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EDUCATION EXHIBIT 157

Lymphoplasmacytic
Sclerosing Pancreatitis
(Autoimmune Pancre-
atitis): Evaluation with
Multidetector CT1
Satomi Kawamoto, MD ● Stanley S. Siegelman, MD ● Ralph H. Hruban,
ONLINE-ONLY
CME MD ● Elliot K. Fishman, MD
See www.rsna Lymphoplasmacytic sclerosing pancreatitis is a form of chronic pancreati-
.org/education tis characterized by a mixed inflammatory infiltrate that centers on the
/rg_cme.html.
pancreatic ducts. It is a cause of benign pancreatic disease that can clini-
cally mimic pancreatic cancer. Preoperative detection of lymphoplasma-
LEARNING cytic sclerosing pancreatitis is important because patients usually respond
OBJECTIVES
to steroid therapy. Patients with lymphoplasmacytic sclerosing pancreatitis
After reading this
article and taking are often referred for computed tomography (CT) when they are sus-
the test, the reader pected of having a pancreatic or biliary neoplasm; therefore, it is important
will be able to:
䡲 List the clinical and to search for potential findings suggestive of lymphoplasmacytic sclerosing
pathologic features of pancreatitis when typical findings of a pancreatic or biliary neoplasm are
lymphoplasmacytic
sclerosing pancreati- not found. Typical CT findings include diffuse or focal enlargement of the
tis. pancreas without dilatation of the main pancreatic duct. Focal enlarge-
䡲 Discuss the spec- ment is most commonly seen in the head of the pancreas, and the involved
trum of CT appear-
ances of lymphoplas- pancreas on contrast material– enhanced CT images may be isoattenuat-
macytic sclerosing ing relative to the rest of the pancreas, or hypoattenuating, especially dur-
pancreatitis.
ing the early postcontrast phase. Thickening and contrast enhancement of
䡲 Describe the CT
features that may the wall of the common bile duct and gallbladder may reflect inflamma-
allow lymphoplasma- tory infiltrate and fibrosis associated with lymphoplasmacytic sclerosing
cytic sclerosing pan-
creatitis to be differ- pancreatitis. There are several features seen at CT that may help to differ-
entiated from pan- entiate lymphoplasmacytic sclerosing pancreatitis from pancreatic cancer,
creatic cancer.
such as diffuse enlargement of the pancreas with minimal peripancreatic
stranding in patients with obstructive jaundice, an absence of significant
TEACHING pancreatic atrophy, and an absence of significant main pancreatic duct
POINTS
See last page
dilatation. When these findings are encountered, clinical, other imaging,
and serologic data should be evaluated.
©
RSNA, 2008

Abbreviation: ERCP ⫽ endoscopic retrograde cholangiopancreatography

RadioGraphics 2008; 28:157–170 ● Published online 10.1148/rg.281065188 ● Content Codes:

