Outcome and Genetic Factors in IgG4-Associated Autoimmune Pancreatitis and Cholangitis: A Single Center Experience

Buechter, Matthias; Manka, Paul; Heinemann, Falko M.; Lindemann, Monika; Juntermanns, Benjamin; Canbay, Ali; Gerken, Guido; Kahraman, Alişan

doi:10.1155/2017/6126707

Cited by 16 publications

(16 citation statements)

References 52 publications

(59 reference statements)

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“…We found a lifetime risk of CCA of 3.4% in our IgG4-SC cohort; a similar lifetime risk of CCA in patients with IgG4-SC has been reported by Hugget et al [15]. Buechter et al reported a higher lifetime risk of CCA of 10% [26]. In a Japanese nationwide study, CCA was diagnosed in only 0.4% [27].…”

Section: Discussion Main Findingssupporting

confidence: 90%

The long-term outcomes of patients with immunoglobulin G4-related sclerosing cholangitis: the Mayo Clinic experience

Ali

Machicado

et al. 2020

J Gastroenterol

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Background The long-term outcomes of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are not well known. Methods The outcomes of patients with IgG4-SC at Mayo Clinic (1999)(2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016)(2017)(2018) were compared to an age-and gendermatched (1:1 ratio) group of patients with primary sclerosing cholangitis (PSC). Results We identified 89 patients with IgG4-SC; median age at diagnosis was 67 years, 81% were males, and the median follow-up was 5.7 years. Seventy-eight patients received prednisone for induction of remission, and 53 received at least one other immunosuppressive agent for maintenance of remission. Of the IgG4-SC group, 10 died (median time from diagnosis until death was 6.5 years): 2 due to cirrhosis, 3 due to cholangiocarcinoma (CCA), and 5 due to non-hepatobiliary causes. Eleven patients in the PSC group underwent liver transplantation, while none did in the IgG4-SC group. The incidence of a hepatobiliary adverse event (cirrhosis or CCA) was 3.4 times greater in the PSC compared to the IgG4-SC group (events per 1000 person-years: 52.6; 95% CI 38-73; vs. 15.6; 95% CI 7-32). The probability of development of a hepatobiliary adverse event within 10 years was 11% in the IgG4-SC compared to 45% in the PSC group (P = 0.0001). The overall survival tended to be higher in the IgG4-SC compared to the PSC group (10-year: 79% vs. 68%, respectively; P = 0.11). Conclusions In a cohort of IgG4-SC patients, 88% of whom were treated with immunosuppressive drugs, the risk of cirrhosis and CCA was significantly lower compared to an age-and gender-matched group with PSC.

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Section: Discussion Main Findingssupporting

confidence: 90%

The long-term outcomes of patients with immunoglobulin G4-related sclerosing cholangitis: the Mayo Clinic experience

Ali

Machicado

et al. 2020

J Gastroenterol

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“…Unlike European studies, AIP and IAC may coexist in most cases reported on many Asian cohorts. [ 31 ] In our study, 35 patients (90%) had AIP, of the 4 patients who did not have AIP, 2 patients were diagnosed with IAC successfully, 1 was misdiagnosed with cholangiocarcinoma, and the other was misdiagnosed with pancreatic cancer. Differential diagnosis between cholangiocarcinoma and IAC is challenging due to significant overlap of clinical manifestations, lab tests, and imaging characteristics.…”

Section: Discussionmentioning

confidence: 61%

Analysis of clinical characteristics and treatment of immunoglobulin G4-associated cholangitis

