<Presentation of a
Case>
This 71-year-old man visited the emergency room (ER)
because of progressive itching and jaundice for 2 weeks
This patient had hypertension and diabetes for which he had
received oral antihypertensives and oral hypoglycemic agents
for 30 years and 5 months, respectively; benign prostate
hypertrophy and gall bladder stone fro which he underwent
transurethral prostatectomy and laparoscopic cholecystectomy 7
and 2 years earlier, respectively. He had smoked for 50 year
but had quitted 2 years earlier. He did not consume alcohol.
He had been quite well before except mild acid regurgitation
occasionally. About 2 weeks prior to this admission,
progressive itching and jaundice developed and his appetite
became poor . He lost weight by 15 kg over the past 5 months (
a weight change from 78 Kg to 63 Kg). In addition, tea-color
urine and clay-color stool was noted for 1 week before entry.
He sought medical attention at a local clinic and was
transferred to an outside hospital because of jaundice. In the
hospital, the level of bilirubin was 8.0 mg/dl and the
sonography revealed mild dilatation of common bile duct (CBD)
(13 mm) and a clear pancreatic shadow . The computed
tomography (CT) of the abdomen revealed dilatation of CBD and
intra-hepatic ducts (IHD) and the diagnosis of chronic
pancreatitis was made. He then came to our ER for further
help.
On physical examination, this patient was clear in
consciousness. The temperature was 36.6 0C, the
pulse was 64 beats per minute and the respiration rate was 20
times per minutes. Blood pressure was 106/70 mmHg. His
conjunctivae were pink and sclera were icteric. His neck was
supple without lymphadenopathy. Chest examination showed
symmetric expansion with clear breathing sound. No spider
angioma or caput medusa was noted. Heart auscultation yielded
regular heart beat without murmur. His abdomen was soft and
flat and no tenderness or rebounding tenderness. The liver and
spleen were not enlarged. Bowel sound was normoactive and his
extremities had no edema.
<Laboratory
data>
1. Hemogram
WBC |
RBC |
Hb |
HT |
MCV |
PLT |
K/uL |
M/uL |
g/dL |
% |
fL |
K/uL |
7.85 |
4.04 |
11.9 |
35.3 |
87.4 |
260 |
Meta |
Band |
Seg |
Eos |
Baso |
Mono |
Lym |
0 % |
0 % |
59.3 % |
0.9% |
0.3% |
8.2 % |
31.3 % |
Alb |
Glo |
T-Bil |
D-Bil |
AST |
ALP |
r-GT |
g/dL |
g/dL |
mg/dl |
mg/dl |
U/L |
u/L |
u/L |
3.2 |
3.7 |
17.07 |
12.56 |
44 |
624 |
77 |
BUN |
CRE |
ALT |
GLU |
Amy |
Lip |
CEA |
CA19-9 |
mg/dl |
mg/dl |
U/L |
mg/dl 1 |
u/L |
u/L |
ng/mL |
u/mL |
11.0 |
1.0 |
52 |
212 |
76 |
13 |
3.15 |
78.1 |
<Course and
Treatment>
At ER, abdominal sonography revealed
dilatation of CBD & IHD and diffuse swelling of pancreas
(Fig.
1 ) with a 3.2 cm hypoechoic
lesion at the pancreatic head with reticular pattern (Fig. 2). The CT of the abdomen
revealed diffuse dilatation of bilateral IHD (Fig. 3) with acute tappering of the
distal CBD and a focal well-defined tumor at the pancreatic
head (Fig. 4
); and spotty calcifications were
noted, which was suggestive of chronic pancreatitis. There was
no evidence of CBD stone. The endoscopic retrograde
cholangiopancreatography (ERCP) was performed but failed due
to difficult cannulation. Under the impression of obstructive
jaundice with chronic pancreatitis and pancreatitic head tumor
, he underwent percutaneous transhepatic cholangiographic
drainage (PTCD) and the jaundice improved gradually. A surgeon
was consulted and Whipple operation was performed after his
condition stabilized. Operative findings showed the whole
pancreas tissue was firm, hard, and adhesive to the
surrounding tissue. The pathology revealed chronic fibrosing
pancreatitis with eosinophilia without evidence of malignancy.
He was then discharged receiving outpatient follow-up.
