Learning objectives
To describe the radiological findings on CT and MRI of the two mainways of pancreatic and biliary involvement in IgG4-Related Disease: Autoimmune Pancreatitis (AIP) and IgG4-Related Sclerosing Cholangitis (IgG4-SC).
To establish an adequate differential diagnosis with other tumoral and non-tumoral biliopancreatic diseases such as Pancreatic Carcinoma,
Primary Sclerosing Cholangitis and Cholangiocarcinoma.
Background
IgG4-Related Disease is a systemic entity that includes a broad spectrum of pathologies associated toelevated serum levels of IgG4 (Fig. 2).
The main ways of pancreatic and biliary involvement are AIP and IgG4-SC,two pathologies with frequent synchronous presentation and typical imaging features that must be known by the radiologist in order to avoid misdiagnosis with otherdiseases.
Findings and procedure details
AUTOIMMUNE PANCREATITIS
- Autoinmune Pancreatitis (AIP) is a fibroinflammatory disease of the pancreas with two histologic subtypes that share similar imaging features(Fig. 3):
a) Type I.
Most frequent subtype.
Associatedwith elevated serum levels of IgG4 and biliary disease (IgG4-SC is present in up to 80-90% of cases),
and less frequently with fibrosing diseases in other organs.
In this exhibit we will focus on this type of AIP.
b) Type II.
Onlyinvolves the pancreas and there is no association with IgG4.
- Serology: In AIP type...
Conclusion
Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis are the two main ways of pancreatic and biliary involvement in IgG4-Related Disease.
They have distinctive radiological featureson CT and MRI (Fig. 22)that must be known by the radiologist in order to allow an early diagnosis and an optimal therapeutic approach,
avoiding misdiagnosis with other diseases and unnecesary invasive procedures.
Both entities are associated in up to 80-90% of cases and share clinical symptoms,
presenting usually as a painless ormildlysymptomatic obstructive jaundice in a 40 to 70 year-old man...
Personal information
Javier Miguez Gonzalez,
MD
Department of Radiology
Hospital Sant Joan Despí Moisès Broggi
Carrer de Jacint Verdaguer 90
08970Sant Joan Despí (Barcelona)
Phone: +34 935 531 200 | Ext.
7335
e-mail:
[email protected]
References
1.Vlachou PA,
Khalili K,
Jang HJ,
et al.
IgG4-related sclerosing disease: autoimmune pancreatitis and extrapancreatic manifestations. RadioGraphics2011; 31:1379–1402
2. A.
Martínez-de-Alegría,
S.
Baleato-González et al.
IgG4-related Disease from Head to Toe.
RadioGraphics 2015; 35:2007–2025
3.
Shimosegawa T,
Chari ST,
Frulloni L,
Kamisawa T,
Kawa S,
MinoKenudson M,
et al.
International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology.
Pancreas 2011; 40:352-8.
[PMID: 21412117]
4.
Sahani DV,
Sainani NI,
Deshpande V,
et al.
Autoimmune pancreatitis: disease evolution,
staging,
response assessment,
and...