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Deciphering Autoimmune Pancreatitis- A Great Mimicker:
Case Report and Review of the Literature
Satya Allaparthi, MD ¹, Mohammed Sageer ,MD ¹, Mark J Sterling ¹, MD,
¹ Lahey Clinic, Burlington, MA.
Abstract
Background
Autoimmune pancreatitis (AIP) is an atypical chronic inflammatory pancreatic
disease that appears to involve autoimmune mechanisms. In recent years, AIP
has presented as a new clinical entity with its protean pancreaticobiliary and
systemic presentations. Its unique pathology and overlap of clinical, radiological
features and absence of serological markers foster the disease’s unique position.
We report a case of diffuse type-1 autoimmune pancreatitis with obstructive
jaundice managed with biliary sphincterotomy, stent placement and
corticosteroids. A 50-year-old Caucasian woman presented to our hospital with
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epigastric pain, nausea, vomiting and jaundice. Workup showed elevated liver
function tests (LFT) suggestive of obstructive jaundice, MRCP done showed
diffusely enlarged abnormal appearing pancreas with loss of normal lobulated
contours and IgG4 antibody level was 765 mg/dL. EUS revealed a diffusely
hypoechoic and rounded pancreatic parenchyma with distal common bile duct
(CBD) stricture and dilated proximal CBD, common hepatic duct (CHD). ERCP
showed tight mid to distal CBD stricture that needed dilatation, sphincterotomy
and placement of stent led to significant improvement in the symptoms and
bilirubin level. Based on clinical, radiological and immunological findings a
definitive diagnosis of AIP was made. She was started on prednisone 40 mg/day
and 4 weeks after treatment clinically, radiologically symptoms resolved.
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Deciphering Autoimmune Pancreatitis- A Great Mimicker:
Case Report and Review of the Literature
Satya Allaparthi, MD¹, Mohammed Sageer ,MD ¹, Mark J Sterling ¹, MD,
¹ Lahey Clinic, Burlington, MA.
Correspondence to: Satya Allaparthi, MD, Department of Gastroenterology ,
Lahey Clinic, 41 Mall Road, Burlington MA 01805 surgsatya@yahoo.com
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Keywords :
Autoimmune pancreatitis ; Pancreatic cancer ; Magnetic resonance
cholangiopancreatography (MRCP) ; Endoscopic retrograde
cholangiopancreatography (ERCP) ; Endoscopic ultrasound-guided fine needle
aspiration (EUS) · IgG4 ·
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Abstract
Background
Autoimmune pancreatitis (AIP) is an atypical chronic inflammatory pancreatic
disease that appears to involve autoimmune mechanisms. In recent years, AIP
has presented as a new clinical entity with its protean pancreaticobiliary and
systemic presentations. Its unique pathology and overlap of clinical, radiological
features and absence of serological markers foster the disease’s unique position.
We report a case of diffuse type-1 autoimmune pancreatitis with obstructive
jaundice managed with biliary sphincterotomy, stent placement and
corticosteroids. A 50-year-old Caucasian woman presented to our hospital with
epigastric pain, nausea, vomiting and jaundice. Workup showed elevated liver
function tests (LFT) suggestive of obstructive jaundice, MRCP done showed
diffusely enlarged abnormal appearing pancreas with loss of normal lobulated
contours and IgG4 antibody level was 765 mg/dL. EUS revealed a diffusely
hypoechoic and rounded pancreatic parenchyma with distal common bile duct
(CBD) stricture and dilated proximal CBD, common hepatic duct (CHD). ERCP
showed tight mid to distal CBD stricture that needed dilatation, sphincterotomy
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and placement of stent led to significant improvement in the symptoms and
bilirubin level. Based on clinical, radiological and immunological findings a
definitive diagnosis of AIP-type 1 was made. She was started on prednisone 40
mg/day and 4 weeks after treatment clinically, radiologically symptoms resolved.
7| P a g e
Introduction:
In 1961, Sarles et al [1] first described a case series of unusual pancreatitis
associated with obstructive jaundice and hyper-g globulinemia. However, it was
not until 1995 when Yoshida et al[2] termed autoimmune pancreatitis to describe
a case of diffusely enlarged pancreas with irregularly narrowing pancreatic duct
that was serologically associated with hyper-g-globulinemia , anti-nuclear
antibody positivity and responsive to steroid treatment .
