Painless Tropical Chronic Pancreatitis with Extensive Calcification

Case Report
DOI: 10.17354/cr/2015/158
Painless Tropical Chronic Pancreatitis with Extensive
Calcification
Kandan Balamurugesan, Stalin Viswanathan
Associate Professor, Department of General Medicine, Indira Gandhi Medical College & Research Institute, Puducherry, India
Chronic pancreatitis (CP) is caused by a wide variety of causes such as alcohol, biliary diseases, trauma, infections, autoimmune diseases,
and hereditary disorders. Patients with CP present with disabling abdominal pain, weight loss, exocrine insufficiency, and diabetes and are
at risk for malignant transformation. Pancreatic calcification narrows down the diagnosis of CP. Uncommon causes of calcific pancreatitis
include autoimmune pancreatitis, hereditary pancreatitis, tropical pancreatitis, groove pancreatitis, and pancreatitis associated with cystic
fibrosis, Ascaris, and ectopic pancreatic tissue. Calcific pancreatitis in young diabetics of tropical countries could be either due to alcohol
or tropical pancreatitis. We report a young man with weight loss and steatorrhea, but without abdominal pain whose evaluation led to a
diagnosis of tropical CP and diabetes.
Keywords: Calcific pancreatitis, Chronic pancreatitis, Diabetes, Exocrine dysfunction, Tropical pancreatitis
INTRODUCTION
Chronic pancreatitis (CP) leads to the replacement
of secretory parenchyma of the pancreas by fibrous
tissue and is characterized by disabling pain, diabetes,
and malnutrition.1 The etiology of CP includes toxins
(alcohol, cigarette smoke), infections (HIV, mumps),
drugs (valproate, azathioprine), metabolic abnormalities
(chronic kidney disease, hypercalcemia), genetic
abnormalities (CFTR and SPINK1 mutation), gallstones,
and recurrent attacks of acute pancreatitis.1 In the AsiaPacific region, the etiology of CP consists of alcohol,
idiopathic, tropical, biliary and hereditary causes.2 CP in
the young may arise due to hereditary, infective, toxic,
or idiopathic reasons.
CP is the most common cause of pancreatic calcification.
Pancreatic calcification also arises due to malignancies
such as neuroendocrine tumors, mucinous neoplasms, and
pancreatic adenocarcinoma.3 Uncommon causes of calcific
pancreatitis include autoimmune pancreatitis, hereditary
pancreatitis, tropical pancreatitis, groove pancreatitis,
and pancreatitis associated with cystic fibrosis, Ascaris,
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and ectopic pancreatic tissue.4 In Asia and in the Indian
subcontinent, tropical pancreatitis constitute a sizeable
portion of patients with CP.5 Tropical pancreatitis has
been reported in the south Indian states, Orissa, Sri Lanka,
Indonesia, Malaysia, China and in African countries such
as Uganda and Nigeria.2,6 This entity has also been known
by other names such as chronic calcific pancreatitis and
non-alcoholic tropical pancreatitis. Patients with tropical
CP are generally young and have ketosis-resistant
diabetes; large ductal calculi are observed, more so in the
pancreatic head. When tropical CP is associated with frank
diabetes, it is labeled as fibrocalculous pancreatic diabetes
(FCPD) that is characterized by a triad of abdominal
pain, malabsorption related steatorrhea, and diabetes.7
We report a patient with tropical CP with endocrine
and exocrine insufficiency but without the characteristic
abdominal pain of pancreatitis.
CASE REPORT
This 27-year-old farmer presented to the outpatient
department of our hospital with 3 months’ symptoms of
easy fatigability, lethargy, progressive weight loss (5 kg in
3 months), and frequent passage of bulky oily stools. He
had been treated with iron and vitamin supplements by
a private practitioner and was referred for evaluation of
steatorrhea. He denied history of alcohol consumption, drug
intake, recurrent pain abdomen and other gastrointestinal
and osmotic symptoms. There was no family history of
diabetes. Clinical examination was normal except for
low body mass index (18.4 kg/m 2). His investigations
Corresponding Author:
Stalin Viswanathan, Department of General Medicine, Indira Gandhi Medical College & Research Institute, Pondicherry - 605009, India.
