scholarly journals Advances in IgG4-related Hepatobiliary Disease

2020 ◽  
Vol 25 (2) ◽  
pp. 100-106
Author(s):  
Sung-Hoon Moon
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Quantitative Polymerase Chain Reaction ◽  
High Serum ◽  
Hepatobiliary Disease ◽  
Glucocorticoid Therapy ◽  
Glucocorticoid Treatment ◽  
Immunoglobulin G4 ◽  
Serum Igg4 ◽  
Polymerase Chain

The emergence of the new disease entity of steroid responsive immunoglobulin G4 (IgG4)-related hepatobiliary disease has generated considerable interest among hepatobiliary society. IgG4-related hepatobiliary disease refers to mainly IgG4-related sclerosing cholangitis (IgG4-SC), and includes a small number of IgG4-related hepatopathy. As IgG4-SC responds well to glucocorticoid therapy, IgG4-SC should be differentiated from cholangiocarcinoma and primary sclerosing cholangitis. Timely diagnosis of IgG4-SC can lead clinicians to prescribe adequate glucocorticoid treatment that can reverse bile duct strictures and cholestatic liver function. Differentiation of IgG4-SC from primary sclerosing cholangitis is sometimes challenging because serum IgG4 and tissue IgG4 have demonstrated low positive predictive value in this setting. Recent research suggested that blood IgG4/IgG RNA ratio by quantitative polymerase chain reaction can be used for differentiation. Although most patients with IgG4-SC/autoimmune pancreatitis respond to glucocorticoid therapy, they frequently experience relapse of disease. The suggested relapse factors included very high serum IgG4, diffuse enlargement of the pancreas, proximal IgG4-SC, and multi-organ involvement. This review discusses the recent advances in the pathogenesis, diagnosis, management, disease relapse, and monitoring disease activity of IgG4-SC.

scholarly journals Immunoglobulin G4 Sclerosing Cholangitis: An Unusual Cause of Obstructive Jaundice—Case Report and Literature Review

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Pragya Shrestha ◽  
Brian Le ◽  
Brent Wagner ◽  
William Pompella ◽  
Paras Karmacharya
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Systemic Disease ◽  
Clinical Manifestations ◽  
Disease Process ◽  
High Serum ◽  
Diagnostic Challenge ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Serum Igg4

IgG4-related sclerosing cholangitis (IgG4-SC) is one of the most common extra-pancreatic manifestation of IgG4-related disease (IgG4-RD) and is clinically distinct from primary sclerosing cholangitis (PSC). IgG4-RD is an increasingly recognized immune-mediated fibroinflammatory systemic disease, mostly affecting middle-aged and older male populations that can affect multiple organs. The presence of extra-biliary clinical manifestations of IgG4-RD, such as parotid and lacrimal swelling, lymphadenopathy, autoimmune pancreatitis, and retroperitoneal fibrosis, if present could provide important clues to diagnosis. High serum IgG4 levels, characteristic radiological (e.g., sausage-shaped pancreas or periaortitis) or biopsy findings (high percentage of IgG4+ plasma cells, lymphoplasmacytic infiltrate, storiform fibrosis, or obliterative phlebitis) in the setting of these features is diagnostic of this disease process. However, isolated IgG4-SC might be a diagnostic challenge, and the distinction is important as management of this disorder is vastly different from other causes of cholangitis such as PSC. Systemic corticosteroid therapy is the mainstay of therapy.

Serum IgG4 cut-off of 70 mg/dL is associated with a shorter time to cirrhosis decompensation and liver transplantation in primary sclerosing cholangitis patients

2021 ◽  
Author(s):  
Farhad Peerani ◽  
Lillian Du ◽  
Ellina Lytvyak ◽  
Vincent G Bain ◽  
Andrew L Mason ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Survival Time ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Area Under The Curve ◽  
Retrospective Chart Review ◽  
Unknown Etiology ◽  
Immunoglobulin G4 ◽  
Free Survival ◽  
Serum Igg4

