Two Cases of Autoimmune Pancreatitis with Coexisting...
Transcript of Two Cases of Autoimmune Pancreatitis with Coexisting...
Case ReportSt. Marianna Med. J.Vol. 37, pp. 261�267, 2009
Two Cases of Autoimmune Pancreatitis with Coexisting Features of
Tumor-Forming Pancreatitis.
Satoshi Kitajima1, Minoru Kobayashi1, Shunitirou Ozawa1, Ryujiro Negushi1,
Mami Hirakawa1, Hirokazu Obara1, Nobuyuki Obinata1, Masahito Nomoto1,
Tadateru Maehata1, Takashi Tuda1, Takefumi Kouro1, Takeshi Sawada1,
Yoshiyuki Watanabe1, Toshiya Ishii2, Akira Nikai2, Akira Sato2,
and Fumio Itoh1
�Received for Publication: June 11, 2009�
Abstract
Case 1: A 61-year-old male was hospitalized for evaluation of jaundice. The antinuclear antibody
�ANA� titer was 160, and rheumatoid factor �RF� was positive. Contrast abdominal CT showed di#useenlargement of the pancreas and a mass in the pancreatic head. ERCP revealed localized narrowing in the
main pancreatic duct in the pancreatic body. The patient was diagnosed as having autoimmune pancreatitis
�AIP�. Treatment with predonisolone �PSL� improved the pancreatic enlargement and reduced the size of themass in the pancreatic head. Case 2: A 74-year-old male was hospitalized with suspected cancer of the
pancreatic head. Serum IgG was 2,212 mg�dl, IgG4 was 915 mg�dl, and the anti-DNA antibody titer was 4.Abdominal US and abdominal contrast CT showed di#use enlargement of the pancreas and a mass in the
pancreatic head. As the jaundice decreased with endoscopic naso-biliary drainage, the pancreatic enlarge-
ment improved, and the mass in the pancreatic head decreased in size. Based on the clinical course, the
patient was diagnosed with AIP. Both of these patients appear to have had AIP with coexisting features of
tumor-forming pancreatitis �TFP�. Thus, AIP should be considered as an etiology of nonalcoholic TFP. Oneof the two patients initially diagnosed based on the 2002 diagnostic criteria for AIP had a confirmatory
diagnosis based on the 2006 revised clinical diagnostic criteria for AIP. Patients should be re-evaluated based
on the later criteria.
Key Words
Autoimmune Pancreatitis, Tumor-Forming Pancreatitis
Introduction
Autoimmune pancreatitis �AIP� was first de-scribed in 1961 by Sarles et al.1� as a chronic infla-
mmatory sclerosis of the pancreas involving an
autoimmune mechanism. In Japan, since 1992,
when Toki et al.2� reported a case of chronic pan-
creatitis with di#use irregular narrowing of the main
pancreatic duct, the number of cases has increased.
On the other hand, tumor-forming pancreatitis
�TFP� is often referred to clinically in the di#erentialdiagnosis of pancreatic cancer, but TFP is not a
well-defined clinical entity.3� In this report, we de-
scribe two patients with AIP and coexisting features
of TFP.
1 Department of Internal Medicine, Division of Gastroenterology and Hepatology, St. Marianna University School ofMedicine.2 Department of Gastroenterology, St Marianna University School of Medicine Yokohama Seibu Hospital.
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Case Reports
Case 1: This 61-year-old male developed general-
ized malaise and dark urine in late August of 2002
and was hospitalized by his physician for evaluation
of hepatic dysfunction. The history was negative for
alcohol consumption. Physical examination re-
vealed jaundice and scleral icterus. Abdominal ex-
amination revealed a palpable, nontender mass be-
neath the right subcostal margin. Laboratory data
on admission included a blood sedimentation rate
of 61 mm�h �moderately elevated�, a direct bilirubinof 5.1 mg�dl, an antinuclear antibody �ANA� titer of160, and a positive rheumatoid factor �RF�. Con-trast abdominal CT during the parenchymal phase
showed di#use enlargement of the pancreas and a
mass in the pancreatic head, but a corresponding
low-density area suspicious of pancreatic cancer
was not present �Fig 1a, b�. MRCP showed narrow-ing of the lower common bile duct and localized
narrowing of the main pancreatic duct �Fig. 2a�.PTBD was performed for obstructive jaundice.