Clinically, AIP is characterized by protean symptoms that have many features in
common with pancreatic cancer. These symptoms include abdominal pain,
obstructive jaundice, weight loss, steatorrhea, new-onset diabetes mellitus (DM)
and elevated levels of serum tumor markers. Hardacre et al in their single
institutional study reported that about 2.5% of pancreato -duodenectomies were
performed in AIP patients following a mistaken diagnosis of pancreatic cancer
[3]. Since AIP responds extremely well to steroid therapy, it is of utmost
importance that it be differentiated from pancreatic cancer to avoid unnecessary
laparotomy or pancreatic resection. AIP is frequently associated with other
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systemic autoimmune diseases, such as rheumatoid arthritis, Sjogren’s syndome,
sarcoidosis and inflammatory bowel diseases.
The diagnosis of AIP is challenging as it closely mimics pancreatic cancer. We
further report a case of 50 year old Caucasian woman who presented with
epigastric pain, nausea, vomiting and jaundice. Subsequent work -up revealed
AIP type-1 as the etiology of her symptoms, and was treated effectively with
steroids.
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Case Presentation and Mangement:
A 50-year-old Caucasian woman presented to our hospital with 2 days duration
of epigastric pain, nausea, vomiting and jaundice. Her physical examination was
unremarkable except for scleral icterus. An abdominal examination revealed
epigastric tenderness without rebound. Laboratory investigations revealed
hemoglobin 12.9 g/dL, white blood cell count 9.6/μL, serum lipase 109 U/L,
serum amylase 10 U/L, total bilirubin 10.6 mg/dl (direct and indirect fractions 8
mg/dL and 2.6 mg/dL, respectively). Her liver enzymes were elevated (aspartate
aminotransferase 110 U/L, alanine aminotransferase 131 U/L and alkaline
phosphatase 389 U/L). Tests for hepatitis A, B and C were negative and
immunoglobulin G4 antibody level of 765 mg/dL. Magnetic resonance
cholangiopancreaticography (MRCP) done showed diffusely enlarged abnormal
appearing pancreas with loss of normal lobulated contours. The pancreatic
parenchyma is diffusely hypo enhancing, with focal hypo enhancement within
pancreatic head, distal body and tail. In view of her clinical presentation with
worsening jaundice and MRCP findings an endoscopic ultrasound (EUS) was
done that revealed a diffusely hypoechoic and rounded pancreatic parenchyma
with distal common bile duct (CBD) stricture and dilated proximal CBD and
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common
hepatic
duct
(CHD).
Endoscopic
retrograde
cholangiopancreaticography (ERCP) showed tight mid to distal CBD stricture
that needed dilatation, sphincterotomy and placement of stent that significantly
improved her clinical symptoms and bilirubin level. She was started on tapering
dose of prednisone 40 mg/day and 4 weeks post treatment she improved
clinically and radiologically. Our ability to recognize AIP type -1 and
differentiate it from pancreatic adenocarcinoma is aided by the use of
international consensus criteria.
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Discussion:
Autoimmune pancreatitis is rare disease with a much lower rate of incidence
than its principal differential diagnosis, pancreatic cancer. The overall incidence
and prevalence is still unclear owing to lack of prospective natural history
studies. Study series from Japan have reported the prevalence of autoimmune
pancreatitis as between 5 and 6% of all patients with chronic pancreatitis and
range from 0.82 per 100,000 to 6-8% of pancreatic resections performed for
presumed pancreatic cancer [4, 5]. Moreover, in the last two decades, there has
been an increase in the number of reports of autoimmune pancreatitis in the
medical literature; however the overall number of patients is still relatively small.
Though this clinical entity was well described initially in Japan, a growing
awareness of the condition has led to reports around the world. Hamano et al [6]
reported that serum IgG4 levels are specifically elevated in Japanese patients
with AIP, however in further reported case series by Hirano et al [7] and Pezzeli
et al [8] an increase of IgG4 levels in AIP cohorts ha s been also confirmed in
Western and Eastern countries . Although, the pathogenesis of the disease is
unknown, current evidence strongly suggests that an autoimmune process has
been implicated [9, 10]. Unlike most autoimmune conditions, AIP has a male
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predominance, with a male:female ratio of 2:1. The peak age of onset is the sixth
and seventh decade [5] [9, 11].