Xiao

Xu²,

Li³

et al. 2018

Medicine

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Immunoglobulin (Ig)G4-associated cholangitis (IAC) is one of the common organ manifestations of IgG4-related systemic disease (ISD). IAC and autoimmune pancreatitis (AIP) may mimic sclerosing cholangitis, cholangiocarcinoma, or pancreatic carcinoma. Diagnosis is based on a combination of clinical, biochemical, radiological, and histological findings.To study the clinical presentation of and treatment strategy for IAC, we reviewed clinical, serologic, and imaging characteristics, as well as treatment response, in 39 patients with IAC. The majority of patients were men (82%). Clinical features on presentation included obstructive jaundice in 26 patients (67%) and abdominal pain in 20 (51%). Positive IgG4 immunostaining was seen in 27 patients. The median serum IgG4 level before treatment was 769.4 mg/dL (range, 309.1–1229.7 mg/dL). After the steroid therapy, the median serum IgG4 level in 23 patients was 247.0 mg/dL (range, 139.0–355.0 mg/dL). Cholangiograms were available in 36 (92%) patients. Stenosis of the lower part of the common bile duct was found in 26 of 39 patients. Stenosis was diffusely distributed in the intra- and extrahepatic bile ducts in 14 of 39 patients. Additionally, strictures of the bile duct were detected in the hilar hepatic lesions in 27 of 39 patients. AIP was the most frequent comorbidity (35/39 in this study) of IAC. Other affected organs included eyes (n = 6), salivary glands (sialadenitis, n = 10), lymph nodes (mediastinal and axillary, n = 3), kidneys (n = 2), and the retroperitoneum (retroperitoneal fibrosis, n = 2).Regarding treatment, 29 patients were treated with steroids, of whom one underwent pancreatoduodenectomy, and one underwent choledochojejunostomy. Eight patients were treated with biliary stents. The remaining 19 patients took prednisolone alone. Eight patients achieved spontaneous resolution. Four patients with suspected pancreatic cancer or cholangiocarcinoma underwent surgery, including 2 patients who also received postoperative steroids. All patients were regularly followed up for 9 to 36 months. Only 2 patients in the steroids treatment group relapsed to manifest obstructive jaundice and high serum IgG4 levels. These 2 patients were treated with steroids and biliary stents, resulting in complete remission.We also review the diagnostic and therapeutic management and discuss recent pathophysiological findings, which might aid in understanding the molecular mechanisms contributing to IAC and other manifestations of IgG4-related diseases (IgG4-RD). Biomarkers that are more accurate are needed to correctly diagnose IAC and prevent misdiagnoses and unnecessary therapeutic interventions.

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“…In the 6 studies published before 2011, 5 were studied in Asia; wherever the 7 studies published after 2011, only 3 studies were in Asia. In contrast to western countries, where type 2 AIP is supposed to be more frequent (up to 50%), Asian studies with large patient population demonstrated that nearly all patients (>90%) suffered from type 1 AIP 46,49,50. That is probably why the pooled DOR suggested the diagnostic accuracy of sIgG4 superior in the studies before 2011.…”

Section: Discussionmentioning

confidence: 94%

“…According to high serum levels of CA 19-9 are indicative of malignancies, whereas low levels were independently associated with AIP, compared with PC 30. Serological markers such as high sIgG4 and CA 19-9 levels along with radiographic examination could be helpful in the discrimination of AIP and PC 8,30,46…”

Section: Discussionmentioning

confidence: 99%

Diagnostic Utility of Serum IgG4 in Autoimmune Pancreatitis

Lee

Yang

Chang

et al. 2021

Journal of Clinical Gastroenterology

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Objectives: Despite many studies suggesting an association between serum immunoglobulin G4 (sIgG4) and autoimmune pancreatitis (AIP), the evidence of utility in differentiation between AIP and pancreatic cancer (PC) remain uncertain. Methods: The analysis based on published studies. Data were pooled by means of a random-effects model, and sensitivity, specificity, diagnostic odds ratios (DOR), areas under summary receiver operating characteristic curves were calculated. Results: In the included thirteen studies, sIgG4 were measured in 594 patients with AIP and 958 patients with PC. The pooled sensitivity, specificity, DOR, and area under the curve were 0.72 [95% confidence interval (CI): 0.68-0.75], 0.93 (95% CI: 0.92-0.95), 51.37 (95% CI: 23.20-113.74), and 0.91 (95% CI: 0.87-0.95). Subgroup analyses of the DORs for region and year: Asia, (112.10; 95% CI: 27.72-453.32), non-Asia (26.01; 95% CI: 12.38-54.65), and year before 2011 (107.61; 95% CI: 39.30-294.68), year after 2011 (26.96; 95% CI: 9.78-74.32). Overall, sIgG4 was associated with AIP, the result revealed a moderate sensitivity 0.72 and high specificity 0.93. In the meta-analysis, the pooled DOR of sIgG4 levels of 2-fold upper limit 50.44 was similar with the DOR 51.37 when 1-fold cut-off value, but the summary receiver operating characteristic was 0.755 and 0.91. The higher specificity (from 93% to 98%) derived from the cut-off value (from 130-140 to 260-280 mg/dL) for sIgG4 occurred at a significant reduction in sensitivity (from 72% to 43%). Conclusions: The study revealed sIgG4 is a good marker of AIP. Screening of sIgG4 may help clinicians differentiate between AIP and PC, and the best cut-off value should be 140 rather than 280 mg/dL.

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Outcome and Genetic Factors in IgG4-Associated Autoimmune Pancreatitis and Cholangitis: A Single Center Experience

Cited by 16 publications

References 52 publications

The long-term outcomes of patients with immunoglobulin G4-related sclerosing cholangitis: the Mayo Clinic experience

The long-term outcomes of patients with immunoglobulin G4-related sclerosing cholangitis: the Mayo Clinic experience

Analysis of clinical characteristics and treatment of immunoglobulin G4-associated cholangitis

Diagnostic Utility of Serum IgG4 in Autoimmune Pancreatitis

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