IgG4-related sclerosing cholangitis
Autoimmune
Pankreatitis und Cholangitis im Verlauf: Oben Ausgangsbefund, unten Kontrolle nach Therapie (2 Monate). Von links nach rechts axial T2-SPIR, koronar T2 SPAIR, MRCP. Vor Therapie ist das Pancreas ödematös, wurstförmig aufgetrieben. Der Pankreasgang ist nahezu komplett verschwollen. Es findet sich eine deutliche Einengung der Gallenwege im Bereich der Hepatikusgabel mit intrahepatischer Cholestase. Nach Therapie Normalisierung des Pankreasparenchyms und weitgehend normalisierte Darstellung des Pankreasgangs. Auch die Enge in der Hepatikusgabel mit der Cholestase ist kaum noch erkennbar.
IgG4-related
disease • IgG4-related sclerosing cholangitis - Ganzer Fall bei Radiopaedia
Autoimmune
pancreatitis • IgG4-related autoimmune pancreatitis and cholangitis - Ganzer Fall bei Radiopaedia
Autoimmune
pancreatitis • IgG4-related disease - retroperitoneal fibrosis - Ganzer Fall bei Radiopaedia
Isolated
variant of IgG4-sclerosing cholangitis masquerading as hilar cholangiocarcinoma — an enigmatic entity. MRI axial cuts at the level of hilum showing bilobar IHBRD with the absence of formation of the primary confluence
Abdominal
manifestations of IgG4-related disease: a pictorial review. Example of IgG4 biliary stricture: MRCP shows a long smooth stricture involving the intrapancreatic portion of the CBD (arrow). The proximal pancreatic duct is also attenuated (arrowhead)
Abdominal
manifestations of IgG4-related disease: a pictorial review. Example of IgG4-related biliary stricture in a different patient. a Strictures are seen in the common hepatic duct and distal CBD (arrows). Sto stomach, Duo duodenum. b ERCP performed 6 months later on the same patient shows resolution of the extrahepatic strictures after treatment with corticosteroids
CT and MR
imaging of primary biliary cholangitis: a pictorial review. A 51-year-old male with IgG4-related sclerosing cholangitis (a–d). The T2WI image shows extensive periportal hyperintensity (arrows) (a). The axial and coronal images of PVP show the localized thickening of the common hepatic duct wall with moderate enhancement nodules (red arrows), resulting in corresponding bile duct stenosis and truncation (b–c). The MRCP image shows the localized truncation of the common hepatic duct (arrow) and significant dilatation of intra- and extrahepatic biliary ducts (d)
IgG4-related sclerosing cholangitis, also known as autoimmune cholangiopathy, is part of the spectrum of IgG4-related disease but can also occur in isolation. It is a separate entity to primary sclerosing cholangitis.
Pathology
The pathogenesis is poorly understood. The disease is characterized by dense bile duct infiltration by IgG4-positive plasma cells resulting in extensive fibrosis. There are often elevated serum IgG4 levels.
Associations
- type 1 autoimmune pancreatitis (90%)
Differential Diagnosis
- primary sclerosing cholangitis
- associated with inflammatory bowel disease
- younger patients
- less acute, longer duration of symptoms
- does not respond to steroids
- tends to produce shorter segments of stricturing ("beaded")
- cholangiocarcinoma
- confined to the bile ducts; IgG4 may have other manifestations of disease such as autoimmune pancreatitis
- secondary sclerosing cholangitis
Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu IgG4-assoziierte Cholangitis:
