Chronic pancreatitis

Topic Completed: 1 August 2012

Minor changes: 22 October 2020

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PubMed Search: Chronic pancreatitis[TI] pancreas[TI] full text[sb]

Deepali Jain, M.D.
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Cite this page: Jain D. Chronic pancreatitis. website. Accessed October 22nd, 2021.
Definition / general
  • Repeated attacks of pancreatic inflammation with loss of pancreatic parenchyma and replacement with fibrosis, variable pain and symptoms of pancreatic insufficiency (malabsorption, diabetes)
  • May simulate or coexist with pancreatic carcinoma
  • Complications: pseudocysts in 10%, also pseudoaneursyms, polyarthropathy, avascular bone necrosis; rarely causes widespread metastatic fat necrosis (from liberation of lipase) affecting legs, mediastinum, pleura, pericardium, bone marrow, liver, skin (erythema nodosum-like lesions), localized portal hypertension due to fibrosis of splenic vein in alcoholic hepatitis (Arch Pathol Lab Med 1997;121:612)
  • Attacks precipitated by alcohol, overeating, opiates, other drugs
  • Biliary disease usually not a factor in chronic pancreatitis
  • Other risk factors: hypercalcemia, hyperparathyroidism, hyperlipoproteinemia, pancreas divisum (seen in 12%), pancreatic neoplasm, cystic fibrosis; cigarette smoking; no known risk factor in 30%
  • Also associated with mumps, polyarteritis nodosa, sarcoidosis, malakoplakia, primary sclerosing cholangitis, HIV (mild changes)
Subtypes of chronic pancreatitis
Familial hereditary pancreatitis:
  • Childhood onset, first decade, increased risk for pancreatic carcinoma
  • Autosomal dominant with mutation at trypsinogen (PRSS1), codon 117 (80% cases) that removes a proteolytic cleavage site, causing persistent trypsin activation
  • Other mutations in cystic fibrosis transmembrane conductance regulator (CFTR) or in the serine protease inhibitor Kazal type 1 (SPINK1)

Paraduodenal or groove pancreatitis:
  • Also called adenomyoma / myoadenomatosis, cystic dystrophy of heterotopic pancreas, pancreatic hamartoma of pancreatic wall or periampullary or periduodenal wall cyst
  • Scar develops between head of pancreas and duodenum
  • Involves an area between the pancreas, common bile duct and duodenum, typically involves the minor papilla
  • Associated with alcohol abuse, tobacco smoking and hypertension
  • Duodenal submucosa and muscularis show a prominent chronic inflammatory cell infiltrate extending to pancreas, myoid proliferation, Brunner gland hyperplasia

Nonalcoholic chronic tropical pancreatitis:
  • Younger individuals, in India, southern Asia, central Africa and Brazil
  • Due to protein calorie malnutrition, dietary cyanogen toxicity, antioxidant deficiency, genetic predisposition (mutations in pancreatic secretory trypsin inhibitor gene)
  • Gross description: shrunken, firm, nodular, gritty pancreas due to calculi present diffusely in dilated ducts
  • Micro description: pancreatic ducts with denuded epithelium, squamous metaplasia, periductal lymphoplasmacytic infiltrate, acinar and islets atrophy, fibrosis

Infectious pancreatitis:
  • In immunocompromised patients
  • Men, 40+, often alcoholics (70%)
  • Clinical triad of steatorrhea, diabetes and pancreatic calcification on radiographic studies requires high degree of suspicion
  • Mildly elevated amylase during attacks
  • CT scan shows calcifications
  • Weight loss, intractable abdominal pain, hypoalbuminemia and associated edema due to pancreatic insufficiency
  • Pancreatic duct drainage, Whipple resection (relieves pain in 50% of patients with pain)
Gross description
  • Hard, shrunken, dilated ducts, visible calcified concretions (protein plugs), pseudocysts common
  • 5% have obstruction due to tumor or stones
Gross images

AFIP images

Whipple resection
specimen due
to chronic

Main pancreatic
duct in head
body region

Microscopic (histologic) description
  • Loss of acini and ductal tissue with relative sparing of islets, irregularly distributed bland periductal fibrosis, variable obstruction of pancreatic ducts of all sizes
  • Chronic inflammation (including mast cells) around lobules and ducts; perineural and intraneural inflammation with hyperplasia of the nerves
  • Dilated ducts with concretions
  • Ductal epithelium is atrophic, hyperplastic or undergoes squamous metaplasia
  • Islets may become sclerotic and disappear
  • Associated with Brunner gland hyperplasia in duodenum
  • May have islet cell proliferation with invasive-like pattern (islet aggregation) in peripancreatic adipose tissue
Microscopic (histologic) images

AFIP images

Focal fibrosis

Advanced chronic pancreatitis

Sclerotic area of pancreas

ducts containing
protein plugs

Ducts are
irregularly sized
but lining lacks
cellular atypia

Ducts have
benign appearing
nuclei, islet is
at bottom

Squamous metaplasia: mucinous epithelial duct cells are partly replaced by squamous epithelium

Positive stains
Differential diagnosis
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