1From the Russell H. Morgan Department of Radiology and Radiological Science (S.K., S.S.S., E.K.F.) and Department of Pathology, Sol Goldman
Pancreatic Cancer Research Center (R.H.H.), Johns Hopkins Medical Institutions, JHOC 3235A, 601 N Caroline St, Baltimore, MD 21287. Recipi-
ent of a Certificate of Merit award for an education exhibit at the 2005 RSNA Annual Meeting. Received November 7, 2006; revision requested April
18, 2007, and received June 21; accepted June 28. All authors have no financial relationships to disclose. Address correspondence to S.K. (e-mail:
skawamo1@jhmi.edu).
©
RSNA, 2008
 158 January-February 2008
Introduction
In recent years, the concept of autoimmune pan-
creatitis has been established to refer to a special
type of chronic pancreatitis with unique clinical
and histologic manifestations. Autoimmune pan-
creatitis can be defined as a chronic inflammatory
process of the pancreas that is caused by an auto-
immune mechanism (1,2). The morphologic hall-
marks are periductal infiltration by lymphocytes
and plasma cells and granulocytic epithelial le-
sions with consequent destruction of the duct epi-
thelium and venulitis (3). Therefore, autoim-
mune pancreatitis has been morphologically
described as lymphoplasmacytic sclerosing pan-
creatitis, non-alcoholic duct-destructive chronic
pancreatitis (4,5), or chronic sclerosing pancreati-
tis (6). The terms autoimmune pancreatitis and
sclerosing pancreatitis have been used interchange-
ably (7).
The gross morphologic alterations produced
by lymphoplasmacytic sclerosing pancreatitis may
simulate malignancy, with characteristics such as
masslike enlargement of the pancreatic head
and/or irregular narrowing of the pancreatic duct
and stricture of the common bile duct. However,
lymphoplasmacytic sclerosing pancreatitis re-
Teaching sponds to oral steroid therapy, with reversible
Point improvement of pancreatic morphology and func-
tion (1,8 –10). Thus, when findings typical of a
pancreatic or biliary neoplasm are not seen on
computed tomographic (CT) images, it is impor-
tant to search for characteristics suggestive of
lymphoplasmacytic sclerosing pancreatitis and
allow accurate diagnosis and appropriate therapy
to occur.
The purpose of this article is to discuss and
illustrate the spectrum of appearances of lym-
phoplasmacytic sclerosing pancreatitis on CT
images. Pathologic and clinical features of lym-
phoplasmacytic sclerosing pancreatitis, CT tech-
niques, and findings with other imaging modali-
ties are also presented. CT features that may be
potentially useful in differentiating lymphoplas-
macytic sclerosing pancreatitis from pancreatic
cancer are discussed and illustrated.
Most of the patients that are discussed in this
article had no serologic parameters measured,
and diagnosis was based on pathologic analysis.
Therefore, in this article, we use the term lym-
phoplasmacytic sclerosing pancreatitis.
Pathologic Features of Lympho-
plasmacytic Sclerosing Pancreatitis
At gross examination, the involved pancreas is
firm or hard and may be enlarged or “mass form-
RG f Volume 28 ● Number 1
Figure 1. Photomicrograph (original magnification,
⫻40; hematoxylin-eosin stain) of a pancreatic duct
specimen from a patient with lymphoplasmacytic scle-
rosing pancreatitis shows dense inflammatory infiltrates
with associated fibrosis surrounding the pancreatic duct.
ing.” These features may lead to surgical resec-
tion because of the suspicion that the lesion is
carcinomatous (7). The inflammatory process
may involve either the entire pancreas, or it may
be limited to only a portion of the pancreas.
When the inflammatory process involves only one
portion of the pancreas, that segment is most of-
ten the pancreatic head. In a minority of cases,
however, the inflammatory process is concen-
trated in the body or in the tail of the pancreas
(7). It is not known how frequently and to what
extent the entire pancreas is affected in lympho-
plasmacytic sclerosing pancreatitis (3).
Microscopic findings of autoimmune pancre-
atitis are consistent with lymphoplasmacytic scle-
rosing pancreatitis. In pathologic specimens,
dense lymphoplasmacytic infiltrate centered
around the medium- and large-sized interlobular
pancreatic ducts is seen (11) (Fig 1). Although
the inflammatory infiltrate consists mainly of lym-
phocytes and plasma cells, it also contains some
macrophages and occasionally neutrophilic and
eosinophilic granulocytes (11). Immunocyto-
chemical typing of the lymphocytes reveals that
most of them are CD8- and CD4-positive T lym-
phocytes, with B lymphocytes present to a lesser
degree (3). The lumen of inflamed pancreatic
ducts is encompassed by infiltrate and is nar-
rowed by infolding of the epithelium (3). A dis-
tinctive venulitis is also seen.
When the pancreas is only slightly affected, the
inflammation centers almost entirely on the
ducts; in severely affected pancreata, the inflam-
matory process involves the acinar parenchyma in
addition to the ducts and leads to diffuse sclerosis
(3). The acinar cells are replaced by inflammatory
RG f Volume 28 ● Number 1
Figure 2. Photomicrograph (original magnification,
⫻40; hematoxylin-eosin stain) of a surgical specimen of
the common bile duct shows dense mixed infiltrate and
fibrosis surrounding the common bile duct.
cells and fibrosis, and the lobular architecture of
the pancreas is almost lost (3). The peripancreatic
and peribiliary lymph nodes are enlarged and
show follicular hyperplasia (3).
It is also well known that patients with lym-
phoplasmacytic sclerosing pancreatitis display
distal common bile duct strictures and inflamma-
tion, features that overlap with those of primary
sclerosing cholangitis. Abraham et al (12) re-
ported that among 20 patients with lymphoplas-
macytic sclerosing pancreatitis treated with pan-
creaticoduodenectomy, inflammatory infiltrates
were seen in the extrapancreatic common bile
duct in 60%, in the intrapancreatic common bile
duct in 84.2% (Fig 2), and in the gallbladder in
60%. However, inflammatory and sclerosing
changes of the intrahepatic bile duct, which are
typical findings in primary sclerosing cholangitis,
are uncommon in lymphoplasmacytic sclerosing
pancreatitis (3). Moreover, unlike typical primary
sclerosing cholangitis, biliary lesions in lympho-
plasmacytic sclerosing pancreatitis usually im-
prove with administration of steroids. These
findings suggest that the mechanism of the devel-
opment of biliary lesions in lymphoplasmacytic
sclerosing pancreatitis may differ from that of
typical primary sclerosing cholangitis (3,13).
Clinical Presentation of Lympho-
plasmacytic Sclerosing Pancreatitis
Lymphoplasmacytic sclerosing pancreatitis is a
rare disorder, and the exact prevalence is un-
known. Ito et al (8) reported that among 161 pa-
tients with chronic pancreatitis evaluated with
endoscopic retrograde pancreatography, three
patients (1.86%) had lymphoplasmacytic scleros-
Kawamoto et al 159
ing pancreatitis. Okazaki et al (13) reported that
among 620 patients with chronic pancreatitis, 30
of them (5%) had lymphoplasmacytic sclerosing
pancreatitis. In a recent Italian multicenter study
on the epidemiology of chronic pancreatitis that
involved 21 centers and enrolled 282 patients suf-
fering from chronic pancreatitis over 2 years, au-
toimmunity was recognized as an associated fac-
tor in 23 patients (6%) (7). The reported mean
age of 41 patients with lymphoplasmacytic scle-
rosing pancreatitis was 62.2 years (range, 32–76
years) (14). Another study reported that the mean
age of 53 patients with lymphoplasmacytic scle-
rosing pancreatitis was 56 years (range, 14 –77
years) (15).
Clinically, lymphoplasmacytic sclerosing pan-
creatitis commonly manifests as obstructive jaun-
dice with no or only mild abdominal pain, weight
loss, and recent-onset diabetes in elderly patients.
Acute attacks seen in severe or acute pancreatitis
do not usually occur (13). Obstructive jaundice is
often caused by stenosis of the intrapancreatic
common bile duct (7) and is seen in 63%–75% of
patients with lymphoplasmacytic sclerosing pan-
creatitis (13–15). Diabetes mellitus is also often
associated with lymphoplasmacytic sclerosing
pancreatitis and has a reported frequency of less
than 20%– 68% (7,13). Because these signs over-
lap with those of pancreatic cancer, lymphoplas-
macytic sclerosing pancreatitis has frequently
been misdiagnosed as pancreatic cancer. Harda-
cre et al (16) reported that between pancreatic
cancer and lymphoplasmacytic sclerosing pancre-
atitis, there were no statistically significant differ-
ences in the rates of abdominal pain, weight loss,
jaundice, preoperative carcinoembryonic antigen,
or CA19-9 levels. Analysis of a large series involv-
ing more than 1200 patients who underwent pan-
creaticoduodenectomy with a presumed preop-
erative diagnosis of pancreatic cancer, periampul-
lary neoplasm, or cholangiocarcinoma revealed
that 2.2%–2.4% of patients had pathologic fea-
tures consistent with lymphoplasmacytic scleros-
ing pancreatitis (16,17).
Elevation of serum gamma globulin or immu-
noglobulin G (IgG) concentration and the pres-
ence of some autoantibodies are often observed.
Serum IgG4 concentrations were reported to be
significantly and specifically high in patients with
lymphoplasmacytic sclerosing pancreatitis and are
closely associated with disease activity (18). Ha-
mano et al (18) reported that the accuracy, sensi-
tivity, and specificity of elevated serum concentra-
tions of IgG4 in the diagnosis of this disease, with
a cutoff value of 135 mg/dL, were 97%, 95%,
160 January-February 2008 RG f Volume 28 ● Number 1