BACKGROUND: Primary sclerosing cholangitis (PSC) is an immune-mediated biliary disorder of unknown etiology with no effective treatment. The purpose of this study was to better prognosticate the development of cirrhosis, decompensation, and requirement for liver transplantation (LT) in PSC patients based on serum immunoglobulin G4 (IgG4) levels. METHODS: A retrospective chart review was conducted on PSC patients seen at the University of Alberta Hospital between 2002 and 2017. PSC patients were categorized as high IgG4 group (≥70 mg/dL) or normal IgG4 group (<70 mg/dL). Laboratory parameters, clinical characteristics, and outcomes were compared between the groups. RESULTS: One hundred and ten patients were followed over a mean period of 7.3 (SD 5 years). Seventy-two patients (66%) were male, the mean age at diagnosis of PSC was 35 (SD 15) years, and inflammatory bowel disease (IBD) was present in 80 patients (73%). High IgG4 levels were found in 37 patients (34%). PSC patients with high IgG4 had a shorter mean cholangitis-free survival time (5.3 versus 10.4 years, p = 0.02), cirrhosis-free survival time (8.7 years versus 13.0 years, p = 0.02), and LT-free survival time (9.3 years versus 18.9 years, p <0.001). IgG4 ≥70 mg/dL was independently associated with liver decompensation and LT-free outcomes. A cut-off IgG4 value of ≥70 mg/dL performed better than a cut-off value of ≥140 mg/dL to predict time to LT (area under the curve [AUC] 0.68, p = 0.03, sensitivity 72%, specificity 78%). CONCLUSIONS: Serum IgG4 ≥70 mg/dL in PSC predicts a shorter time to cirrhosis decompensation and LT.

scholarly journals Immunoglobulin G4-related masses surrounding coronary arteries: a case report

2021 ◽  
Vol 5 (3) ◽  
Author(s):  
Takuya Nakamura ◽  
Yutaka Goryo ◽  
Takuya Isojima ◽  
Hiroyuki Kawata
Keyword(s):  
Coronary Artery ◽  
Coronary Angiography ◽  
Cardiovascular System ◽  
Coronary Arteries ◽  
Elevated Serum ◽  
High Serum ◽  
Immunoglobulin G4 ◽  
Coronary Arteritis ◽  
Serum Igg4 ◽  
Mass Formation

Abstract Background Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition with high serum IgG4 levels affecting various organs, such as the pancreas, lacrimal and salivary glands, thyroid, kidney, and lung. Typical cardiovascular manifestations of IgG4-RD include periaortitis, coronary arteritis, and pericarditis. However, reports of IgG4-RD associated with coronary arteritis are rare. Here, we report a case of IgG4-related masses surrounding the coronary arteries. Case summary A 59-year-old man was referred to our hospital because of mediastinal masses detected by computed tomography (CT). Coronary CT angiography revealed masses surrounding the right coronary artery and the left anterior descending coronary artery. An elevated serum level of IgG4 and histological findings led to the diagnosis of IgG4-related coronary arteritis with mass formation. Coronary angiography showed numerous feeding arteries to the masses, which were demonstrated as multiple microchannels in the intravascular ultrasound (IVUS) images. Discussion IgG4-RD involving the cardiovascular system has been reported. However, coronary artery disease associated with IgG4-RD is very rare, and the mechanism of mass formation in IgG4-related coronary arteritis is unclear. In our case, within the cardiovascular system, IgG4-RD was limited to the coronary arteries, suggesting that the affected coronary arteries may provide the necessary blood supply to the mass, thus, aiding its growth. These findings were supported by the images from coronary angiography and IVUS.

scholarly journals Serum IgG4 Concentration is a Potential Predictive Biomarker in Glucocorticoid Treatment for Idiopathic Retroperitoneal Fibrosis

2022 ◽  
Author(s):  
Shoichiro Mukai ◽  
Naotaka Sakamoto ◽  
Hiroaki Kakinoki ◽  
Tadamasa Shibuya ◽  
Ryosuke Moriya ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Predictive Marker ◽  
Predictive Biomarker ◽  
Complete Response ◽  
Glucocorticoid Therapy ◽  
Smoking History ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Glucocorticoid Treatment ◽  
Female Ratio ◽  
Serum Igg4

Abstract Objectives: To evaluate the management and outcome of idiopathic retroperitoneal fibrosis (iRPF) in Japan, and identify its clinical biomarker. Methods: We retrospectively analyzed 129 patients with iRPF treated between January 2008 and May 2018 at 12 university and related hospitals. Patients treated with glucocorticoid were analyzed to identify a predictive biomarker. These patients were classified into three groups according to overall effectiveness (no change: NC, complete response: CR and partial response groups: PR), and each parameter was compared statistically.Results: Male-female ratio was 5: 1, and median age at diagnosis was 69 (33-86) years. Smoking history was reported in 59.6% of the patients. As treatment, 95 patients received glucocorticoid therapy with an overall response rate of 84%. As a result, serum concentration of IgG4 was significantly decreased in NC group compared with the other two groups (56.6mg/dL vs 255mg/dL, 206mg/dL, P=0.0059 and 0.0078). ROC analysis was performed between the non-responder (NC) and responder groups (CR+PR) to identify the cut-off value of serum IgG4 as a predictive marker. As a result, AUC was 0.793 and the values of sensitivity and specificity were 0.85 and 0.64, respectively, under the cut-off values of 67.6mg/dL. Conclusions: In the majority of iRPF patients, glucocorticoid therapy resulted in a favorable response. Pre-treatment serum IgG4 concentration may have potential as a predictive biomarker of steroid treatment.