The histopathological hallmark findings in patients with AIP include dense
infiltration of T lymphocytes, IgG4-positive plasma cells, storiform fibrosis, and
obliterative phlebitis in the pancreas; this form is termed lymphoplasmacytic
sclerosing pancreatitis (LPSP) [12-14]. Of late, recent studies have provided
evidence that there is another subtype of AIP with different histological findings
named as idiopathic duct-centric pancreatitis (IDCP) that is more prevalent in
Europe and the United States [15, 16].Recent studies showed consensus that
LPSP and IDCP are regarded as two distinct subtypes of AIP, and it has been
proposed that LPSP be called ‚type 1 AIP‛ and IDCP ‚type 2 AIP‛[17, 18].
In 2011, an international panel of experts met during the 14th Congress of the
International Association of Pancreatology held in Fukuoka, Japan and an
international consensus diagnostic criterion for AIP was proposed [19] .
According to these, AIP is classified into type 1 and 2. Five cardinal features of
AIP are used: imaging of pancreatic parenchyma and ducts; serology; other
organ involvement; pancreatic histology; and an optional criterion of response to
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steroid therapy. Each feature is categorized as a level 1 or 2 finding, depending
on the diagnostic reliability. The diagnosis of type 1 and type 2 AIP can be
definitive or probable and in some cases, the distinction between the subtypes
may not be possible (AIP-not otherwise specified) (Tables 1 and 2).
AIP should always be included in the differential diagnosis particularly in
elderly presenting with obstructive jaundice and a pancreatic mass. Prior to
initiation of therapy it is of the paramount importance to differentiate AIP from
pancreatic cancer. Various strategies help differentiate the clinical,
immunological, radiological presentations in between AIP and pancreatic cancer.
Obstructive jaundice induced by bile duct stenosis secondary to pancreatic
cancer typically progresses steadily, whereas the jaundice of AIP in IgG4-related
sclerosing disease sometimes fluctuates or, in rare cases, improves spontaneously
[13, 20]. Elevated serum levels of IgG4 (> 135 mg/dL) are seen in more than 90%
of patients with AIP [6]. This is the most sensitive and specific diagnostic test for
type I AIP, with 95% sensitivity, 97% specificity, and 97% accuracy for
discrimination from pancreatic cancer [6]. Elevation of serum IgG4 levels alone
cannot rule out pancreatic cancer as stated in their study series by Ghazale et al
[21], where in a serum IgG4 levels were elevated in 13/135 (10%) of pancreatic
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cancer patients; however, only 1% had IgG4 levels > 280 mg/dL, compared with
53% of AIP patients. Presence of other organ involvements such as bilateral
salivary gland swelling, retroperitoneal fibrosis and hilar or intrahepatic
sclerosing cholangitis is highly suggestiveof AIP rather than pancreatic cancer.
Radiological studies aid in differentiation based on the characteristic features on
computed tomography (CT) and magnetic resonance imaging (MRI) that include
diffuse or focal pancreatic enlargement, a peri-pancreatic capsule-like rim,
enhancement at the late phase of contrast-enhanced images, and abnormal signal
intensity on MRI.. Diffuse enlargement of the pancreas and effacement of the
lobular contour of the pancreas, the so-called ‚sausage-like‛ appearance, is a
typical finding in AIP, and is rarely seen in pancreatic cancer. On delayed phase
of dynamic CT and MRI, enhancement of an enlarged pancreas is characteristic
of AIP. As fibro inflammatory changes involve the peripancreatic adipose tissue,
a capsule-like rim surrounding the pancreas, is specifically detected in some AIP
patients [22, 23] . The role of trans abdominal ultrasonography in the diagnosis of
autoimmune pancreatitis is not well established. Ultrasonographic images of the
pancreas, obtained transabdominally are rarely diagnostic of autoimmune
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pancreatitis. Furthermore, findings on ultrasonography may be similar for
autoimmune pancreatitis and for other forms of acute and chronic pancreatitis.
The hallmark finding on endoscopic retrograde cholangiopancreatography
(ERCP) in patients with autoimmune pancreatitis is diffuse or segmental
attenuation of the main pancreatic duct (MPD ), in contrast to the segmental
stenoses often encountered with pancreatic adenocarcinoma.. The other common
findings are narrowing of the intrapancreatic portion of the common bile duct,
irregular narrowing of extrahepatic bile ducts, and less frequently, enlarged
intrahepatic bile ducts [24]. It is of paramount importance to distinguish reliably
distinguish AIP from PSC as making a reliable diagnosis is critical due to the
often dramatic response of AIP related biliary strictures to steroid therapy in
contrast to equivocal response seen in primary sclerosing cholangitis (PSC) and
cholangiocarcinoma. The presence of a long, monomorophic stenosis of the
intrapancreatic bile duct is suggestive of AIP, while band-like strictures, beading
or prune-tree appearance is most often found in PSC. Magnetic resonance
cholangiopancreaticography (MRCP) has become popular as a non-invasive
method and it is becoming preferable to diagnostic ERCP. However, the
narrowest MPD seen on ERCP cannot be visualized by MRCP due to the inferior
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resolution of MRCP compared with ERCP, so distinguishing between narrowing
of the MPD in AIP and stenosis of the MPD in pancreatic cancer is not possible
[25].
