Idiopathic Duct Centric Pancreatitis (Autoimmune Type 2)
Definition
- Mass forming infiltrate of lymphocytes and plasma cells with neutrophilic destruction of ducts
Alternate/Historical Names
- Autoimmune pancreatitis with granulocytic epithelial lesions (GEL)
- Duct-centric pancreatitis
- Sclerosing pancreatitis
Note
- Autoimmune pancreatitis has recently been split into two types
- Lymphoplasmacytic sclerosing pancreatitis (autoimmune type 1)
- Idiopathic duct centric pancreatitis
(covered here)
- Neutrophilic infiltration and destruction of ducts in addition to lymphoplasmacytic infiltrate
Diagnostic Criteria
- Dense periductal infiltrate
- Centered on medium sized pancreatic ducts and intrapancreatic common bile duct
- Predominantly lymphoplasmacytic
- Predominantly T cells
- Plasma cells polyclonal
- May include eosinophils and germinal centers
- Neutrophils infiltrate ulcerate and destroy ductal epithelium
- Termed "granulocytic epithelial lesions" (GEL)
- Lobules variably involved
- Predominantly lymphocytes and plasma cells but may include neutrophils
- May form neutrophilic abcesses
- Predominantly lymphocytes and plasma cells but may include neutrophils
- Moderate fibrosis centered on ducts
- Less prominent than in lymphoplasmacytic sclerosing pancreatitis
- No obliterative venulitis or arteritis
- Non-obliterative venulitis may be seen
- No increase in IgG4 expression
- Immunoperoxidase staining reveals only rare IgG4 positive plasma cells
- Serum elevation of IgG4 is rare
- May form a mass (80%), usually in the head, or diffuse enlargement
- Frequently patchy in distribution
- May be associated with inflammatory bowel disease
- Extra-pancreatic involvement by lymphoplasmacytic sclerosing lesions not seen
- Not associated with:
- Duct dilation
- Lithiasis
- Pseudocyst formation
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
- Age mean 50 years
- Male and female incidence equal
- No association with ethanol abuse
- Frequently presents with abdominal pain
- Obstructive jaundice is rare
- Usually responds to steroids
- Relapse is rare
- May be associated with inflammatory bowel disease (16%)
- No association with other autoimmune or fibroblastic processes
- Appears to be rare in Japan
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 |
| 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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