Figure 3. Venous phase axial (a, b) and coronal (c) re-

formatted CT images show mild diffuse enlargement of
the pancreas with minimal peripancreatic stranding. The
distal common bile duct shows smooth beaklike stenosis in
the region of the pancreatic head with dilatation of the upper
common bile duct. Mild thickening and contrast material
enhancement of the common bile duct wall are also seen
(arrows in b and c). Pathologic analysis revealed an intense
lymphoplasmacytic process involving the common bile duct.

and 97% respectively. However, other groups

reported lower sensitivity (19 –21). Ghazale et al
(21) reported that serum concentrations of IgG4
were elevated in 10% of pancreatic cancer pa-
tients (13 of 135), but that only 1% had serum
IgG4 levels of ⬎280 mg/dL, compared with 53%
of patients with lymphoplasmacytic sclerosing
pancreatitis.
Occasionally, patients with lymphoplasmacytic
sclerosing pancreatitis have extrapancreatic le-
sions that are seen in other autoimmune diseases,
including Sjögren syndrome, sclerosing cholangi-
tis, primary biliary cirrhosis, interstitial nephritis,
sialoadenitis, enlarged mediastinal or cervical
lymph nodes, ulcerative colitis, and retroperito-
neal fibrosis (22). The incidence of associated
extrapancreatic autoimmune lesions is reported to
range from 19% to more than 50% (7,15,22).
These extrapancreatic autoimmune diseases may
be recognized at the time of diagnosis or they may
develop later (7). The presence of an associated
autoimmune disease may help in the diagnosis of
lymphoplasmacytic sclerosing pancreatitis (7).
Lymphoplasmacytic sclerosing pancreatitis is
difficult to diagnose. In 2002, the Japan Pancreas
Society proposed the following diagnostic criteria
for autoimmune pancreatitis: (a) pancreatic im-
aging studies show diffuse enlargement of the
pancreas and diffuse narrowing of the main pan-
creatic duct with an irregular wall (more than
one-third the length of the entire pancreas),
(b) laboratory data demonstrate abnormally el-
evated levels of serum gamma globulin and/or
IgG or the presence of autoantibodies, and (c) fi-
brotic change with dense lymphoplasmacytic in-
filtration is noted in the pancreas at histopatho-
logic examination. A diagnosis of lymphoplasma-
cytic sclerosing pancreatitis can be established if
all of the criteria are present or if criterion a is
present with either criterion b or criterion c (23).
Recently, these criteria have been modified by the
Japan Pancreas Society to allow diagnosis of auto-
immune pancreatitis to include focal pancreatic
mass and focal pancreatic duct stricture (24). Re-
cently, other groups have also proposed diagnos-
tic criteria for autoimmune pancreatitis (19,25).
CT Technique
CT examinations were performed with multide-
tector CT scanners, including a Siemens Volume
Zoom scanner (4 ⫻ 1 mm collimation), a Sie-
mens Sensation 16 scanner (16 ⫻ 0.75 mm colli-
mation), and a Siemens Sensation 64 scanner
(64 ⫻ 0.6 mm collimation) (Siemens Medical
RG f Volume 28 ● Number 1 Kawamoto et al 161

Figure 4. (a, b) Venous phase axial images show mild enlargement of the pancreatic head, with a stent present in
the common bile duct. Nondilated main pancreatic duct is seen in the body and tail (arrowheads in a). Focal hypoat-
tenuating lesions are seen in both kidneys (arrows in b). Although biopsy of the lesions was not performed, follow-up
CT showed that these lesions became less obvious. (c, d) Contrast-enhanced chest CT scans (d, lung window set-
ting) show a left hilar mass with bulky mediastinal adenopathy. Results of biopsy performed after mediastinoscopy
and limited anterior thoracotomy revealed anthracotic lymph nodes with fibrosis in the mediastinal lymph nodes, fi-
brous tissue of chronic inflammation in the mediastinal soft tissue, and mild chronic inflammation and pleural fibro-
sis in the left upper lobe.

Solutions, Malvern, Pa). The data were recon-
structed to obtain 1.25-mm section thickness at
1-mm intervals (0.25-mm overlap) with the Sie-
mens Volume Zoom Scanner and 0.75-mm sec-
tion thickness at 0.5-mm intervals (0.25-mm
overlap) with the Siemens Sensation 16 and 64
scanners. After fasting for at least 2–3 hours, each
patient ingested 750 –1000 mL of water over a
15–20 minute period before scanning was begun.
Arterial and venous phase images were acquired
at 25 seconds and 50 – 60 seconds from the start
of intravenous administration of contrast mate-
rial. We injected 120 mL of iohexol (Omnipaque
350; Amersham Health, Princeton, NJ) through
the peripheral venous line at a rate of 3 mL/sec.
Other scanning parameters included 120 kV and
150 –200 mAs.
All image data were reconstructed with the
body soft tissue algorithm and sent to the work-
station (Leonardo, Siemens Medical Solutions).
InSpace software (Siemens Medical Solutions)
was used for data analysis, which was the volume
imaging application for interactive viewing of vol-
ume data available on the Leonardo workstation.
CT Findings of Lympho-
plasmacytic Sclerosing Pancreatitis
Enlargement of the Pancreas
Typically, CT images show diffuse enlargement of
the pancreas (Fig 3). Focal enlargement may also be Teaching
seen, particularly in the pancreatic head (Fig 4), but Point
also in the body or tail (5) (Fig 5). The presence
162 January-February 2008 RG f Volume 28 ● Number 1

Figure 5. Venous phase oblique axial (a) and coronal (b) reformatted CT images show focal enlargement of
the pancreatic tail with a distinct area of decreased attenuation (arrows). Minimal stranding is seen around the
enlarged pancreatic tail.