Endoscopic ultrasonography (EUS) emerged as a particularly important
pancreatic imaging tool due to its ability to provide high-resolution imaging
along with short working distances for transluminal pancreatic interventions.
Though non- specific most common finding on EUS is diffuse or focal pancreatic
enlargement along with a diffusely hypoechoic parenchyma, as well as features
of generalized parenchymal inflammation including inhomogeneous echo
pattern, stranding and calcification [26]. EUS guided fine-needle aspiration or
core biopsy of the pancreas may aid in the cytologic or histologic diagnosis,
however this approach to tissue acquisition was generally proven inadequate in
providing a definitive diagnosis of AIP owing to a small sample size and lack of
preserved tissue architecture and has not been evaluated in larger trials [26, 27].
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Conclusion:
In summary, we further report another case of AIP that reemphasizes the
importance and various strategies of distinguishing it from pancreatic
adenocarcinoma in order to avoid unnecessary surgical intervention. The concept
of a ‘great mimicker’ may be invoked and a heightened vigilance of AIP in one’s
differential diagnosis must be emphasized. As it is sometimes difficult to obtain
adequate biopsy material from the pancreas, AIP is currently diagnosed based on
careful consideration of a combination of characteristic clinical, serological,
morphological, and histo pathological features. More widespread use of
pancreatic biopsy will aid in the diagnosis of autoimmune pancreatitis and
provide a secure basis for the treatment with corticosteroids. Combined with a
lack of prospectively validated clinical criteria that reliably establish the
diagnosis, it is expected that the endoscopist will continue to play a central role
in the diagnosis and management of AIP in the future.
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Abbreviations:
Auto immune pancreatitis (AIP),
Main pancreatic duct (MPD),
Common bile duct (CBD)
Common hepatic duct (CHD).
Lymphoplasmacytic sclerosing pancreatitis (LPSP),
Idiopathic duct-centric pancreatitis (IDCP)
Computer tomography (CT),
Magnetic resonance imaging (MRI)
Magnetic resonance cholangiopancreatography (MRCP)
Endoscopic retrograde cholangiopancreatography (ERCP)
Endoscopic ultrasound-guided fine needle aspiration (EUS)
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Acknowledgements:
Author contributions: Allaparthi S, Sageer M, Sterling M, reviewed,
designed, edited, and organized the report; Sterling M served as the attending
doctors for the patient; Allaparthi S performed the literature review and wrote
the paper.
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REFERENCES:
1.
Sarles, H., et al., Chronic inflammatory sclerosis of the pancreas--an autonomous
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2.
Yoshida, K., et al., Chronic pancreatitis caused by an autoimmune abnormality.
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1561-8.
3.
Hardacre, J.M., et al., Results of pancreaticoduodenectomy for
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discussion 858-9.
4.
Kim, K.P., et al., Autoimmune chronic pancreatitis. Am J Gastroenterol, 2004.
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Nishimori, I., A. Tamakoshi, and M. Otsuki, Prevalence of autoimmune
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6.
Hamano, H., et al., High serum IgG4 concentrations in patients with sclerosing
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7.
Hirano, K., et al., Pancreatic mass lesions associated with raised concentration of
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Okazaki, K., et al., Autoimmune-related pancreatitis is associated with
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Kamisawa, T., et al., Autoimmune pancreatitis and IgG4-related sclerosing
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Kawaguchi, K., et al., Lymphoplasmacytic sclerosing pancreatitis with
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Park, D.H., M.H. Kim, and S.T. Chari, Recent advances in autoimmune
pancreatitis. Gut, 2009. 58(12): p. 1680-9.