E-mail: [email protected]
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IJSS Case Reports & Reviews | November 2015 | Vol 2 | Issue 6
Balamurugesan and Viswanathan: Tropical Chronic Pancreatitis
included: Random blood sugar 363 mg%, normal renal
and liver function tests, glycosuria without ketonuria and
negative stool examination for ova and cysts. An abdominal
radiograph revealed dense pancreatic calcification that was
confirmed with a computed tomography of the abdomen
(Figure 1a-d). His sugars were controlled with twice daily
subcutaneous premixed insulin. Steatorrhea could not be
proven because of lack of facilities in our institute. He was
initiated on pancreatic enzyme supplementation (thrice
daily with meals) and a fat-restricted diet, with which his
symptoms subsided.
DISCUSSION
CP is caused by a large number of etiological factors.1 Young
individuals are more likely to either have a hereditary,
alcohol-related, cystic fibrosis-related, or pancreas divisum
related pancreatitis. Gallstones, viruses, trauma and drugs
cause CP without calcification. The differential diagnosis of
pancreatic calcification is also broad and include alcohol,
hereditary, infectious, autoimmune diseases, and malignant
causes.3 The pattern of calcification, pancreatic atrophy,
and dilated ducts help in narrowing the diagnosis even
further. Diffuse parenchymal distribution of calcification,
intraductal calcification, and parenchymal atrophy
generally favors a diagnosis of CP.3 The calcification in
alcohol-related pancreatitis is generally speckled and
diffuse with irregular margins. Alcohol still remains
the most common cause of pancreatic calcifications. 8
Conversely, the calculi in tropical CP are large, discrete
and are located in the pancreatic duct causing its dilatation,
especially in the head of the pancreas. Tropical CP is a triad
of abdominal pain, steatorrhea, and diabetes (spectrum of
impaired glucose tolerance to frank diabetes). According
a
b
c
d
Figure 1: (a) Abdominal radiograph shows large dense calculi at level of L1
vertebra, (b) coronal computed tomography (CT) section of pancreas confirms
dense calculi in the head, body and tail of the pancreas, (c) axial section of
CT abdomen shows calculi occupying most of the pancreas, with pancreatic
atrophy, (d) axial section of CT abdomen reveals large intraductal calculi with
filling defect
IJSS Case Reports & Reviews | November 2015 | Vol 2 | Issue 6
to one study, tropical CP was the most common cause of
CP causing diabetes in south India.9 This condition is male
predominant, seen almost exclusively in non-alcoholics
from lower socio-economic groups of tropical countries.7
India and Pakistan have the highest prevalence in Asia.5
Genetic susceptibility (SPINK 1 mutation) leading to loss
of protease inhibition, malnutrition, and dietary toxins
especially from cassava, are some of the etiological factors.
In a study from Bangladesh, patients with tropical CP had
selective preservation of alpha cell function in the pancreas
along with CP.10 Steatorrhea is uncommon, being observed
in ~20% and has been attributed to low-fat diet. They are
not ketosis-prone due to preserved beta cell function and
reduced glucagon reserve.7 Fecal fat estimation followed
by calculation of the coefficient of fat absorption is the gold
standard for steatorrhea, but cumbersome for the patient.11
Hence, less expensive tests such as fecal chymotrypsin,
fecal elastase, and serum trypsin have been used instead of
fecal fat estimation.2 As none of these tests were available,
a therapeutic trial was given in our patient.
CONCLUSION
Diabetes in the young, when associated with pancreatic
calcifications, should make one consider CP of either
tropical, hereditary, or alcohol-related etiologies. Based on
the nature, distribution of calcification and ductal dilatation
tropical CP with diabetes (FCPD) was diagnosed. Unusual
in this patient was absence of episodic attacks of abdominal
pain and the immensely large calculi taking the shape of
the entire pancreas. Even without chronic pain, imaging of
the pancreas must be considered in young diabetics to rule
out tropical CP related diabetes
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Balamurugesan and Viswanathan: Tropical Chronic Pancreatitis
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How to cite this article: Balamurugesan K, Viswanathan S. Painless Tropical
Chronic Pancreatitis with Extensive Calcification. IJSS Case Reports &
Reviews 2015;2(6):24-26.
Source of Support: Nil, Conflict of Interest: None declared.
IJSS Case Reports & Reviews | November 2015 | Vol 2 | Issue 6