Figure 6. Venous phase axial (a) and coronal (b) reformatted CT images show a diffusely hypoattenuating pan-
creas that appears to be normal in size. The pancreas appears featureless, and the normal lobular appearance is ef-
faced. A stent is seen in the common bile duct.

of multiple masses has also been reported (26). In
prior studies with CT, diffuse enlargement of the
pancreas was more commonly encountered than
was focal enlargement, and the incidence of dif-
fuse enlargement ranged from 56% to 100%
(9,27–31). Sahani et al (27) reported that among
25 patients with lymphoplasmacytic sclerosing
pancreatitis who underwent helical CT examina-
RG f Volume 28 ● Number 1 Kawamoto et al 163

Figure 7. Venous phase axial (a) and coronal (b, c) re-

formatted CT images show diffuse enlargement of the en-
tire pancreas. The pancreas appears featureless, and the
normal lobular appearance is effaced. There is minimal
peripancreatic stranding, and a stent is seen in the com-
mon bile duct.

phoplasmacytic sclerosing pancreatitis (25). Atro-

phy of the pancreas is not usually seen (28), al-
though the normal lobular appearance of the pan-
creas may be effaced and the gland may appear
featureless in the involved region (32) (Figs 6, 7).

Contrast Enhancement Pat-

tion, 14 showed a diffusely enlarged pancreas,
seven had smooth focal enlargement or a masslike
appearance in the pancreatic head and/or the un-
cinate process, and four had a pancreas that ap-
peared to be normal in size (Fig 6). Kamisawa et
al (28) reported that among 17 patients with lym-
phoplasmacytic sclerosing pancreatitis, 10 had
diffuse enlargement and seven had segmental en-
largement of the pancreatic head. However, an-
other study reported that diffuse enlargement was
seen in only six of 22 patients (27%) with lym-
tern of the Pancreatic Parenchyma
The reported contrast enhancement pattern of
the involved pancreas seen in lymphoplasmacytic
sclerosing pancreatitis is variable. Previous stud-
ies reported that on arterial phase or early post-
contrast phase images, the involved portion of the
pancreas appears hypoattenuating compared with
unaffected pancreatic parenchyma and occasion-
ally has a distinct margin (9,33) (Fig 5). On ve-
nous phase images, the involved portion of the
pancreas may remain hypoattenuating (33) or
may become nearly isoattenuating compared with
164 January-February 2008 RG f Volume 28 ● Number 1

Figure 8. (a, b) Arterial phase axial (a) and coro-

nal (b) reformatted CT images show enlargement of the
pancreatic head with a distinct area of decreased attenua-
tion within the enlarged pancreatic head (arrowheads).
The body and tail of the pancreas are relatively atrophic.
(c) Venous phase axial image shows that the hypoattenu-
ating area seen in the pancreatic head on arterial phase
images becomes nearly isoattenuating relative to adjacent
pancreatic parenchyma. A stent (arrow in a and c) is seen
in the common bile duct.

unaffected pancreatic parenchyma (Figs 8, 9).

However, a discrete area of differential contrast
enhancement may not be observed on arterial or
venous phase images in the areas of focal enlarge-
ment, and the pancreas may show homogeneous
contrast enhancement (27,30,32,34). In previous
CT studies, the area of focal enlargement may
appear homogeneously isoattenuating relative to
the pancreas in 25%–71% of patients, or it may
appear hypoattenuating in 29%–75% of patients
(27,34). When delayed phase images are obtained
at several minutes after intravenous administra-
tion of contrast material, the involved portion is
reported to become homogeneously isoattenuat-
ing or hyperattenuating compared with the sur-
rounding pancreatic parenchyma (31,34,35).
However, another study reported that the in-
volved lesion remains hypoattenuating compared
with unaffected pancreatic parenchyma on de-
layed phase images (33). These differences may
be attributed to the different CT techniques and
the degree of inflammatory infiltrate and fibrosis
of the involved pancreas.
 RG f Volume 28 ● Number 1 Kawamoto et al 165

Figure 9. (a, b) Arterial phase oblique axial (a) and

coronal (b) reformatted CT images show enlargement of
the pancreatic head with a distinct area of decreased at-
tenuation within the enlarged pancreatic head (black ar-
rows). The main pancreatic duct (white arrow) is mini-
mally dilated (3.8 mm) proximal to the enlarged portion.
(c) Venous phase axial image shows that a hypoattenuat-
ing area seen in the pancreatic head on arterial phase im-
ages becomes nearly isoattenuating (arrows) relative to
adjacent pancreatic parenchyma.

creas (Fig 9). However, significant dilatation of

the main pancreatic duct, a characteristic of pan-
creatic ductal adenocarcinoma, is not found in
lymphoplasmacytic sclerosing pancreatitis.

Narrowing of the Pancreatic Duct
Thin-section CT with multiplanar reformation
helps to delineate the main pancreatic duct. In
patients with lymphoplasmacytic sclerosing pan-
creatitis, the main pancreatic duct is diffusely or
segmentally narrowed. On CT images, the main
pancreatic duct may be seen as a small nondilated
Teaching
duct, or it may appear attenuated, particularly in
Point
the area of the pancreatic enlargement. Mild dila-
tation of the main pancreatic duct may also be
seen proximal to the enlarged portion of the pan-
Other Pancreatic/
Peripancreatic Findings
Minimal peripancreatic stranding, which may
simulate mild edematous acute pancreatitis, is
often seen at CT (27,33) (Figs 3, 7). A capsule-
like low-attenuation rim has also been described
in 12%– 80% of reported cases of lymphoplasma-
cytic sclerosing pancreatitis (Fig 10) (19,27,28,
30 –32). On delayed phase images, a capsulelike
low-attenuation rim may show subtle delayed en-
hancement (31,33).
166 January-February 2008 RG f Volume 28 ● Number 1

Figure 10. Venous phase oblique axial (a), coronal (b, c), and sagittal (d) reformatted CT images show mild dif-
fuse enlargement of the pancreas with a capsulelike low-attenuation rim (arrows in a and b). The splenic vein is sur-
rounded by the capsulelike rim and is narrowed (arrowhead in d). A stent is present in the distal common bile duct,
and the proximal common bile duct is dilated. Pathologic analysis revealed prominent lymphoplasmacytic infiltrate
involving the distal common bile duct.