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Zamboni, G., et al., Histopathological features of diagnostic and clinical
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Kamisawa, T., K. Notohara, and T. Shimosegawa, Two clinicopathologic
subtypes of autoimmune pancreatitis: LPSP and IDCP. Gastroenterology,
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Sah, R.P., et al., Differences in clinical profile and relapse rate of type 1 versus
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Shimosegawa, T., et al., International consensus diagnostic criteria for
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Ghazale, A., et al., Value of serum IgG4 in the diagnosis of autoimmune
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Gastroenterol, 2007. 102(8): p. 1646-53.
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Bodily, K.D., et al., Autoimmune pancreatitis: pancreatic and extrapancreatic
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Clinical Images:
Image:1 MRCP images arrow showing sausage like pancreas in delayed
phase:
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Image :2 :
Endoscopic ultrasound images arrows showing (clock- wise) 1) CBD
stricture 2) Dilated CHD , 3) Reactive lymph node 4) Homogenous
pancreatic body.
1)
2)
4)
3)
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Images 3: ERCP arrows showing CBD and MPD strictures pre Figs-1
and 2 ; post stent insertion 3 and 4 (clock-wise):
1) Common Bile duct stricture
3)Stent in Common Bile Duct
2) Main pancreatic duct stricture
4) Stent in Main Pancreatic Duct
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Table 1 Level 1 and level 2 criteria for Type-1 AIP.
Criterion
Level 1
Level 2
Typical: diffuse enlargement with
Indeterminate (including
delayed enhancement (sometimes
atypical): segmental/focal
Parenchymal
associated with rim-like
enlargement with delayed
imaging
enhancement)
enhancement
Long (>1/3 length of the main
pancreatic duct) or multiple
Segmental/focal narrowing
Ductal imaging strictures without marked upstream without marked upstream
(ERP)
Serology
dilatation
dilatation (duct size, <5 mm)
IgG4, >2x_ upper limit of normal
IgG4, 1–2x_upper limit of normal
value
value
a or b
a or b
Other organ
involvement
(OOI)
a. Histology of extrapancreatic
a. Histology of extrapancreatic
organs including endoscopic
organs
biopsy of bile duct
Any three of the following:
Both of the following:
(1) Marked lymphoplasmacytic
(1) Marked lymphoplasmacytic
infiltration with fibrosis and
infiltration with fibrosis without
without granulocytic infiltration
granulocytic infiltration
(2) Storiform fibrosis granulocytic
(2) Abundant (>10 cells/HPF)
infiltration
IgG4-positive cells
(3) Obliterative phlebitis
(4) Abundant (>10 cells/HPF) IgG4positive cells
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Criterion
Level 1
Level 2
b. Physical or radiological
b. Typical radiological evidence
evidence
At least one of the following:
At least one of the following:
(1) Segmental/multiple proximal
(hilar/intrahepatic) or proximal and (1) Symmetrically enlarged
distal bile duct stricture
salivary/lacrimal glands
(2) Radiological evidence of renal
involvement described in
(2) Retroperitoneal fibrosis
association with AIP
LPSP (core biopsy/resection)
LPSP (core biopsy)
At least 3 of the following:
Any 2 of the following:
(1) Periductal lymphoplasmacytic
(1) Periductal
infiltrate without granulocytic
lymphoplasmacytic infiltrate
infiltration
without granulocytic infiltration
(2) Obliterative phlebitis
(2) Obliterative phlebitis
(3) Storiform fibrosis
(3) Storiform fibrosism
Histology of the
pancreas
(4) Abundant (>10 cells/HPF) IgG4- (4) Abundant (>10 cells/HPF)
positive cells
IgG4-positive cells
(Reproduced with permission from 2012 Kamisawa, Tabata, Hara, Kuruma, Chiba,
Kanno, Masamune and Shimosegawa.)
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Table 2:
Diagnosis of definitive and probable Type-1 AIP using international consensus
diagnostic criteria.
Primary
basis for
Diagnosis
diagnosis
Definitive
Type-1 AIP
Imaging evidence
Typical/indetermi
Histology
Imaging
nate
Typical
Collateral evidence
Histologically confirmed
LPSP (level 1 H)
Any non-D level 1/level 2
Two or more from level 1
Indeterminate
Response to
steroid
(+level 2 D*)
Level 1 S/OOI + Rt or level
Indeterminate
1 D + level 2 S/OOI/H + Rt
Probable
Type-1 AIP
*
Indeterminate
Level 2 S/OOI/H + Rt
Level 2 D is counted as level 1 in this setting.
(Reproduced with permission from 2012 Kamisawa, Tabata, Hara, Kuruma, Chiba,
Kanno, Masamune and Shimosegawa.)[25]
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