AJR:183, October 2004 915
Lymphoplasmacytic Sclerosing
Pancreatitis with Obstructive
Jaundice: CT and Pathology Features
OBJECTIVE. The clinical presentation of lymphoplasmacytic sclerosing pancreatitis (LPSP)
can be very similar to that of pancreatic cancer, with no statistically significant differences in the
rates of abdominal pain, weight loss, jaundice, or levels of carcinoembryonic agent or cancer an-
tigen 19-9. The purpose of this study is to describe and illustrate the CT features of LPSP present-
ing with obstructive jaundice and to correlate CT and pathology findings.
MATERIALS AND METHODS. Five patients with LPSP were evaluated. Morphologic
features of the pancreas on CT scans, including the size of the pancreas, presence or absence
of a mass, segmental difference of contrast enhancement, pancreatic duct, major pancreatic
vasculature, and biliary tract, were retrospectively evaluated and correlated with histopathol-
ogy. The degree of contrast enhancement of the pancreas was compared in 10 patients without
LPSP, who were scanned with the same protocol.
RESULTS. CT scans showed diffuse (n = 2) or focal (n = 3) enlargement of the pancreatic
head. The normal lobular appearance of the pancreas was effaced, and the gland appeared fea-
tureless in the involved region. Enlarged areas showed an enhancement pattern similar to that
of the rest of the pancreas, and no segmental difference of contrast enhancement was identi-
fied. Pancreatic duct dilatation was not seen in any patient. Thickening and contrast enhance-
ment of the common bile duct wall (n = 4) and gallbladder wall (n = 3) were observed and
were pathologically correlated with inflammatory infiltrate and fibrosis of the common bile
duct (n = 3) and gallbladder (n = 1).
CONCLUSION. When these findings are encountered, further evaluation with serologic
tests or biopsy may aid in the diagnosis of LPSP.
ancreaticoduodenectomy, also
known as the Whipple procedure,
is now a relatively safe procedure
at centers of excellence with a low mortality
rate and acceptable morbidity rate [1]. At
leading centers, surgeons have espoused an
aggressive approach to patients with putative
periampullary malignancy, often eschewing
biopsy before or during operation [2]. As a
consequence, small numbers of patients (40/
442 at Johns Hopkins [3], 29/603 at the Mayo
Clinic [4], and 14/220 at the University of
Amsterdam [5]) have Whipple procedures
performed for benign disease that cannot be
distinguished from cancer before surgery.
Pancreatitis is the most common benign con-
dition that mimics pancreatic cancer, and in
recent experience at Johns Hopkins, lympho-
plasmacytic sclerosing pancreatitis (LPSP) is the
most common form of pancreatitis in patients
who are subjected to pancreaticoduodenectomy
for suspected cancer.
LPSP is a distinctive form of chronic pan-
creatitis characterized by a mixed inflamma-
tory infiltrate that centers on the pancreatic
ducts [3]. LPSP is also variously termed
“nonalcoholic duct destructive chronic pan-
creatitis” [6], “sclerosing pancreatitis” or
“sclerosing pancreatic cholangitis,” and “au-
toimmune pancreatitis” [7, 8]. The pathogen-
esis of LPSP is unknown, but it is
hypothesized to be a form of autoimmune
pancreatitis. Yoshida et al. [7] reported
unique features of this disorder, including in-
creased serum γ-globulin, presence of au-
toantibodies, absence of acute attacks of
pancreatitis, diffuse enlargement of the pan-
creas, and diffuse irregular narrowing of the
main pancreatic duct on endoscopic retro-
grade pancreatography. Occasional associa-
Satomi Kawamoto
1
Stanley S. Siegelman
1
Ralph H. Hruban
2
Elliot K. Fishman
1
Received September 29, 2003; accepted after revision
February 16, 2004.
1
The Russell H. Morgan Department of Radiology and
Radiological Science, Johns Hopkins Hospital, 600 N Wolfe
St., Baltimore, MD 21287. Address correspondence to
S. Kawamoto (skawamo1@jhmi.edu).
2
Department of Pathology, Johns Hopkins Hospital,
Baltimore, MD.
AJR 2004;183:915–921
0361–803X/04/1834–915
© American Roentgen Ray Society
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