In contrast to other forms of chronic pancreati-
tis, lymphoplasmacytic sclerosing pancreatitis
does not commonly manifest with parenchymal
calcifications and pseudocysts (3,28,36,37).
However, intraductal calcifications may occur late
in the course of the disease (9,31,33,38), and for-
mation of pseudocysts associated with lympho-
plasmacytic sclerosing pancreatitis has also been
reported (39).
Major pancreatic vascular involvement is un-
common in lymphoplasmacytic sclerosing pancre-
atitis compared with pancreatic adenocarcinoma
(27), although cases with venous occlusion or
narrowing, particularly the splenic vein, have
been reported (9,30,32) (Fig 10). Major arterial
involvement—such as narrowing of the celiac ar-
tery and the superior mesenteric artery— has not
been reported. At conventional angiography,
however, irregular narrowing and encasement of
the small peripancreatic arteries were reported in
up to 57% of cases (28,35). Poor opacification of
portal or splenic veins due to stenosis or obstruc-
tion with collateral veins was also reported in 23%
of cases at conventional angiography (28).
Enlarged peripancreatic lymph nodes may also
be observed on CT images. Sahani et al (27) re-
ported that nine of 25 patients with lymphoplas-
macytic sclerosing pancreatitis had enlarged
peripancreatic nodes that measured more than 1
cm in diameter on the short axis on CT scans.
Biliary Tract Findings
Stricture of the common bile duct is often seen in
Teaching
patients with lymphoplasmacytic sclerosing pan- Point
creatitis, particularly in patients whose pancreatic
head is affected, with a reported frequency of
33%–90% (5,27,28,30,31,40). At endoscopic
RG f Volume 28 ● Number 1 Kawamoto et al 167

Figure 11. Venous phase axial (a, b) and coronal (c)

reformatted CT images show mild diffuse enlargement of
the pancreas with minimal peripancreatic stranding. The
pancreas appears featureless, and the normal lobular ap-
pearance is effaced. The distal common bile duct shows
smooth beaklike stenosis in the region of the pancreatic
head (arrow in c) with dilatation of the proximal common
bile duct. Mild thickening and contrast enhancement of
the common bile duct wall are present (arrowheads in b
and c). At pathologic analysis, the common bile duct also
showed marked chronic inflammation and fibrosis.

wall have been described, and these findings cor-

respond to the inflammatory infiltrate and fibrosis
observed microscopically (Figs 3, 11) (30,32,42).
However, when a biliary stent is present at the
time of CT scanning, these biliary tract findings
may be obscured due to pneumobilia or changes
related to stent placement.

