Chronic pancreatitis classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Iqra Qamar M.D.[2]

Overview

Chronic pancreatitis may be divided based upon underlying morphology into large-duct type or small-duct type with or without calcification. The classification systems that have been used for chronic pancreatitis include Marseille, Marseille-Rome system, Cambridge system, TIGAR-O system, ABC grading system and Manchester system.

Classification

Classification based upon morphology

Chronic pancreatitis may be divided based on underlying morphology into:

• Large-duct type
• Small-duct type
  • With calcification
  • Without calcification

Classification system for chronic pancreatitis	Year	Key features
Clinical description	1946	Description of the clinical presentation of chronic pancreatitis and its association with increased alcohol consumption
Marseille	1963	Description of morphologic characteristics and etiological factors of the disease No discussion of the correlation between anatomic and functional changes No categorization according to disease severity or clinical presentation No inclusion of pancreatic imaging findings
Marseille	1984	Further description and subclassification of morphological changes “Obstructive chronic pancreatitis” listed as distinct form No discussion of the correlation between anatomic and functional changes No categorization according to disease severity or clinical presentation No inclusion of pancreatic imaging findings
Marseille-Rome	1988	Description of “chronic calcifying” and “chronic inflammatory” pancreatitis as distinct forms Description of etiological factors No further elaboration of clinical, functional or imaging criteria
Cambridge	1984	Classification of disease severity based on pancreatic imaging criteria (Ultrasound, CT scanning, endoscopic retrograde cholangiopancreatography) Further discussion of etiological factors, pancreatic function, and testing for pancreatic insufficiency Morphologic characteristics not clearly defined
Clinical stages	1994	Detailed subclassification of chronic pancreatitis with correlation of etiological factors with different morphological forms of the disease Differentiation of clinical stages of the disease Linkage of pancreatic imaging findings and functional testing with stages of the disease
Japan Pancreas Society	1997	Description of clinical presentation and classification of disease in “definite” and “probable” chronic pancreatitis according to imaging findings, functional testing, and histological examination
Zürich Workshop	1997	Description of clinical presentation and classification of disease in “definite” and “probable” chronic pancreatitis according to imaging findings, functional testing, and histological examination
TIGAR-O	2001	Detailed categorization of etiological risk factors
ABC grading system	2002	Disease grading according to clinical criteria, but limited separation of different disease severities Not all clinical presentations can be categorized
Manchester system	2006	Disease grading according to clinical criteria, but limited separation of different disease severities Not all clinical presentations can be categorized

Classification of pancreatitis based upon area of involvement and etiology

There are various forms of chronic pancreatitis based upon the area of involvement and etiology are as follows:

Groove pancreatitis:

Groove pancreatitis is a form of segmental pancreatitis that involves confinement of the inflammation process to the groove between the duodenum, common bile duct, and head of the pancreas without any involvement of the head of pancreas.

Hereditary pancreatitis:

• Hereditary pancreatitis is a subtype of chronic pancreatitis.
• It is an autosomal dominant inheritance with 80% of penetrance rate.
• It usually involves mutation in cationic trypsinogen gene (PRSS1) resulting in  loss of autoregulation of activated trypsin.
• Symptoms usually develop <20 year of age and often <5yr age.
• It is associated with an increased risk of pancreatic adenocarcinoma.
• The management is similar to the other causes of chronic pancreatitis.

Autoimmune pancreatitis (AIP):

• Autoimmune pancreatitis may be seen on the continuum of IgG4 related sclerosing diseases.
• It usually presents as obstructive jaundice or acute recurrent pancreatitis.
• The underlying pathology involves  bile duct or pancreatic duct compression due to pancreatic swelling and ductal strictures leading to obstructive jaundice or acute recurrent pancreatitis presentation.
• Elevated immunoglobulin IgG4 levels are usually seen on lab analysis.
• Imaging findings include:
  • Focal or diffuse pancreatic enlargement
  • Narrowed pancreatic duct

• Corticosteroid therapy results in rapid and marked improvement in clinical and radiographic features.

Tropical pancreatitis:

• Tropical pancreatitis is one of the most common causes of chronic pancreatitis in tropical areas including south India.
• It was thought to be caused by cassava fruit but no longer associated with it and has no clear etiology.
• It usually affects children leading to early adulthood death due to endocrine and exocrine dysfunction.
• Serine protease inhibitor SPINK1 mutations are identified in some of the patients.

Idiopathic pancreatitis:

• Idiopathic pancreatitis usually includes the non-alcohol induced cases of chronic pancreatitis.
• It usually accounts for 30% of cases of pancreatitis.
• It can be divided into early onset and late-onset forms.
• Contributing factors towards idiopathic pancreatitis may include:
  • Concealed alcohol intake
  • Hypersensitivity to alcohol
  • Unreported pancreatic trauma
  • Mutations in the cationic trypsinogen gene and the cystic fibrosis gene