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IgG4 Levels in Bile for Distinguishing IgG4-Associated Cholangiopathy from Other Biliary Disorders: A Single Blinded Pilot Study.

Navaneethan U, Gutierrez NG, Jegadeesan R, Venkatesh PG, Poptic E, Sanaka MR, Vargo JJ, Parsi MA - Clin Endosc (2014)

Bottom Line: The median bile IgG4 levels were markedly elevated in patients with IAC (5.5 mg/dL; interquartile range [IQR], 5.1 to 15.6) as compared to patients with benign biliary conditions (0 mg/dL; IQR, 0 to 0.1; p=0.003).A cutoff value of 3.8 mg/dL distinguished IAC from PSC and CCA patients with 100% and 76.9% sensitivity and specificity, respectively.The results of this pilot study suggest that measurement of biliary IgG4 levels may have clinical value in distinguishing patients with IAC from biliary disorders that can mimic IAC.

View Article: PubMed Central - PubMed

Affiliation: Department of Gastroenterology, Digestive Disease Institute, Cleveland Clinic, Cleveland, OH, USA.

ABSTRACT

Background/aims: Immunoglobulin G4 (IgG4)-associated cholangiopathy (IAC) is an inflammatory disease and may mimic primary sclerosing cholangitis (PSC), cholangiocarcinoma (CCA), or pancreatic cancer on cholangiography. We investigated whether IgG4 levels in bile aspirated during endoscopic retrograde cholangiopancreatography (ERCP) can distinguish IAC from PSC, CCA, and pancreatic cancer.

Methods: Bile was aspirated directly from the common bile duct during ERCP in patients with IAC prior to steroid therapy. For control purposes, bile was obtained from patients with PSC, CCA, pancreatic cancer, and benign biliary conditions (sphincter of oddi dysfunction/choledocholithiasis).

Results: Biliary IgG4 levels were measured in 54 patients. The median bile IgG4 levels were markedly elevated in patients with IAC (5.5 mg/dL; interquartile range [IQR], 5.1 to 15.6) as compared to patients with benign biliary conditions (0 mg/dL; IQR, 0 to 0.1; p=0.003). The median biliary IgG4 levels in PSC, CCA, and pancreatic cancer were 1.2 (IQR, 0.2 to 3.8), 0.9 (IQR, 0.2 to 3.4), and 0.2 mg/dL (IQR, 0.1 to 0.8), respectively. A cutoff value of 3.8 mg/dL distinguished IAC from PSC and CCA patients with 100% and 76.9% sensitivity and specificity, respectively.

Conclusions: The results of this pilot study suggest that measurement of biliary IgG4 levels may have clinical value in distinguishing patients with IAC from biliary disorders that can mimic IAC.

No MeSH data available.


Related in: MedlinePlus

IgG4 levels in bile among different study groups. IgG4, immunoglobulin G4; PSC, primary sclerosing cholangitis; CA, carcinoma; IAC, IgG4-associated cholangiopathy.
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Figure 1: IgG4 levels in bile among different study groups. IgG4, immunoglobulin G4; PSC, primary sclerosing cholangitis; CA, carcinoma; IAC, IgG4-associated cholangiopathy.

Mentions: Biliary IgG4 levels were significantly higher in patients with IAC compared to those with benign biliary conditions (5.5; interquartile range [IQR], 5.1 to 15.6 vs. 0; IQR, 0 to 0.1; p=0.003). All three patients with IAC had evidence of associated pancreatic involvement. The first of these patients had a mid common bile duct stricture raising suspicion for possible CCA. However, further evaluation including IgG4 elevation in the serum to 553 mg/dL (normal <112 mg/dL) confirmed the diagnosis of IAC and the cholangiogram normalized with steroid treatment. The second patient had a mild elevation of serum IgG4 to 118 mg/dL and a pancreatic mass which was diagnosed as AIP based on pancreatic biopsy. This pancreatic mass resolved completely with steroid treatment. Subsequently, this patient developed multifocal biliary strictures resembling PSC. Further treatment with steroids resulted in complete normalization of the cholangiogram. The third patient had a distal bile duct stricture with elevation of serum IgG4 to 315 mg/dL. This patient also had a pancreatic mass which was diagnosed as AIP based on pancreatic biopsy on endoscopic ultrasound-guided fine needle aspiration. The pancreatic mass resolved completely with steroid treatment. All of these patients had ampullary biopsies, and only one had a positive result. Endoscopic ultrasound was performed in all three patients, two of whom had evidence of AIP from core biopsies of the pancreas, and 1 who did not have any pancreatic mass or features of AIP. Fig. 1 shows the median bile IgG4 levels in all patient groups.


