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ueg education
Mistakes in... 2021
a
b
Figure 5 | Autoimmune pancreatitis. a | A diffuse pancreatic enlargement with hypoechoic, patchy and heterogeneous parenchyma. b | A focal hypoechoic mass, located
in the pancreatic head-neck inducing 'mass-effect' on splenoportal confluence and lymphadenopathy.
serology, other organ involvement, pancreatic
histopathology, and response to steroid
treatment.62
Type 1 AIP (referred to as a
lymphoplasmacytic sclerosing pancreatitis) is the
pancreatic manifestation of a systemic disease
(so-called IgG4 disease) and is frequently
associated with other organ involvement.63
Type 2 AIP is also known as idiopathic
duct-centric pancreatitis. Despite consensus
diagnostic criteria, the diagnosis of AIP often
remains challenging.64,65
The IgG4 serum level is
helpful to establish the diagnosis of type 1
AIP, but lacks sensitivity and specificity,
and only 22-23% of patients fulfil the
criteria to diagnose an IgG4-related
disease.63,66
Patients with AIP present with typical acute
pancreatitis or abdominal pain, but also with
a
jaundice and/or a pancreatic mass that often
mimics pancreatic carcinoma. The classic EUS
findings for AIP include diffuse pancreatic
enlargement with hypoechoic, patchy and
heterogeneous parenchyma (figure 5a).67-69
EUS
may also demonstrate a focal hypoechoic mass,
most frequently located in the pancreatic head,
induce main pancreatic duct (MPD) narrowing with
duct-wall thickening and usually without
upstream dilation. Sometimes, the mass may
appear to involve peripancreatic vessels
(figure 5b), induce upstream MPD dilation,
associated with enlarged peripancreatic lymph
nodes, mimicking pancreatic cancer.67,68
The
presence of diffuse pancreatic enlargement, a
hypoechoic thickened MPD and/or bile-duct
wall, hypoechoic peripancreatic halo has
been seen more frequently in patients with
b
a confirmed AIP diagnosis than pancreatic
cancer.
A key feature and clue to the presence
of AIP is the finding of IgG4-associated
cholangitis with markedly thickened bile ducts
and in some cases gallbladder wall.70,71
In
contrast with pancreatobiliary malignancies, in
which the biliary involvement is more irregular,
the bile duct thickening in AIP IgG4-cholangitis
is regular, homogeneous, with smooth inner and
outer margins. The thickening may be extended to
the cystic duct and gallbladder (figure 6).
Given the lack of pathognomonic EUS
features for the differential diagnosis of a
pancreatic solid mass, several imaging-enhancing
techniques have been developed. CH-EUS
allows the assessment of pancreatic tumour
enhancement using ultrasound contrast
Figure 6 | EUS images of patients with IgG4-disease-related cholangitis. a | Regular bile-duct thickening extended to the cystic duct. b | gallbladder.
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