retrograde cholangiopancreatography (ERCP),
smooth stenosis of the distal common bile duct
localized in the pancreatic head, with dilatation of
the more proximal common bile duct, is the most
common finding in patients with lymphoplasma-
cytic sclerosing pancreatitis (27,41) (Figs 3, 11).
Narrowing of the intrapancreatic common bile
duct is thought to be induced mainly by compres-
sion of the swollen pancreas (7). Coronal three-
dimensional or reformatted CT images help to
delineate smooth, beaklike stenosis of the com-
mon bile duct in the intrapancreatic portion and
dilatation of the proximal common bile duct (Figs
3, 11). Stenosis or strictures of the proximal and
middle extrahepatic bile duct and the intrahepatic
bile duct—findings that resemble those seen in
primary sclerosing cholangitis— have also been
observed at ERCP and magnetic resonance (MR)
cholangiopancreatography (7,27,28).
At CT, thickening and enhancement of the
gallbladder wall and/or the common bile duct
Extrapancreatic Findings
Involvement with multiple organ systems has
been reported, and these findings may be seen on
CT images. Such changes include, but are not
limited to, retroperitoneal fibrosis, renal involve-
ment, lung disease, and mediastinal adenopathy
(27,43).
Findings at
Other Imaging Modalities
Patients with lymphoplasmacytic sclerosing pan-
creatitis typically show diffuse or segmental nar-
rowing of the main pancreatic duct at ERCP. The
secondary branches are usually not visualized (7–9).
MR imaging may reveal diffuse pancreatic en-
largement with hypointensity on T1-weighted
images. The low-attenuation capsulelike rim de-
scribed at CT is hypointense on T2-weighted im-
ages and shows delayed contrast enhancement,
which suggests fibrous tissue rather than a fluid
168 January-February 2008
collection or phlegmon (31). MR cholangiopan-
creatography may show stenosis of the bile ducts
mainly in the intrapancreatic area, which results
in dilatation of the proximal biliary tract (7). Scle-
rosing changes of the intrahepatic bile ducts or
common bile duct that are similar to primary scle-
rosing cholangitis are sometimes observed (7,44).
Although stenosis of the main pancreatic duct
may be observed, determining whether it is due to
lymphoplasmacytic sclerosing pancreatitis or to
pancreatic carcinoma may be difficult with MR
cholangiopancreatography (40).
At ultrasonographic (US) examination, the
pancreas appears hypoechoic and diffusely en-
larged with a so-called sausagelike appearance
(8,9,28). It may also appear as a hypoechoic mass
in the affected site (5,28). Endoscopic US may
show diffuse hypoechoic pancreatic enlargement
or a focal, irregular hypoechoic mass (20). Stric-
ture of the common bile duct in the pancreatic
head, widespread bile duct wall thickening, and
diffuse strong enhancement of the bile duct sys-
tem, including the gallbladder, proximal bile
duct, and distal bile duct, have also been seen
with endoscopic US when contrast material is
used (41). Farrell et al (20) reported that findings
from endoscopic US-guided fine-needle aspira-
tion may support the diagnosis of lymphoplasma-
cytic sclerosing pancreatitis in combination with
endoscopic US findings and clinical data.
Gallium scintigraphy (45,46) and fluorine 18
fluorodeoxyglucose positron emission tomogra-
phy (47) have been reported to show increased
uptake in the affected site of the pancreas during
the active stage of the disease, and such findings
may be confused with the increased uptake seen
in pancreatic cancer or lymphoma.
CT Features to Help
Differentiate Pancreatic Cancer
Lymphoplasmacytic sclerosing pancreatitis has
several typical characteristics on CT scans that
may be useful in differentiating the condition
from pancreatic cancer. When diffuse enlarge-
ment of the pancreas with mild peripancreatic
stranding is seen on CT images of patients with
obstructive jaundice without clinical features of
acute pancreatitis, lymphoplasmacytic sclerosing
pancreatitis should be considered as a potential
diagnosis. Clinical, serologic, and other imaging
studies should be carefully evaluated.
In patients with focal enlargement of the pan-
creas, diagnosing lymphoplasmacytic sclerosing
pancreatitis is more challenging. When a patient
with obstructive jaundice presents with a “mass”
in the pancreatic head, differentiating between
RG f Volume 28 ● Number 1
lymphoplasmacytic sclerosing pancreatitis and
pancreatic cancer can be extremely difficult (48).
The mass may also be seen in the body or tail of
the pancreas, and it may simulate pancreatic can-
cer. CT findings more typically seen in pancreatic
cancer than in lymphoplasmacytic sclerosing pan-
creatitis include (a) significant dilatation of the
main pancreatic duct proximal to the narrowed Teaching
Point
segment, (b) atrophy of the pancreatic paren-
chyma proximal to the mass or focal enlargement,
and (c) involvement of the major peripancreatic
vessels. In patients with segmental narrowing of
the main pancreatic duct due to lymphoplasma-
cytic sclerosing pancreatitis, the main pancreatic
duct proximal to the segmental narrowing typi-
cally shows minimal or no dilatation (19,28). Pre-
vious studies that compared ERCP images of fo-
cal lymphoplasmacytic sclerosing pancreatitis and
those of pancreatic cancer showed that the caliber
of the main pancreatic duct proximal to the stric-
ture is smaller in patients with lymphoplasmacytic
sclerosing pancreatitis (⬍4 mm in 67% of pa-
tients and 4 – 6 mm in 33%) than in those with
pancreatic ductal adenocarcinoma (⬍4 mm in
4%, 4 – 6 mm in 22%, and ⬎6 mm in 74%)
(30,35,49). Major peripancreatic vascular in-
volvement, particularly arterial involvement, is an
uncommon CT finding in lymphoplasmacytic
sclerosing pancreatitis. Atrophy of the pancreas
proximal to the mass is often seen in patients with
pancreatic cancer, but it is usually not found in
lymphoplasmacytic sclerosing pancreatitis (28).
Although the acinar parenchyma becomes atro-
phic in lymphoplasmacytic sclerosing pancreati-
tis, it is replaced with fibrous tissue (36), which
probably explains why the overall size of the pan-
creas does not usually change. Fine-needle aspira-
tion biopsy may remain a necessary step to con-
firm the diagnosis (7).
Smooth narrowing of the distal common bile
duct can be seen in patients with lymphoplasma-
cytic sclerosing pancreatitis; however, pancreatic
cancer may display similar findings. Wakabayashi
et al (35) reported that there were no significant
differences among patients with focal lympho-
plasmacytic sclerosing pancreatitis and those with
pancreatic cancer in terms of the frequency of
common bile duct stenosis and its character and
length as seen with ERCP.
Procacci et al (33) reported that CT findings
can be used to correctly diagnose lymphoplasma-
cytic sclerosing pancreatitis with an accuracy of
92.5%. They evaluated seven patients with lym-
phoplasmacytic sclerosing pancreatitis and 20
patients with other pancreatic diseases (acute or
chronic pancreatitis and adenocarcinoma of the
pancreas). The criteria for a diagnosis of lym-
phoplasmacytic sclerosing pancreatitis included
RG f Volume 28 ● Number 1
focal or diffuse enlargement of the pancreas, pos-
sible capsulelike rim, possible stenosis or com-
plete obstruction of the biliary duct, and possible
stenosis (either focal or diffuse) of the main pan-
creatic duct. The affected pancreatic parenchyma
was isoattenuating relative to the spleen and adja-
cent unaffected parenchyma on unenhanced CT
images and hypoattenuating on arterial phase,
venous phase, and delayed phase images (33).
Procacci and colleagues reported one false-nega-
tive diagnosis of lymphoplasmacytic sclerosing
pancreatitis in a case of chronic pancreatitis su-
perimposed with lymphoplasmacytic sclerosing
pancreatitis that showed diffuse calcifications in
the pancreas. They also recorded one false-posi-
tive diagnosis for lymphoplasmacytic sclerosing
pancreatitis in a case of mild edematous acute
pancreatitis with imaging findings similar to those
of lymphoplasmacytic sclerosing pancreatitis.
Conclusions
In conclusion, lymphoplasmacytic sclerosing pan-
creatitis is a unique clinical entity that is becom-
ing more frequently recognized in clinical prac-
tice. Because patients with lymphoplasmacytic
sclerosing pancreatitis often present with obstruc-
tive jaundice, their disease has been frequently
misdiagnosed as pancreatic cancer. However, be-
cause lymphoplasmacytic sclerosing pancreatitis
responds to steroid therapy, it is important to rec-
ognize this entity in order to avoid surgery. Char-
acteristics seen on CT images that suggest lym-
phoplasmacytic sclerosing pancreatitis include
diffuse or focal enlargement of the pancreas, ab-
sence of significant pancreatic atrophy, absence of
substantial main pancreatic duct dilatation, and
in some cases a rim of low attenuation surround-
ing the pancreas. However, diagnosis of this dis-
ease can be difficult, especially with patients who