IgG4 Levels in Bile for Distinguishing IgG4-Associated Cholangiopathy from Other Biliary Disorders: A Single Blinded Pilot Study.

Navaneethan U, Gutierrez NG, Jegadeesan R, Venkatesh PG, Poptic E, Sanaka MR, Vargo JJ, Parsi MA - Clin Endosc (2014)

IgG4 levels in bile among different study groups. IgG4, immunoglobulin G4; PSC, primary sclerosing cholangitis; CA, carcinoma; IAC, IgG4-associated cholangiopathy.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4260104&req=5

Figure 1: IgG4 levels in bile among different study groups. IgG4, immunoglobulin G4; PSC, primary sclerosing cholangitis; CA, carcinoma; IAC, IgG4-associated cholangiopathy.
Mentions: Biliary IgG4 levels were significantly higher in patients with IAC compared to those with benign biliary conditions (5.5; interquartile range [IQR], 5.1 to 15.6 vs. 0; IQR, 0 to 0.1; p=0.003). All three patients with IAC had evidence of associated pancreatic involvement. The first of these patients had a mid common bile duct stricture raising suspicion for possible CCA. However, further evaluation including IgG4 elevation in the serum to 553 mg/dL (normal <112 mg/dL) confirmed the diagnosis of IAC and the cholangiogram normalized with steroid treatment. The second patient had a mild elevation of serum IgG4 to 118 mg/dL and a pancreatic mass which was diagnosed as AIP based on pancreatic biopsy. This pancreatic mass resolved completely with steroid treatment. Subsequently, this patient developed multifocal biliary strictures resembling PSC. Further treatment with steroids resulted in complete normalization of the cholangiogram. The third patient had a distal bile duct stricture with elevation of serum IgG4 to 315 mg/dL. This patient also had a pancreatic mass which was diagnosed as AIP based on pancreatic biopsy on endoscopic ultrasound-guided fine needle aspiration. The pancreatic mass resolved completely with steroid treatment. All of these patients had ampullary biopsies, and only one had a positive result. Endoscopic ultrasound was performed in all three patients, two of whom had evidence of AIP from core biopsies of the pancreas, and 1 who did not have any pancreatic mass or features of AIP. Fig. 1 shows the median bile IgG4 levels in all patient groups.

Bottom Line: The median bile IgG4 levels were markedly elevated in patients with IAC (5.5 mg/dL; interquartile range [IQR], 5.1 to 15.6) as compared to patients with benign biliary conditions (0 mg/dL; IQR, 0 to 0.1; p=0.003).A cutoff value of 3.8 mg/dL distinguished IAC from PSC and CCA patients with 100% and 76.9% sensitivity and specificity, respectively.The results of this pilot study suggest that measurement of biliary IgG4 levels may have clinical value in distinguishing patients with IAC from biliary disorders that can mimic IAC.

View Article: PubMed Central - PubMed

Affiliation: Department of Gastroenterology, Digestive Disease Institute, Cleveland Clinic, Cleveland, OH, USA.

ABSTRACT

Background/aims: Immunoglobulin G4 (IgG4)-associated cholangiopathy (IAC) is an inflammatory disease and may mimic primary sclerosing cholangitis (PSC), cholangiocarcinoma (CCA), or pancreatic cancer on cholangiography. We investigated whether IgG4 levels in bile aspirated during endoscopic retrograde cholangiopancreatography (ERCP) can distinguish IAC from PSC, CCA, and pancreatic cancer.

Methods: Bile was aspirated directly from the common bile duct during ERCP in patients with IAC prior to steroid therapy. For control purposes, bile was obtained from patients with PSC, CCA, pancreatic cancer, and benign biliary conditions (sphincter of oddi dysfunction/choledocholithiasis).

Results: Biliary IgG4 levels were measured in 54 patients. The median bile IgG4 levels were markedly elevated in patients with IAC (5.5 mg/dL; interquartile range [IQR], 5.1 to 15.6) as compared to patients with benign biliary conditions (0 mg/dL; IQR, 0 to 0.1; p=0.003). The median biliary IgG4 levels in PSC, CCA, and pancreatic cancer were 1.2 (IQR, 0.2 to 3.8), 0.9 (IQR, 0.2 to 3.4), and 0.2 mg/dL (IQR, 0.1 to 0.8), respectively. A cutoff value of 3.8 mg/dL distinguished IAC from PSC and CCA patients with 100% and 76.9% sensitivity and specificity, respectively.

Conclusions: The results of this pilot study suggest that measurement of biliary IgG4 levels may have clinical value in distinguishing patients with IAC from biliary disorders that can mimic IAC.

No MeSH data available.


Related in: MedlinePlus