P53: SERUM IGG4 LEVEL AND ASSOCIATED AUTOANTIBODIES: A 10‐YEAR RETROSPECTIVE REVIEW

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 >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.

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Comment to “Characteristics of autoimmune pancreatitis based on serum IgG4 level”

Digestive and Liver Disease ◽

10.1016/j.dld.2012.04.010 ◽

2012 ◽

Vol 44 (10) ◽

pp. 884

Author(s):

Ersan Ozaslan

Keyword(s):

Autoimmune Pancreatitis ◽

Serum Igg4 Level ◽

Serum Igg4 ◽

Igg4 Level

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Hepatobiliary and Pancreatic: Pancreatic cancer with elevated serum IgG4 level due to multiple myeloma mimicking localized autoimmune pancreatitis

Journal of Gastroenterology and Hepatology ◽

10.1111/jgh.14088 ◽

2018 ◽

Vol 33 (7) ◽

pp. 1310-1310 ◽

Cited By ~ 3

Author(s):

S Kato ◽

M Kuwatani ◽

K Kawakubo ◽

R Sugiura ◽

K Hirata ◽

...

Keyword(s):

Pancreatic Cancer ◽

Multiple Myeloma ◽

Autoimmune Pancreatitis ◽

Elevated Serum ◽

Serum Igg4 Level ◽

Serum Igg4 ◽

Igg4 Level

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

International Journal of Rheumatology ◽

10.1155/2012/232960 ◽

2012 ◽

Vol 2012 ◽

pp. 1-6 ◽

Cited By ~ 54

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

International Journal of Endocrinology ◽

10.1155/2019/5382640 ◽

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.

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Serum Immunoglobulin G Fraction 4 Levels in Pancreatic Cancer: Elevations Not Associated With Autoimmune Pancreatitis

Archives of Pathology & Laboratory Medicine ◽

10.5858/2008-132-48-sigfli ◽

2008 ◽

Vol 132 (1) ◽

pp. 48-53 ◽

Cited By ~ 8

Author(s):

Amit Raina ◽

Alyssa M. Krasinskas ◽

Julia B. Greer ◽

Janette Lamb ◽

Erin Fink ◽

...

Keyword(s):

Pancreatic Cancer ◽

Cancer Patients ◽

Immunoglobulin G ◽

Autoimmune Pancreatitis ◽

Elevated Serum ◽

Serum Immunoglobulin ◽

Pancreatic Mass ◽

Serum Igg4 Level ◽

Serum Igg4 ◽

Igg4 Level

Abstract Context.—Autoimmune pancreatitis is an uncommon, inflammatory disease of the pancreas that presents with clinical features, such as painless jaundice and a pancreatic mass, similar to those caused by pancreatic cancer. Patients with autoimmune pancreatitis frequently have elevated serum immunoglobulin G fraction 4 (IgG4) levels, and their pancreatic tissue may show IgG4-positive plasma cell infiltration. It is imperative to differentiate autoimmune pancreatitis from pancreatic cancer because autoimmune pancreatitis typically responds to corticosteroid treatment. A previous Japanese study reported that serum IgG4 greater than 135 mg/dL was 97% specific and 95% sensitive in predicting autoimmune pancreatitis. Objective.—To prospectively measure serum IgG4 levels in pancreatic cancer patients to ascertain whether increased levels might be present in this North American population. Design.—We collected blood samples and phenotypic information on 71 consecutive pancreatic cancer patients and 103 healthy controls who visited our clinics between October 2004 and April 2006. IgG4 levels were determined using a single radial immunodiffusion assay. A serum IgG4 level greater than 135 mg/dL was considered elevated. Results.—Five cancer patients had IgG4 elevation, with a mean serum IgG4 level of 160.8 mg/dL. None of our cancer patients with plasma IgG4 elevation demonstrated evidence of autoimmune pancreatitis. One control subject demonstrated elevated serum IgG4 unrelated to identified etiology. Conclusions.—As many as 7% of patients with pancreatic cancer have serum IgG4 levels above 135 mg/dL. In patients with pancreatic mass lesions and suspicion of cancer, an IgG4 level measuring between 135 and 200 mg/dL should be interpreted cautiously and not accepted as diagnostic of autoimmune pancreatitis without further evaluation.

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Abstract 12071: Serum Immunoglobulin G4 Level is an Independent Predictor of Major Adverse Cardiovascular Events After Percutaneous Coronary Intervention over Long-Term Follow-Up

Circulation ◽

10.1161/circ.132.suppl_3.12071 ◽

2015 ◽

Vol 132 (suppl_3) ◽

Author(s):

Aiko Sakamoto ◽

Nobukazu Ishizaka ◽

Jiro Ando ◽

Ryozo Nagai ◽

Issei Komuro

Keyword(s):

Coronary Intervention ◽

Serum Levels ◽

Major Adverse Cardiovascular Events ◽

Immunoglobulin G4 ◽

Serum Igg4 Level ◽

Percutaneous Coronary ◽

Serum Igg4 ◽

Long Term Follow Up ◽

Igg4 Level

Background: Inflammatory processes contribute to the development of atherosclerosis. However, it remains unclear whether immunoglobulin G4 (IgG4)-related immuno-inflammation may affect the progression of cardiovascular disease. We therefore analyzed the relationship between serum IgG4 level and future major adverse cardiovascular events (MACE) after percutaneous coronary intervention (PCI). Methods: We measured serum levels of IgG4 in 562 patients (mean age, 66.9 ± 10.3 years) who underwent coronary angiography from October 2005 to September 2008. Furthermore, a total of 108 patients who subsequently received first PCI were followed-up for a median period of 2721 days (interquartile range [IR] 1591-3183). MACE was defined as cardiac death, acute coronary syndrome, cerebral stroke, and target vessel revascularization. Results: Serum levels of IgG4 were significantly higher in patients with coronary artery disease (34.1 mg/dL, IR 18.7-58.9) than in those without (30.5 mg/dL, IR 15.8-50.3, P=0.045). Among patients who received PCI, 40 (37.0%) patients developed MACE during the follow-up period. Patients with serum IgG4 levels of median or greater (≥37.2 mg/dL) had a significantly higher risk of MACE than those with serum IgG4 levels below median (log-rank test, P=0.017). In Cox regression analysis using age, gender, estimated glomerular filtration rate (eGFR), hypertension, dyslipidemia, diabetes, smoking, and serum levels of high-sensitivity C-reactive protein (hsCRP) as covariates, serum IgG4 level was an independent predictor of MACE (hazard ratio 2.39 per 100 mg/dL increase, 95% confidence interval 1.41-4.07, P=0.001). Conclusions: Elevated serum IgG4 level at baseline significantly predicted MACE after PCI, as well as present coronary stenosis, over long-term follow-up even among patients without apparent IgG4-related disease. IgG4-related immuno-inflammation may play a certain role in the progression of coronary atherosclerosis.

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