Surgical Pathology Criteria

Idiopathic Duct Centric Pancreatitis (Autoimmune Type 2)

Definition

Alternate/Historical Names

Note

Diagnostic Criteria

Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting/updates: 1/19/08, 12/31/08, 1/20/09, 10/31/10, 2/16/11

Clinical

Differential Diagnosis

Lymphoplasmacytic Sclerosing Pancreatitis (Autoimmune Type 1) Idiopathic Duct Centric Pancreatitis (Autoimmune Type 2)
No neutrophilic invasion and destruction of ductal epithelium Neutrophilic invasion and destruction of ductal epithelium
Prominent sclerosis Less prominent sclerosis
Frequent obliterative venulitis Lacks obliterative venous damage
IgG4 plasma cells >20-50/HPF IgG4 plasma cells infrequent (<10/HPF)
Serum IgG4 usually elevated Serum IgG4 rarely elevated
Frequently associated with extrapancreatic inflammatory lesions Associated only with inflammatory bowel disease
Responds to steroids but frequently relapses Responds to steroids but rarely relapses
Rare under age 50 Average age 50

Idiopathic Duct Centric Pancreatitis (Autoimmune Type 2) Ductal Adenocarcinoma of the Pancreas
Prominent lymphoplasmacytic infiltrate Inflammation not a prominent part of mass
No infiltrating pattern of ducts Infiltrating pattern prominent
More of a gross problem than a microscopic problem

Idiopathic Duct Centric Pancreatitis (Autoimmune Type 2) Chronic Pancreatitis, NOS
Overall pattern is mass lesion composed of lymphocytes and plasma cells, no duct dilation Overall pattern is acinar atrophy with dilated ducts
Both chronic and acute infiltrate centered on ducts Lacks substantial focused infiltrate

Idiopathic Duct Centric Pancreatitis (Autoimmune Type 2) Alcoholic Pancreatitis
Overall pattern is mass lesion composed of lymphocytes and plasma cells, no duct dilation Pattern is basically atrophic with duct dilation
Stones, fat necrosis and pseudocysts not seen Stones, fat necrosis and pseudocysts common

Idiopathic Duct Centric Pancreatitis (Autoimmune Type 2) Primary Sclerosing Cholangitis
Frequently forms a pancreatic mass No pancreatic mass
No involvement of intrahepatic ducts Frequent involvement of intrahepatic ducts
pANCA negative pANCA positive in most cases

Bibliography

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