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Journal of Liver and Clinical Research

Case Report

Difficult to Diagnose Case of

Primary Sclerosing Cholangitis in
Young Woman
Shalikiani Nino*, Bakulin Igor and Kharlamenkov Evgeny

*Corresponding author
Shalikiani Nino, Moscow Clinical Scientific Center, 86,
Shosse Entuziastov, Moscow, 111123, Russian Federation;
Russia, Tel: 7929 669 04 32; Email:
Submitted: 08 October 2015
Accepted: 23 November 2015
Published: 24 November 2015
ISSN: 2379-0830
Copyright
2015 Nino et al.

Department of Hepatology, Moscow Clinical Scientific Center, Russia

OPEN ACCESS

Abstract

Keywords

Primary sclerosing cholangitis (PSC) is a chronic liver disease leading to strictures

and dilations of the intra- and extrahepatic bile ducts. PSC can eventually progress
to liver cirrhosis and cholangiocarcinoma. Liver function tests reveal cholestasis,
predominantly on behalf of alkaline phosphatase (ALP) elevation. ERC and MRC are
diagnostic modalities of choice. Liver biopsy is not necessary to confirm the large bile
ducts PSC if a cholangiogram is normal. In case of small bile ducts PSC, liver biopsy
allows to exclude the overlap syndromes.

Cholangitis
Cholestatic liver
Liver function tests

This article represents a case of a patient who presented with fatigue, right
upper quadrant discomfort, mildly elevated ALP and high level of aminotransferases.
Characteristic pattern of radiological changes of PSC was not identified, and the
patient was treated with corticosteroids. Later the cholestatic pattern of the biochemical
profile and dilated bile ducts in the absence of biliary obstruction raised the suspicion
of PSC again. We repeatedly used all diagnostic tools including the liver biopsy. In 4
years the radiological and morphological picture had significantly changed, and PSC
was established.

INTRODUCTION
PSC is a chronic cholestatic liver disease characterized by
inflammation and fibroses of both the intra- and extra hepatic bile
ducts, developing into their multifocal strictures and dilations. Its
prevalence is up to 16 cases per 100,000 persons, and incidence
has increased over the last decade with more than 35% [1]. The
exact etiological factor of the disease remains unclear. Infectious,
immunologic and especially genetic factors are being lately
investigated as potential contributors to the pathogenesis and
etiology [2]. PSC is strongly associated with IBD, mostly ulcerative
colitis (about 80% of cases) [3]. The serious, life threatening
complications include liver cirrhosis and cholangiocarcinoma.

Liver function tests indicate cholestasis: elevated alkaline

phosphatase (3-5 times reference range) is a dominant feature of
the profile, although normal values do not exclude the diagnosis
of PSC. Serum aminotransferase (2-3 upper times upper
limits of normal) and bilirubin levels (mainly the conjugated
component) might rise, but not markedly so. IgG levels might be
slightly elevated in more than half of patients [4]. A number of
serum autoantibodies (perinuclrear antineutrophil cytoplasmic
antibodies (p-ANCA); anticardiolipin (aCL) antibodies and
antinuclear antibodies) reveal an altered immune state, but their
prevalence rates and titers are low. The endoscopic retrograde
cholangiopancreatography (ERCP) is regarded as the criterion
standard for establishing a diagnosis of PSC; if it is unsuccessful,

transhepatic cholangiography may be considered. Magnetic

resonance cholangiopancreatography (MRCP-sensitivity 86%,
specificity 94%) is a preferred diagnostic modality [5], as it
is non-invasive and potential complications of ERCP such as
bacterial cholangitis and pancreatitis can be avoided.

The most common histopathologic liver change is a periductal

concentric fibroses known as onion skin fibroses, but the finding
is quite infrequent and is not an obligatory diagnostic modality in
case of abnormal cholangiogram [6].
No pharmacologic treatment has proven effectiveness in PSC.
Ursodeoxycholic acid (UDCA) might improve the biochemical test
profile in some cases and in complex with endoscopic dilation,
might increase the survival rate.

CASE PRESENTATION

A 31 old female (Mrs. S.) was referred to our clinical center in

June 2015. She reported fatigue, chills and right upper quadrant
discomfort. The patient denied abdominal pain, change in bowel
patterns, weight loss, fever or jaundice.

Mrs. S. worked as a teacher in the primary school, married

with two children. She did not smoke, denied alcohol use, and
consumed two or more caffeinated beverages every day. Her
past medical history consisted of vitiligo and erosive gastritis.
There was no family history of cancer or other significant

Cite this article: Nino S, Igor B, Evgeny K (2015) Difficult to Diagnose Case of Primary Sclerosing Cholangitis in Young Woman. J Liver Clin Res 2(3): 1019.

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Nino et al. (2015)

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gastrointestinal diseases. She did not have known allergies.
She had not left the country for the past year and had not got
antibiotics or any other medications other than UDCA.

