 17.4, p=0.001) and male-prone (78.6% vs 40.4%, p=0.003) and had a susceptibility of multiple organ involvement (78.6% vs 35.0%, p=0.001) compared to those with normal serum IgG4 levels. Males were older at disease onset (61.5 ± 12.6 vs 42.9 ± 18.8, p<0.001) and had a higher IgG4 concentration (425.0 vs 152.5, p=0.029) and a greater number of involved organs (2.0 vs 0.0, p=0.001), while isolated hypophysitis was more prominent in female (63.3% vs 26.1%, p=0.001). Conclusion. In this review, we found that there were different characteristics between different genders. Patients with elevated serum IgG4 level in terms of some clinical features were also different from those with normal serum IgG4 level. However, the data in this review were limited by bias and confounding. Further clinical studies with larger sample sizes are warranted.

scholarly journals IgG4-Related Kidney Disease: A Curious Case of Interstitial Nephritis with Hypocomplementemia

2019 ◽  
Vol 9 (2) ◽  
pp. 49-54
Author(s):  
Emmett Tsz Yeung Wong ◽  
Manjari Lahiri ◽  
Ming Teh ◽  
Christopher Cheang Han Leo
Keyword(s):  
Kidney Disease ◽  
Interstitial Nephritis ◽  
Elevated Serum ◽  
Kidney Injury ◽  
Acute Interstitial Nephritis ◽  
Clinical Syndrome ◽  
Normal Serum ◽  
Serum Igg4 Level ◽  
Serum Igg4 ◽  
Igg4 Level

IgG4-related kidney disease has been relatively newly recognized over the last two decades as a combination of an autoimmune and allergic disorder, with elevated serum IgG4 level and hypocomplementemia among its characteristic features. Here we report the case of a man with interstitial nephritis presenting with acute kidney injury and hypocomplementemia but normal serum IgG4 level and provide a literature review of IgG4-related kidney disease. This case highlights the importance of IgG4-related kidney disease as an important differential diagnosis in any patient presenting with a clinical syndrome mimicking acute interstitial nephritis with hypocomplementemia. A high index of suspicion with a low threshold for performing a native kidney biopsy would be paramount as patients do respond well to corticosteroid therapy.

scholarly journals Usefulness of measuring serum IgG4 level as diagnostic and treatment marker in IgG4-related disease

2012 ◽  
Vol 35 (1) ◽  
pp. 30-37 ◽  
Author(s):  
Motohisa YAMAMOTO ◽  
Norihiro NISHIMOTO ◽  
Tetsuya TABEYA ◽  
Yasuyoshi NAISHIRO ◽  
Keisuke ISHIGAMI ◽  
...  
Keyword(s):  
Serum Igg4 Level ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

scholarly journals Low serum IgG4 level: a potential diagnostic biomarker for IgA nephropathy

2021 ◽  
Vol 9 (9) ◽  
pp. 781-781
Author(s):  
Xinyu Tian ◽  
Zhuan Cui ◽  
Song Wang ◽  
Yuejuan Pan ◽  
Lata A ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Diagnostic Biomarker ◽  
Serum Igg4 Level ◽  
Serum Igg4 ◽  
Igg4 Level ◽  
Low Serum

scholarly journals Relapse predictors and serologically unstable condition of IgG4-related disease: a large Chinese cohort

Rheumatology ◽  
2020 ◽  
Vol 59 (8) ◽  
pp. 2115-2123 ◽  
Author(s):  
Yanying Liu ◽  
Qiaozhu Zeng ◽  
Lijuan Zhu ◽  
Jingyuan Gao ◽  
Ziqiao Wang ◽  
...  
Keyword(s):  
Risk Factors ◽  
Relapse Rate ◽  
Unstable Condition ◽  
Serum Igg4 Level ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Younger Age ◽  
Serum Igg4 ◽  
History Of ◽  
Igg4 Level

Abstract Objectives Patients with IgG4-related disease (IgG4-RD) typically respond well to initial glucocorticoid therapy, but always relapse with tapered or maintenance dosage of steroid. We aimed to identify the risk factors for relapse of IgG4-RD and explore the impact of active intervention on the serologically unstable condition. Methods We performed a retrospective study of 277 IgG4-RD patients at Peking University People’s Hospital from February 2012 through February 2019. They were all followed for &gt;4 months. The primary outcome was patient relapse. Data on recurrence of IgG4-RD symptoms, laboratory and image findings were recorded, along with information on treatment in the serologically unstable condition. Results The cumulative relapse rate was 12.86%, 27.84% and 36.1% at 12, 24 and 36 months, respectively. Younger age at onset, younger age at diagnosis, longer time from diagnosis to treatment and history of allergy were associated with relapse. Identified independent risk factors were longer time from diagnosis to treatment and history of allergy. When serum IgG4 level was 20%, 50% or 100% higher than that of the remission period, similar percentages of patients finally relapsed, regardless of whether they were in the immunosuppression intensified or non-intensified group. Median duration from serum IgG4 level instability to relapse in the intensified and non-intensified group was not statistically different. Conclusion The risk factors of relapse were longer time from diagnosis to treatment and history of allergy. Intervention in the serologically unstable condition was not helpful for reducing relapse rate.

scholarly journals Isolated IgG4‐related sclerosing cholangitis with normal serum IgG4 levels—A case report

2020 ◽  
Vol 8 (11) ◽  
pp. 2186-2190
Author(s):  
Yanyun Shu ◽  
Juanjuan Cheng ◽  
Jin Ye ◽  
Xiaoli Pan
Keyword(s):  
Case Report ◽  
Sclerosing Cholangitis ◽  
Normal Serum ◽  
Serum Igg4

scholarly journals Spectrum of Disorders Associated with Elevated Serum IgG4 Levels Encountered in Clinical Practice

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Jay H. Ryu ◽  
Ryohei Horie ◽  
Hiroshi Sekiguchi ◽  
Tobias Peikert ◽  
Eunhee S. Yi
Keyword(s):  
Clinical Practice ◽  
Wide Spectrum ◽  
Systemic Vasculitis ◽  
Elevated Serum ◽  
Serum Igg4 Level ◽  
Igg4 Related Disease ◽  
Clinical Presentations ◽  
Serum Igg4 ◽  
Disease Associations ◽  
Igg4 Level

IgG4-related disease (IgG4-RD) is a recently described systemic fibroinflammatory disease associated with elevated circulating levels of IgG4 and manifests a wide spectrum of clinical presentations. Although serum IgG4 level has been described to be the most sensitive and specific laboratory test for the diagnosis of IgG4-RD, it is recognized that an elevated serum IgG4 level can be encountered in other diseases. In this study, we sought to identify the frequency of IgG4-RD and other disease associations in patients with elevated serum IgG4 levels seen in clinical practice. Among 3,300 patients who underwent IgG subclass testing over a 2-year period from January 2009 to December 2010, 158 (4.8%) had an elevated serum IgG4 level (>140 mg/dL). IgG4 subclass testing was performed for evaluation of suspected IgG4-RD or immunodeficiency. Twenty-nine patients (18.4%) had definite or possible IgG4-RD. Among those patients without IgG4-RD, a broad spectrum of biliary tract, pancreatic, liver, and lung diseases, as well as systemic vasculitis, was diagnosed. We conclude that patients with elevated serum IgG4 levels encountered in clinical practice manifest a wide array of disorders, and only a small minority of them has IgG4-RD.

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