Korean J Gastroenterol.  2013 Jul;62(1):69-74. 10.4166/kjg.2013.62.1.69.

A Case of IgG4 Associated Sclerosing Cholangitis without Clinical Manifestations of Autoimmune Pancreatitis

• Chun SW
• Choi JS
• Kang BD
• Kim YJ
• Han KJ
• Cho HG
• Oh HE
• Cho JH

• KMID: 1711283
• DOI: http://doi.org/10.4166/kjg.2013.62.1.69

Abstract

IgG4-related systemic diseases are characterized by a diffuse or mass forming inflammatory reaction rich in lymphocytes and IgG4-positive plasma cells (lymphoplasmacytic infiltration), fibrosclerosis of variable organs and obliterative phlebitis. They usually involve various organs including the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate. However, most of them are accompanied by autoimmune pancreatitis, and good response to steroid treatment is one of the hallmarks of this disease. We report a case of an 67-year-old man with IgG4 associated sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography and successfully treated with steroid therapy.

Keyword

Immunoglobulin G4; Cholangitis, sclerosing; Pancreatitis; Cholangiocarcinoma

MeSH Terms

Aged
Anti-Inflammatory Agents/therapeutic use
Autoimmune Diseases/complications/diagnosis
Bile Ducts, Intrahepatic/pathology/ultrasonography
Cholangiopancreatography, Endoscopic Retrograde
Cholangitis, Sclerosing/complications/*diagnosis/drug therapy
Common Bile Duct/pathology/ultrasonography
Humans
Immunoglobulin G/*blood
Immunohistochemistry
Male
Pancreatitis/complications/diagnosis
Prednisolone/therapeutic use
Tomography, X-Ray Computed
Anti-Inflammatory Agents
Immunoglobulin G
Prednisolone

Figure

• 

  Fig. 1. Abdominal CT findings. (A) Initial coronal CT scan showed diffusely enhancing ductal wall which was thickened from the intrahepatic duct to proximal common bile duct (arrow). (B) Follow up CT scan after 2 months of steroid treatment showed ductal wall thickening of the biliary tree improved (arrow). (C) The pancreas initially appeared swollen and somewhat enlarged in the axial scan of abdominal CT. (D) After steroid treatment, the size of the pancreas decreased compared to initial CT scan. However, there was no other evidence of pancreatitis.

• 

  Fig. 2. EUS findings. Diffuse wall thickening of the common bile duct was noted on the EUS. The wall seemed to be hypoechoic, homogenous and smooth.

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  Fig. 3. ERCP findings. (A) Initial ERCP cholangiogram demonstrated diffuse and relatively irregular stricture of the intrahepatic duct and common hepatic duct. (B) After 2 months of steroid treatment, narrowings of the common and intrahepatic bile duct were resolved.

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  Fig. 4. Pathologic findings. Photos of H&E stain and IgG4 immunohistochemical stain of bile ductal tissue which were retrieved during ERCP. (A) H&E stain showed diffusely infiltrated inflammatory cells and some plasma cells were observed among numerous lymphocytes (×400). (B) Before steroid treatment, plasma cells were strongly stained by IgG4 immunochemical stain (×400). (C) After 2 months of steroid treatment, lymphocytes and plasma cells were not seen (H&E, ×100). (D) IgG4 immunohistochemical stain was also negative (×100).

Reference

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