PTC showed smooth extrinsic narrowing of the
lower common bile duct margin �Fig. 3a�. OnERCP, localized narrowing of the main pancreatic
duct in the pancreatic body, but no post-stenotic
dilation, was observed. In addition, there was no
narrowing or obstruction of the main pancreatic
duct in the pancreatic head �Fig. 3b�. On cholan-gioscopy, there was no irregularity or redness of the
common bile duct mucosa; thus, the narrowing was
due to extrinsic compression. Based on these fin-
dings, the patient was diagnosed as having AIP, and
treatment with predonisolone �PSL� 30 mg per day
Figure 1. Contrast abdominal CT image of case 1 during the parenchymal phase.
a: CT showed di#use enlargement of the pancreas.
b: A mass was seen in the pancreatic head, but a corresponding low-density area suspicious of pancreatic
cancer was not present. The arrow indicates a mass in the pancreas head.
Figure 2. MRCP image of case 1.
a: MRCP showed narrowing of the lower common bile duct and localized narrowing of the main
pancreatic duct.
b: Narrowing of the lower common bile duct improved after treatment with PSL.
Kitajima S Kobayashi M et al262
86
was started. The pancreatic enlargement and nar-
rowing of the lower common bile duct improved
�Fig. 2b�.
Case 2: This 74-year-old male began to experience
left-sided abdominal pain in March 2003. In May
2003, he noticed clay-colored stools and jaundice.
Since abdominal CT showed enlargement of the
pancreatic head, he was admitted to hospital with
suspected pancreatic cancer. His alcohol consump-
tion consisted of one bottle beer per day. Physical
examination revealed jaundice and scleral icterus.
Abdominal examination revealed an elastic, hard
mass on the left side; the patient complained of
abdominal pain in the area of the mass, with associ-
ated tenderness on palpation. Laboratory data on
admission showed a blood sedimentation rate of 30
mm�h �mildly elevated�, direct bilirubin of 10.8mg�dl, serum IgG of 2,212 mg�dl, IgG4 of 915mg�dl, and an anti-DNA antibody titer of 4. Con-trast abdominal CT during the parenchymal phase
showed di#use enlargement of the pancreas and a
low-density mass in the pancreatic head �Fig. 4a, b�.On abdominal US, sausage-shaped enlargement of
the entire pancreas, a 5-cm mass in the pancreatic
head, and mild dilation of the main pancreatic duct
in the pancreatic body were present �Fig. 5a, b�.Contrast abdominal MRI showed no di#erence in
signal intensity between the pancreatic parenchyma
and the mass in the pancreatic head. MRCP showed
no significant dilation in the main pancreatic duct
of the pancreatic body and tail, but intrahepatic bile
duct dilation and lower common bile duct narrow-
ing were present �Fig. 6�. On ERCP, as on MRCP,intrahepatic bile duct dilation and smooth narrow-
ing of the lower common bile duct margin were
observed. Contrast visualization of the main pan-
creatic duct was di$cult. Endoscopic naso-biliary
drainage was performed, and the jaundice im-
proved. After catheter removal, there was no exac-
erbation of jaundice, and contrast abdominal CT
showed improvement in the pancreatic enlargement
and a reduction in size of the mass in the pancreatic
head �Fig. 4c, d�. The patient’s symptoms improvedduring follow-up.
Discussion
Interest in AIP has increased, with a growing
number of case reports. Etemad and Whitcomb4�
have proposed a new classification for chronic pan-
creatitis �TIGAR-O�, which includes AIP as an eti-ology. In 2002, the Japan Pancreas Society pub-
lished diagnostic criteria for AIP. In the present
report, the diagnosis of AIP in our patients was
based on these 2002 diagnostic criteria for AIP. 5� In
patient 1, with pancreatic enlargement, positive
ANA, and positive RF, the criteria regarding nar-
rowing of the main pancreatic duct were not met.
Patient 2 had a history of alcohol consumption, but
pancreatic calcification and irregular pancreatic
Figure 3. PTC and ERCP image of case 1.
a: PTC showed smooth extrinsic narrowing of the lower common bile duct margin. The arrow indicates
narrowing of the lower common bile duct margin.
b: Localized narrowing of the main pancreatic duct in the pancreatic body, but no post-stenotic dilation,
was observed. In addition, there was no narrowing or obstruction of the main pancreatic duct in the
pancreatic head. The arrow indicates localized narrowing of the main pancreatic duct in the pancreatic
body.
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Figure 4. Contrast abdominal CT image of case 2 during the parenchymal phase.
a, b: CT showed di#use enlargement of the pancreas and a low-density mass in the pancreatic head. The
arrow indicates a mass in the pancreas head.
c, d: CT showed improvement in the pancreatic enlargement and a reduction in size of the mass in the
pancreatic head after endoscopic naso-biliary drainage.