Her clinical problems started in 2011. She presented to the

outpatient clinic with a slight pruritus and right upper quadrant
discomfort. Clinical examination revealed ALP elevation and ALT
AST 10 times normal. All auto antibodies, including IgG4, were
negative. No pathology was found on ultrasound. The patient
refused to make liver biopsy. The condition was treated as an
autoimmune hepatitis; corticosteroids were prescribed. Pruritus
and laboratory abnormalities regressed. She did not visit the
clinic during the next year. A year later (2013) an endoscopic
ultrasound revealed signs of the biliary hypertension and bile
duct strictures. ALP was elevated and aminotransferases were
up to 5 times normal again. Firstly MRCP and then ERCP were
performed. The visualization of bile ducts was less than optimal.
Liver biopsy was performed. Based on morphology, biliary
papilomatosis was diagnosed (Figure 1).
Corticosteroids were weaned off. The patient was
recommended to go on treatment with UDCA. On examination
in our clinic Mrs.S. was well oriented and not distressed. Vital
signs were as follows: temperature of 36.5C, respirations of 17,
heart rate of 80. The chest was clear. Cardiac rhythm was regular,
no murmurs. The abdomen was soft, non-tender, no masses,
hepatomegaly or splenomegaly was palpated. Rectal exam: stool
heme negative, no anal or perianal lesions. Skin exam was benign:
no rash, jaundice or edema.
Full blood count and biochemical profile was normal except
the elevation of ALP - 268, aspartate aminotransferase (AST)
- 86, alanine aminotransferase (ALT) - 144. Anti-neutrophil
cytoplasmic, antinuclear, anti-smooth muscle, anti-endothelial,
anti-cardiolipin antibodies were negative.

Large bile duct wall thickening was identified on abdominal

ultrasound. The liver and spleen size was normal, no ascetic
fluid found. Recent MRCP, in contrast to the previous study, was
consistent with the diagnosis of PSC. Colonoscopy was normal.
Carbohydrate antigen 19-9 was not elevated. Transcutaneous
liver biopsy was performed. Periductal fibroses and peribiliary
gland hyperplasia were identified (Figure 2). So, PSC was
diagnosed.

The patient was recommended UDCA 750 mg/day. A month

Figure 2 Onion skin fibroses.

later the liver function tests were normal. The patient is being
followed up.

DISCUSSION

There were several unusual points in our case. The first

one was the epidemiological point, as in contrast to our patient,
typically PSC is diagnosed in middle-aged male patients with IBD
presenting with abnormalities in biochemical profile [3,6].

Cholestatic liver diseases of unknown origin are frequently

seen in clinical practice of gastroenterologists and there are
number of entities, which have to be ruled out, including skeletal
diseases. PSC is always important to consider as the potential
cause. The initial presentation was quite unspecific in our case.
The patient was treated as having an autoimmune hepatitis.
Later the cholestatic pattern of the biochemical profile and
dilated bile ducts in the absence of biliary obstruction raised the
suspicion of PSC. ERC and MRC are diagnostic imaging modalities
of choice with a similar accuracy [6]. Although ERC is a gold
standard, MRC is preferred as it lacks radiologic exposure and
potential complications. In our case the characteristic pattern of
radiological changes - annular strictures and bile duct dilations
were not identified during the first referral. Based on results of
the liver biopsy, biliary papilomatosis was suspected.

Later we repeatedly used all diagnostic tools again, as the

case was still confounding. So, we faced the cholestatic liver
function profile, mildly elevated transaminases, autoantibodies
negative and nonspecific radiological and morphological changes
on previous examination. Liver biopsy is not usually compulsory;
it is used when the bile ducts are not finely visualized, or if an
overlap syndrome with autoimmune hepatitis or primary biliary
cirrhosis is suspected.In our case the morphological study had
to be done twice because of unreliable imaging study results. We
found that during 4 years the radiological and morphological
picture had significantly changed, and PSC was established.
Treatment options for PSC are controversial. The benefits of
using hydrophilic UDCA have been debated for years. Although
American Association for the Study of Liver Diseases (AASLD)
does not recommend UDCA in PSC patients, due the European
guidelines UDCA (1520 mg/kg BW) can be used. In our case
preliminary results of using UDCA turned to be positive [7].

FOLLOW-UP
Figure 1 Epithelium hyperplasia of the medium size bile ducts.
J Liver Clin Res 2(3): 1019 (2015)

PSC patients with high levels of carbohydrate antigen 19-

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9, even in the absence of cholangiocarcinoma, more rapidly
progress to advanced liver disease and have worse survival rates
without transplantation compared to those with a normal CA 19-9
value. Although our patient has not have elevated CA 19-9, she
still needs to be closely followed up, as the cumulative incidence
of cholangiocarcinoma among PSC patients is obviously higher,
being more than 10% [8,9]. And besides, CA 19-9 is not sensitive
in early stages of cholangiocarcinoma.
The mean time from the diagnosis of PSC to fatal complications
if not having the liver transplant is 12-18 years. So, in future as
the disease progresses, the only curative option for the patient
will be the liver transplantation.

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Cite this article

Nino S, Igor B, Evgeny K (2015) Difficult to Diagnose Case of Primary Sclerosing Cholangitis in Young Woman. J Liver Clin Res 2(3): 1019.

J Liver Clin Res 2(3): 1019 (2015)

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