present with a focal mass or a masslike enlarge-
ment of the pancreas. When these CT findings
are encountered, lymphoplasmacytic sclerosing
pancreatitis should be considered as a potential
diagnosis, and clinical, other imaging, and sero-
logic data should be carefully evaluated. Fine-
needle aspiration biopsy may remain a necessary
step for excluding malignancy and for final defini-
tive diagnosis.
References
1. Yoshida K, Toki F, Takeuchi T, Watanabe S,
Shiratori K, Hayashi N. Chronic pancreatitis
caused by an autoimmune abnormality: proposal
of the concept of autoimmune pancreatitis. Dig
Dis Sci 1995;40(7):1561–1568.
2. Okazaki K, Chiba T. Autoimmune related pancre-
atitis. Gut 2002;51(1):1– 4.
3. Kloppel G, Luttges J, Sipos B, Capelli P, Zamboni
G. Autoimmune pancreatitis: pathological find-
ings. JOP 2005;6(1 suppl):97–101.
Kawamoto et al 169
4. Ectors N, Maillet B, Aerts R, et al. Non-alcoholic
duct destructive chronic pancreatitis. Gut 1997;
41(2):263–268.
5. Van Hoe L, Gryspeerdt S, Ectors N, et al. Nonal-
coholic duct-destructive chronic pancreatitis: im-
aging findings. AJR Am J Roentgenol 1998;
170(3):643– 647.
6. Sood S, Fossard DP, Shorrock K. Chronic scleros-
ing pancreatitis in Sjogren’s syndrome: a case re-
port. Pancreas 1995;10(4):419 – 421.
7. Pearson RK, Longnecker DS, Chari ST, et al.
Controversies in clinical pancreatology: autoim-
mune pancreatitis— does it exist? Pancreas 2003;
27(1):1–13.
8. Ito T, Nakano I, Koyanagi S, et al. Autoimmune
pancreatitis as a new clinical entity: three cases of
autoimmune pancreatitis with effective steroid
therapy. Dig Dis Sci 1997;42(7):1458 –1468.
9. Furukawa N, Muranaka T, Yasumori K, Matsu-
bayashi R, Hayashida K, Arita Y. Autoimmune
pancreatitis: radiologic findings in three histologi-
cally proven cases. J Comput Assist Tomogr 1998;
22(6):880 – 883.
10. Kawa S, Hamano H. Autoimmune pancreatitis
and bile duct lesions. J Gastroenterol 2003;38(12):
1201–1203.
11. Abraham SC, Leach S, Yeo CJ, et al. Eosinophilic
pancreatitis and increased eosinophils in the pan-
creas. Am J Surg Pathol 2003;27(3):334 –342.
12. Abraham SC, Cruz-Correa M, Argani P, Furth
EE, Hruban RH, Boitnott JK. Lymphoplasma-
cytic chronic cholecystitis and biliary tract disease
in patients with lymphoplasmacytic sclerosing
pancreatitis. Am J Surg Pathol 2003;27(4):441–
451.
13. Okazaki K. Autoimmune pancreatitis: etiology,
pathogenesis, clinical findings and treatment—the
Japanese experience. JOP 2005;6(1 suppl):89 –96.
14. Kawa S, Ota M, Yoshizawa K, et al. HLA
DRB10405-DQB10401 haplotype is associated
with autoimmune pancreatitis in the Japanese
population. Gastroenterology 2002;122(5):1264 –
1269.
15. Zamboni G, Luttges J, Capelli P, et al. His-
topathological features of diagnostic and clinical
relevance in autoimmune pancreatitis: a study on
53 resection specimens and 9 biopsy specimens.
Virchows Arch 2004;445(6):552–563.
16. Hardacre JM, Iacobuzio-Donahue CA, Sohn TA,
et al. Results of pancreaticoduodenectomy for
lymphoplasmacytic sclerosing pancreatitis. Ann
Surg 2003;237(6):853– 858; discussion 858 – 859.
17. Weber SM, Cubukcu-Dimopulo O, Palesty JA, et
al. Lymphoplasmacytic sclerosing pancreatitis:
inflammatory mimic of pancreatic carcinoma. J
Gastrointest Surg 2003;7(1):129 –137; discussion
137–129.
18. Hamano H, Kawa S, Horiuchi A, et al. High se-
rum IgG4 concentrations in patients with scleros-
ing pancreatitis. N Engl J Med 2001;344(10):732–
738.
19. Kim KP, Kim MH, Kim JC, Lee SS, Seo DW,
Lee SK. Diagnostic criteria for autoimmune
chronic pancreatitis revisited. World J Gastroen-
terol 2006;12(16):2487–2496.
20. Farrell JJ, Garber J, Sahani D, Brugge WR. EUS
findings in patients with autoimmune pancreatitis.
Gastrointest Endosc 2004;60(6):927–936.
170 January-February 2008
21. Ghazale A, Chari ST, Smyrk TC, et al. Value of
serum IgG4 in the diagnosis of autoimmune pan-
creatitis and in distinguishing it from pancreatic
cancer. Am J Gastroenterol 2007;102(8):1646 –
1653.
22. Ohara H, Nakazawa T, Sano H, et al. Systemic
extrapancreatic lesions associated with autoim-
mune pancreatitis. Pancreas 2005;31(3):232–237.
23. Diagnostic criteria for autoimmune pancreatitis by
the Japan Pancreas Society. J Jpn Pancreas Soc
2002;17:585–587.
24. Okazaki K, Kawa S, Kamisawa T, et al. Clinical
diagnostic criteria of autoimmune pancreatitis:
revised proposal. J Gastroenterol 2006;41(7):626 –
631.
25. Chari ST, Smyrk TC, Levy MJ, et al. Diagnosis of
autoimmune pancreatitis: the Mayo Clinic experi-
ence. Clin Gastroenterol Hepatol 2006;4(8):
1010 –1016; quiz 1934.
26. Ohana M, Okazaki K, Hajiro K, Kobashi Y. Mul-
tiple pancreatic masses associated with autoimmu-
nity. Am J Gastroenterol 1998;93(1):99 –102.
27. Sahani DV, Kalva SP, Farrell J, et al. Autoim-
mune pancreatitis: imaging features. Radiology
2004;233(2):345–352.
28. Kamisawa T, Egawa N, Nakajima H, Tsuruta K,
Okamoto A, Kamata N. Clinical difficulties in the
differentiation of autoimmune pancreatitis and
pancreatic carcinoma. Am J Gastroenterol 2003;
98(12):2694 –2699.
29. Nishino T, Toki F, Oyama H, Shimizu K, Shira-
tori K. Long-term outcome of autoimmune pan-
creatitis after oral prednisolone therapy. Intern
Med 2006;45(8):497–501.
30. Yang DH, Kim KW, Kim TK, et al. Autoimmune
pancreatitis: radiologic findings in 20 patients. Ab-
dom Imaging 2006;31(1):94 –102.
31. Irie H, Honda H, Baba S, et al. Autoimmune pan-
creatitis: CT and MR characteristics. AJR Am J
Roentgenol 1998;170(5):1323–1327.
32. Kawamoto S, Siegelman SS, Hruban RH, Fish-
man EK. Lymphoplasmacytic sclerosing pancre-
atitis with obstructive jaundice: CT and pathology
features. AJR Am J Roentgenol 2004;183(4):915–
921.
33. Procacci C, Carbognin G, Biasiutti C, et al. Auto-
immune pancreatitis: possibilities of CT character-
ization. Pancreatology 2001;1(3):246 –253.
34. Wakabayashi T, Kawaura Y, Satomura Y, et al.
Clinical study of chronic pancreatitis with focal
irregular narrowing of the main pancreatic duct
and mass formation: comparison with chronic
pancreatitis showing diffuse irregular narrowing of
the main pancreatic duct. Pancreas 2002;25(3):
283–289.
35. Wakabayashi T, Kawaura Y, Satomura Y, et al.
Clinical and imaging features of autoimmune pan-
This article meets the criteria for 1.0 AMA PRA Category 1 Credit . To obtain credit, see www.rsna.org/education
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RG f Volume 28 ● Number 1
creatitis with focal pancreatic swelling or mass for-
mation: comparison with so-called tumor-forming
pancreatitis and pancreatic carcinoma. Am J Gas-
troenterol 2003;98(12):2679 –2687.
36. Abraham SC, Wilentz RE, Yeo CJ, et al. Pancre-
aticoduodenectomy (Whipple resections) in pa-
tients without malignancy: are they all ‘chronic
pancreatitis’? Am J Surg Pathol 2003;27(1):110 –
120.
37. Notohara K, Burgart LJ, Yadav D, Chari S, Smyrk
TC. Idiopathic chronic pancreatitis with periduc-
tal lymphoplasmacytic infiltration: clinicopatho-
logic features of 35 cases. Am J Surg Pathol 2003;
27(8):1119 –1127.
38. Takayama M, Hamano H, Ochi Y, et al. Recur-
rent attacks of autoimmune pancreatitis result in
pancreatic stone formation. Am J Gastroenterol
2004;99(5):932–937.
39. Nishimura T, Masaoka T, Suzuki H, Aiura K,
Nagata H, Ishii H. Autoimmune pancreatitis with
pseudocysts. J Gastroenterol 2004;39(10):1005–
1010.
40. Kamisawa T, Chen PY, Tu Y, et al. MRCP and
MRI findings in 9 patients with autoimmune pan-
creatitis. World J Gastroenterol 2006;12(18):
2919 –2922.
41. Hyodo N, Hyodo T. Ultrasonographic evaluation
in patients with autoimmune-related pancreatitis. J
Gastroenterol 2003;38(12):1155–1161.
42. Nikfarjam M, Muralidharan V, Christophi C,
Tang H, Clouston D. Autoimmune pancreatitis.
ANZ J Surg 2002;72(6):450 – 452.
43. Brennan D, Pedrosa I. Lymphoplasmacytic scle-
rosing pancreatitis. AJR Am J Roentgenol 2005;
185(5):1367–1368; author reply 1368.
44. Eerens I, Vanbeckevoort D, Vansteenbergen W,
Van Hoe L. Autoimmune pancreatitis associated
with primary sclerosing cholangitis: MR imaging
findings. Eur Radiol 2001;11(8):1401–1404.
45. Horiuchi A, Kaneko T, Yamamura N, et al. Auto-
immune chronic pancreatitis simulating pancreatic
lymphoma. Am J Gastroenterol 1996;91(12):
2607–2609.
46. Saegusa H, Momose M, Kawa S, et al. Hilar and
pancreatic gallium-67 accumulation is characteris-
tic feature of autoimmune pancreatitis. Pancreas
2003;27(1):20 –25.
47. Nakamoto Y, Saga T, Ishimori T, et al. FDG-
PET of autoimmune-related pancreatitis: prelimi-
nary results. Eur J Nucl Med 2000;27(12):1835–
1838.
48. Pezzilli R, Casadei R, Calculli L, Santini D. Auto-
immune pancreatitis: a case mimicking carcinoma.
JOP 2004;5(6):527–530.
49. Inoue K, Ohuchida J, Ohtsuka T, et al. Severe lo-
calized stenosis and marked dilatation of the main
pancreatic duct are indicators of pancreatic cancer
instead of chronic pancreatitis on endoscopic ret-
rograde balloon pancreatography. Gastrointest
Endosc 2003;58(4):510 –515.
TM
RG Volume 28 • Volume 1 • January-February 2008 Kawamoto et al