Figure 5. Abdominal US image of case 2.
a: US showed sausage-shaped enlargement of the entire pancreas.
b: US showed a 5-cm mass in the pancreatic head and mild dilation of the main pancreatic duct in the
pancreatic body were present. The arrow indicates a mass in the pancreas head.
Kitajima S Kobayashi M et al264
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margins, as defined in the diagnostic criteria for
chronic pancreatitis, were not present. Di#use en-
largement of the pancreas was observed. Elevated
IgG and IgG4 levels suggested AIP, but ERCP did
not visualize the main pancreatic duct, thus not
meeting the diagnostic criteria. However, in patient
1, based on the revised clinical diagnostic criteria
for AIP in 2006,6�7� except for narrowing of�1�3 ofthe main pancreatic duct, the diagnostic criteria
were fitted. In addition, elevated IgG4 is reported to
be useful in the di#erential diagnosis between AIP
and other disorders, including pancreatic cancer,8�9�
and in the 2006 clinical diagnostic criteria for AIP,
elevated IgG4 is listed. Therefore, in patient 2, even
though the main pancreatic duct was not visualized
with contrast, the findings were consistent with AIP.
Among the case reports that we reviewed, AIP was
often diagnosed, as in our patients, based on clini-
cal findings, even though all diagnostic criteria were
not fitted. Re-evaluation based on the revised
guidelines is necessary.10�11�
TFP is a clinical entity that is often di$cult to
distinguish from cancer and may represent the so-
called tumor-forming stage of dynamically chang-
ing chronic pancreatitis.3� The etiology of TFP is
broadly classified as alcoholic and nonalcoholic,
and in the latter, an autoimmune mechanism is
thought to play a role.12� TFP often involves the
pancreatic head, but as compared to pancreatic
cancer, the incidence of jaundice and obstruction �narrowing of the main pancreatic duct is lower.
Even if the main pancreatic duct is narrowed, dila-
tion of the pancreatic duct in the pancreatic tail is
usually not present. Furthermore, smooth narrow-
ing of the common bile duct is often observed.13� The
di#erential diagnosis between TFP and pancreatic
cancer, especially with tumor fibrosis, can be
di$cult based on CT or MRI contrast e#ects in the
parenchyma. In these cases, the “duct penetrating
sign” may be useful in the di#erential diagnosis.14�
AIP is usually associated with di#use pancreatic
enlargement, but localized enlargement can also
occur. This can be a factor in TFP, and, in particu-
lar, when the pancreatic head is enlarged, the di#e-
rential diagnosis with pancreatic cancer may be
di$cult. However, when pancreatic head enlarge-
ment is part of more di#use pancreatic enlargement,
the presence of a capsule-like rim around the pan-
creas is helpful in diagnosis. Histologically, this is a
fibrous capsule, so on dynamic CT or MRI, there is
gradual enhancement.15� Our each patient also had
mass in the pancreatic head, making it di$cult to
distinguish from pancreatic cancer, but the di#use
pancreatic enlargement and absence of main pan-
creatic duct dilation helped in the di#erential diag-
nosis.
However, in AIP, narrowing of the common
bile duct within the pancreas can cause obstructive
jaundice, thus making it di$cult to di#erentiate
from pancreatic head cancer or bile duct cancer.
The narrowing often occurs in the lower common
bile duct and is relatively smooth; it is probably due
to compression by the enlarged pancreas, but coex-
isting biliary lesions in AIP have also been
reported.16� It is necessary to care for diagnosis be-
cause CT and�or intraductal ultrasound show
thickening of the bile duct wall, and the findings in
some cases may resemble primary sclerosing
cholangitis.17�18� Both of our patients had smooth
narrowing of the lower bile ducts, but no other
findings were evident.
Conclusions
1. We reported two patients with AIP and coexist-
ing features of TFP.
2. Much remains unknown about the etiology of
TFP, but in nonalcoholic TFP, underlying AIP
should be kept in mind.
3. Suspected cases of AIP should be re-evaluated
based on the revised clinical diagnostic criteria for
AIP.
Figure 6. MRCP image of case 2.
MRCP showed no significant dilation in the main
pancreatic duct of the pancreatic body and tail, but
intrahepatic bile duct dilation and lower common bile
duct narrowing were present.
AIP Coexist Tumor-Forming Pancreatitis 265
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