Clinical practice guidelines for IgG4-related sclerosing cholangitis

Abstract

IgG4-related sclerosing cholangitis (IgG4-SC) is a distinct type of cholangitis frequently associated with autoimmune pancreatitis and currently recognized as a biliary manifestation of IgG4-related disease. Although clinical diagnostic criteria of IgG4-SC were established in 2012, differential diagnosis from primary sclerosing cholangitis and cholangiocarcinoma is sometimes difficult. Furthermore, no practical guidelines for IgG4-SC are available. Because the evidence level of most articles retrieved through searching the PubMed, Cochrane Library, and Igaku Chuo Zasshi databases was below C based on the systematic review evaluation system of clinical practice guidelines MINDS 2014, we developed consensus guidelines using the modified Delphi approach. Three committees (a guideline creating committee, an expert panelist committee for rating statements according to the modified Delphi method, and an evaluating committee) were organized. Eighteen clinical questions (CQs) with clinical statements were developed regarding diagnosis (14 CQs) and treatment (4 CQs). Recommendation levels for clinical statements were set using the modified Delphi approach. The guidelines explain methods for accurate diagnosis, and safe and appropriate treatment of IgG4-SC.

Keywords: Bile duct; Guideline; IgG4; Sclerosing cholangitis; Steroid.

Figure 1

Cholangiographic classification of IgG4‐SC referred from Reference 18

Figure 2

Histological findings of transmural inflammatory cell infiltration and fibrosis in the bile duct of IgG4‐SC (arrow). (Scale: 100 μm)

Figure 3

Histological findings of storiform fibrosis in the bile duct of IgG4‐SC. (Scale: 100 μm)

Figure 4

Histological findings of obliterative phlebitis (arrows) in the bile duct of IgG4‐SC. (Scale: 100 μm)

Figure 5

Immunohistochemical findings of infiltration of numerous IgG4‐positive cells in the bile duct of IgG4‐SC. IgG4‐immunostaining. (Scale: 100 μm)

Figure 6

Algorithm for diagnosis and treatment of IgG4‐SC referred from Reference 18

Figure 7

Algorithm for diagnosis and treatment of IgG4‐SC referred from Reference 18

Figure 8

Algorithm for diagnosis and treatment of IgG4‐SC referred from Reference 18

Figure 9

Algorithm for diagnosis and treatment of IgG4‐SC referred from Reference 18

Figure 10

Summary of imaging findings suggestive of IgG4‐SC

Figure 11

Schematic illustration of treatment of IgG4‐SC

Figure 12

Ultrasonographic image of IgG4‐SC. Ultrasonography detects marked wall thickening in the extrahepatic bile duct (arrows)

Figure 13

EUS image of IgG4‐SC. EUS shows a homogeneous, high‐low‐high echo of the bile duct wall thickening (arrows)

Figure 14

CT image of IgG4‐SC. Contrast‐enhanced CT (delayed image) demonstrates concentric wall thickening of the hilar bile duct (arrows)

Figure 15

CT and MRCP images of IgG4‐SC. (a) In CT, the hilar bile duct shows marked thickening (arrow). (b) MRCP demonstrates bile duct strictures in the hilar bile duct (arrows)

Figure 16

ERCP image of a patient with AIP in which the lesion was limited to the pancreatic head. Narrowing of the main pancreatic duct in the head (arrows) and strictures of the lower bile ducts are recognized

Figure 17

IgG4‐related dacryoadenitis. Swelling of right and left lacrimal glands (right > left) is recognized (arrows)

Figure 18

CT image of IgG4‐related retroperitoneal fibrosis. Soft‐tissue mass surrounding the abdominal aorta is recognized (arrow)

Figure 19

Cholangiographic classification of IgG4‐SC and the differential diagnosis of IgG4‐SC referred from Reference 3

Figure 20

Schematic illustration comparing the cholangiographic findings in PSC and IgG4‐SC referred from Reference 3

Figure 21

Cholangiogram of PSC showing (a) band‐like strictures and (b) beaded appearance (arrows)

Figure 22

Cholangiogram of PSC showing (a) pruned‐tree appearance and (b) diverticulum‐like outpouching (arrows)

Figure 23

Cholangiopancreatogram of type 1 IgG4‐SC showing the lower bile duct stricture and irregular narrowing of the main pancreatic duct (arrows)

Figure 24

Cholangiogram of (a) type 2a IgG4‐SC showing dilatation after a confluent stricture (arrow) and (b) type 2b IgG4‐SC showing no dilatation after a confluent stricture (arrows)

Figure 25

Cholangiogram of type 3 IgG4‐SC showing the hilar hepatic and lower bile duct strictures (arrows)

Figure 26

(a) Endoscopic image showing a swollen ampulla of Vater of a patient with IgG4‐SC. (b) Immunohistochemical findings of abundant infiltration of IgG4‐positive plasma cells in biopsy specimen taken from the ampulla of Vater of a patient with IgG4‐SC

Figure 27

IDUS image of (a) biliary stricture and (b) non‐biliary stricture in a patient with IgG4‐SC

Figure 28

POCS image of dilated and tortuous vessels in IgG4‐SC referred from Reference 112