Article

Endoscopic retrograde cholangiography does not reliably distinguish IgG4-associated cholangitis from primary sclerosing cholangitis or cholangiocarcinoma.

Department of Gastroenterology, University College Hospital, London, United Kingdom.
Clinical gastroenterology and hepatology: the official clinical practice journal of the American Gastroenterological Association (impact factor: 5.64). 06/2011; 9(9):800-803.e2. DOI:10.1016/j.cgh.2011.05.019 pp.800-803.e2
Source: PubMed

ABSTRACT Distinction of immunoglobulin G4-associated cholangitis (IAC) from primary sclerosing cholangitis (PSC) or cholangiocarcinoma is challenging. We aimed to assess the performance characteristics of endoscopic retrograde cholangiography (ERC) for the diagnosis of IAC.
Seventeen physicians from centers in the United States, Japan, and the United Kingdom, unaware of clinical data, reviewed 40 preselected ERCs of patients with IAC (n = 20), PSC (n = 10), and cholangiocarcinoma (n = 10). The performance characteristics of ERC for IAC diagnosis as well as the κ statistic for intraobserver and interobserver agreement were calculated.
The overall specificity, sensitivity, and interobserver agreement for the diagnosis of IAC were 88%, 45%, and 0.18, respectively. Reviewer origin, specialty, or years of experience had no statistically significant effect on reporting success. The overall intraobserver agreement was fair (0.74). The operating characteristics of different ERC features for the diagnosis of IAC were poor.
Despite high specificity of ERC for diagnosing IAC, sensitivity is poor, suggesting that many patients with IAC may be misdiagnosed with PSC or cholangiocarcinoma. Additional diagnostic strategies are likely to be vital in distinguishing these diseases.

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  • Article: Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy.
    Amaar Ghazale, Suresh T Chari, Lizhi Zhang, Thomas C Smyrk, Naoki Takahashi, Michael J Levy, Mark D Topazian, Jonathan E Clain, Randall K Pearson, Bret T Petersen, Santhi Swaroop Vege, Keith Lindor, Michael B Farnell
    [show abstract] [hide abstract]
    ABSTRACT: Immunoglobulin (Ig)G4-associated cholangitis (IAC) is the biliary manifestation of a steroid-responsive multisystem fibroinflammatory disorder in which affected organs have a characteristic lymphoplasmacytic infiltrate rich in IgG4-positive cells. We describe clinical features, treatment response, and predictors of relapse in IAC and compare relapse rates in IAC with intrapancreatic vs proximal bile duct strictures. We reviewed clinical, serologic, and imaging characteristics and treatment response in 53 IAC patients. IAC patients generally were older (mean age, 62 y) men (85%), presenting with obstructive jaundice (77%) associated with autoimmune pancreatitis (92%), increased serum IgG4 levels (74%), and abundant IgG4-positive cells in bile duct biopsy specimens (88%). At presentation, biliary strictures were confined to the intrapancreatic bile duct in 51%; the proximal extrahepatic/intrahepatic ducts were involved in 49%. Initial presentation was treated with steroids (n = 30; median follow-up period, 29.5 months), surgical resection (n = 18; median follow-up period, 58 months), or was conservative (n = 5; median follow-up period, 35 months). Relapses occurred in 53% after steroid withdrawal; 44% relapsed after surgery and were treated with steroids. The presence of proximal extrahepatic/intrahepatic strictures was predictive of relapse. Steroid therapy normalized liver enzyme levels in 61%; biliary stents could be removed in 17 of 18 patients. Fifteen patients treated with steroids for relapse after steroid withdrawal responded; 7 patients on additional immunomodulatory drugs remain in steroid-free remission (median follow-up period, 6 months). IAC should be suspected in unexplained biliary strictures associated with increased serum IgG4 and unexplained pancreatic disease. Relapses are common after steroid withdrawal, especially with proximal strictures. The role of immunomodulatory drugs for relapses needs further study.
    Gastroenterology 04/2008; 134(3):706-15. · 11.68 Impact Factor
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Keywords

40 preselected ERCs
 
Additional diagnostic strategies
 
cholangiocarcinoma
 
clinical data
 
different ERC features
 
endoscopic retrograde cholangiography
 
ERC
 
IAC
 
IAC diagnosis
 
immunoglobulin G4-associated cholangitis
 
interobserver agreement
 
intraobserver agreement
 
operating characteristics
 
performance characteristics
 
primary sclerosing cholangitis
 
Reviewer origin
 
statistically significant effect
 
United States
 
vital
 
κ statistic