Lymphoplasmacytic Sclerosing Pancreatitis (Autoimmune

Pancreatitis): Evaluation with Multidetector CT
Satomi Kawamoto, MD, et al
RadioGraphics 2008; 28:157–170 ● Published online 10.1148/rg.281065188 ● Content Codes:

Page 158
However, lymphoplasmacytic sclerosing pancreatitis responds to oral steroid therapy, with reversible
improvement of pancreatic morphology and function.

Page 161
Typically, CT images show diffuse enlargement of the pancreas. Focal enlargement may also be seen,
particularly in the pancreatic head, but also in the body or tail.

Page 165
On CT images, the main pancreatic duct may be seen as a small nondilated duct, or it may appear
attenuated, particularly in the area of the pancreatic enlargement.

Page 166
Stricture of the common bile duct is often seen in patients with lymphoplasmacytic sclerosing
pancreatitis, particularly in patients whose pancreatic head is affected, with a reported frequency of
33%–90%.

Page 168
CT findings more typically seen in pancreatic cancer than in lymphoplasmacytic sclerosing
pancreatitis include (a) significant dilatation of the main pancreatic duct proximal to the narrowed
segment, (b) atrophy of the pancreatic parenchyma proximal to the mass or focal enlargement, and
(c) involvement of the major peripancreatic vessels. In patients with segmental narrowing of the main
pancreatic duct due to lymphoplasmacytic sclerosing pancreatitis, the main pancreatic duct proximal
to the segmental narrowing typically shows minimal or no dilatation.