scholarly journals Immunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review

2020 ◽  
Vol 48 (10) ◽  
pp. 030006052095921
Author(s):  
Cheng Xu ◽  
Yongmei Han
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Immunoglobulin G4 ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
History Of ◽  
The Common

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a novel clinical disease that is characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltrated by IgG4+ plasma cells. The clinical manifestations of IgG4-RD depend on the type of tissues affected. IgG4-related sclerosing cholangitis is a type of IgG4-RD. We report a patient who initially visited a local hospital with a 5-month history of jaundice. He was found to have a mass in the upper part of the common bile duct that mimicked cholangiocarcinoma. He underwent surgery in our hospital and was later diagnosed with IgG4-related sclerosing cholangitis. We administered prednisolone 40 mg once a day for treatment. Taking into account the possible side effects of moderate-dose hormone therapy, we also administered teprenone, potassium chloride, and calcium carbonate. The patient did not have any recurrence of symptoms or adverse drug reactions during follow-up.

scholarly journals Primary sclerosing cholangitis with increased immunoglobulin G4 levels

Medicine ◽  
2019 ◽  
Vol 98 (50) ◽  
pp. e18411
Author(s):  
Qing-Ling Chen ◽  
Rui Zhong ◽  
Xiao-Xue Zhang ◽  
Li-Na Feng ◽  
Xiao-Yu Wen ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Immunoglobulin G4

Elevated Serum IgG4 Concentration in Patients with Primary Sclerosing Cholangitis

2006 ◽  
Vol 101 (9) ◽  
pp. 2070-2075 ◽  
Author(s):  
Flavia D Mendes ◽  
Roberta Jorgensen ◽  
Jill Keach ◽  
Jerry A Katzmann ◽  
Thomas Smyrk ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Elevated Serum ◽  
Serum Igg4

652 Prevalence and Significance of Elevated Serum IgG4 Levels in Primary Sclerosing Cholangitis

2015 ◽  
Vol 148 (4) ◽  
pp. S-990
Author(s):  
Nazir Ahmed ◽  
Raghuwansh P. Sah ◽  
Naoki Takahashi ◽  
Keith Lindor ◽  
Suresh T. Chari
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Elevated Serum ◽  
Serum Igg4

Diagnosis, Differential Diagnosis, and Epidemiology of Primary Sclerosing Cholangitis

2015 ◽  
Vol 33 (Suppl. 2) ◽  
pp. 134-139 ◽  
Author(s):  
Cyriel Y. Ponsioen
Keyword(s):  
Bile Duct ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Large Population ◽  
Cumulative Risk ◽  
Population Based ◽  
Small Duct ◽  
Serum Igg4 ◽  
Profile Characteristic ◽  
Secondary Causes

According to recent guidelines, primary sclerosing cholangitis (PSC) is diagnosed when a patient has a cholestatic liver enzyme profile, characteristic bile duct changes on imaging, and when secondary causes of sclerosing cholangitis are excluded. In patients with a clinical suspicion but normal cholangiography, a liver biopsy is indicated to establish a diagnosis of small duct PSC. Several other disease entities such as IgG4-associated cholangitis (IAC), cholangiocarcinoma (CCA), and secondary causes of sclerosing cholangitis such as choledocholithiasis, AIDS-cholangiopathy, ischemia, surgical bile duct trauma, or mast cell cholangiopathy can mimic PSC. IAC can be differentiated from PSC by applying the HISORt criteria including the serum IgG4 level. In cases where serum IgG4 is less than 2 × ULN, the ratio of IgG4/IgG1 >0.24 is indicative for IAC. Choledocholithiasis with recurrent cholangitis as a cause of sclerosing cholangitis can pose a conundrum, since PSC itself is associated with an increased prevalence of gallstones. The epidemiology of PSC worldwide has been poorly described. Incidence and prevalence rates vary from 0-1.3 and 0-16.2 per 100,000 inhabitants respectively. However, these figures are not based on population-based cohorts. A recent large population-based cohort from the Netherlands reported an incidence of 0.5 and a prevalence of 6/100,000. Approximately 10% fulfil the criteria for small duct PSC. At diagnosis of PSC, concurrent inflammatory bowel disease (IBD), primarily ulcerative colitis or Crohn's colitis is present in 50%, but increasing to 80%, 10 years or more after diagnosis. Conversely, 3% of IBD patients will develop PSC. PSC predisposes to malignancy. The estimated cumulative risk of developing CCA after 30 years is 20%. For colorectal carcinoma in PSC/colitis patients, the estimated cumulative risk at 30 years is 13%.

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