However, several episodes of pancreatitis occurred to him and
were managed at outside hospitals. His diabetes became
difficult to control. Elevated IgG (1730 mg/dL; normal range:
700-1600 mg/dL) and IgG4 (564 mg/dL; normal range: 60-130
mg/dL) were noted. ANA, C3 and C4 were relatively normal.
Autoimmune pancreatitis was diagnosed from the clinical course
and therefore prednisolone (10 mg tid) was given for 2 months
and was tapered off gradually. His blood glucose became
stabilized and he was doing well with during follow-up as an
outpatient
<Discussion and
Analysis>
Sarles el al. has reported a type of chronic pancreatitis
with hyperglobulinaemia and probably an autoimmune mechanism.
The nomenclature included autoimmune pancreatitis (AIP),
sclerosing pancreatitis, primary inflammatory pancreatitis,
lymphoplasmatocytic sclerosing pancreatitis (LPSP), chronic
pancreatitis with irregular narrowing of the main pancreatic
duct and sclerosing pancreatocholangitis. AIP has been
associated with several autoimmune diseases, including
Sjogren's syndrome, primary biliary cirrhosis (PBC), primary
sclerosing cholangitis (PSC), inflammatory bowel disease,
retroperitoneal fibrosis, and autoimmune hepatitis.
AIP usually presented with obstructive jaundice, weight
loss, abdominal pain, general malaise, and DM.
Hypergammaglobulinemia was its feature and maybe associated
with elevated autoantibody titers (ANA, anti-lactoferrin
antibod, anti-carbonic anhydrase II antibodies, RF). High
levels of IgG and/or IgG4 was most characteristic. Radiologic
findings included diffuse enlargement of the pancreas and
focal masses in the pancreas. Pancreatic calcification or
pseudocyst formation is seldom observed. ERCP may shown
segmental or diffuse narrowing of the main pancreatic duct and
frequent stenosis of the lower bile duct. MRCP is poor in
showing stenosis of the pancreatic duct but it can adequately
demonstrate stenosis of the bile ducts. Histologically, it
showed a chronic, immune-mediated, fibroinflammatory process.
Dense lymphocytic infiltrate centered around the medium-sized
interlobular and large pancreatic ducts. Plasma cells ,
eosinophils and neutrophils infiltration may also be found.
Clustering of lymphocytes around veins ('periphlebitis') with
occasional obliterative phlebitis is another feature.
The diagnosis of AIP was based in the combination of
clinical, laboratory findings and imaging studies. Japan
Pancreas Society has set a diagnostic criteria on 2002, which
included:
- Pancreatic image studies show
diffuse narrowing of the main pancreatic duct with irregular
walls and diffuse enlargement of the pancreas;
- Laboratory data demonstrate
abnormally elevated serum gamma-globulin, and/or IgG, or the
presence of autoantibodies;
- Histopathological examination shows fibrotic changes
with lymphocyte and plasma cell infiltration. The diagnosis
of AIP is made when criteria 1 plus criteria 2 and/or 3 are
met.
DM is often (43-68%) present in patients with AIP, and the
majority of them showed type 2 DM. Some type 2 DM patients
associated with AIP improve after steroid therapy. The
mechanism is still unclear.
The relationship of AIP and pancreatic cancer deserves
special attention. Clinical difficulties in differential
diagnosis were the most important problem. They may share
similar features such as frequent stenosis of the bile duct,
elevation of serum CA 19-9, segmental pancreatic enlargement
or narrowing of the main pancreatic duct. At least 5% of
patients who undergo surgery for cancer of the head of the
pancreas are found to have benign inflammatory disease
The treatment of AIP was mainly based on steroid, which is
usually effective for extra-pancreatic and pancreatic lesions.
The jaundice sometimes needs PTCD or ERCP to relieve. Some
patients may spontaneously improve. Surgical intervention may
be necessary to those unresponsive to steroid therapy for
symptomatic relief. Long-term prognosis of AIP remains
unknown.
<References>
- Ketikoglou I, et al. Dig Liver Dis. 2005;37(3):211-5
- Okazaki K, et al. Gut 2002;51:1-4
- Tanaka S, et al. Lancet 2000;356:910-1
- Kamisawa T, et al. Am J Gastroenterol.
2003;98(12):2694-9
- Van Gulik TM, et al. Gastrointest Endosc 1997;46:
417–23
- Okazaki K, et al. Intern. Med. 2005; 44:
1215-1223
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