Lymphoplasmacytic Sclerosing Pancreatitis (Autoimmune Type 1)
Definition
- Mass forming, duct centered infiltrate of lymphocytes and plasma cells
Alternate/Historical Names
- Autoimmune pancreatitis
- Duct-centric pancreatitis
- IgG4 related pancreatitis
- Sclerosing pancreatitis
Note
- Autoimmune pancreatitis has recently been split into two types
- Lymphoplasmacytic sclerosing pancreatitis (covered here)
- Idiopathic duct centric pancreatitis (autoimmune type 2)
- 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
- Ductal epithelium preserved
- Predominantly lymphoplasmacytic
- Predominantly T cells
- Plasma cells polyclonal
- May include eosinophils and germinal centers
- Neutrophils are rare
- Lobules frequently involved
- Results in lobular atrophy
- Infiltrate can extend into peripancreatic fat
- Centered on medium sized pancreatic ducts and intrapancreatic common bile duct
- Storiform, swirling fibrosis centered on ducts
- May have onion-skin pattern
- Frequently causes duct stenosis
- Frequent obliterative venulitis
- Lymphoplasmacytic infiltrate in vein walls
- Frequent lumenal obliteration
- Infrequent arteritis
- Increased IgG4 expression
- Immunoperoxidase staining with >30 IgG4 positive plasma cells / HPF is strongly suggestive of the diagnosis
- Some use a cutoff of >10
- Serum elevation of IgG4 is suggestive of the diagnosis
- Occasional elevations with other disorders
- Increased IgG4 positive plasma cells in ampullary biopsies has been reported (Sepehr)
- Proposed as a diagnostic aid
- Immunoperoxidase staining with >30 IgG4 positive plasma cells / HPF is strongly suggestive of the diagnosis
- May form a mass (80%), usually in the head, or diffuse enlargement
- Frequently patchy in distribution
- Extra-pancreatic involvement frequently present (see Clinical at left sidebar)
- Histologically similar lesions
- 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 range 17-80 years, mean 60-65 years
- 85% >50
- Male predominance, reported 2:1 to 10:1
- No association with ethanol abuse
- Frequently presents with painless obstructive jaundice
- Accounts for about 5% of chronic pancreatitis (in Japan)
- Usually responds to steroids
- Frequent relapse on withdrawal
- About 40% associated with other autoimmune or fibroblastic processes
- Mediastinal lymphadenopathy
- Intra and extrahepatic sclerosing cholangitis
- Lymphocytic thyroiditis
- Mediastinal and retroperitoneal fibrosis
- Chronic lacrymadenitis and sialadenitis
- Interstitial nephritis
- Pulmonary infiltrates
- Gastritis
- No association with inflammatory bowel disease
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 |
| Lymphoplasmacytic Sclerosing Pancreatitis (Autoimmune Type 1) | Ductal Adenocarcinoma of the Pancreas |
|---|---|
| Prominent lymphoplasmacytic infiltrate | Inflammation not a prominent part of mass |
| No infiltrative pattern of ducts | Infiltrative pattern prominent |
| Elevated serum IgG4 | No elevation of IgG4 |
| Immunohistologic demonstration of >30 IgG4 plasma cells per hpf | Few IgG4 plasma cells |
| Lymphoplasmacytic Sclerosing Pancreatitis (Autoimmune Type 1) | 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 |
| Infiltrate frequently centered on ducts | Lacks substantial focused infiltrate |
| Frequent obliterative venulitis | Lacks venous damage |
| IgG4 plasma cells >20-50/HPF | IgG4 plasma cells infrequent (<10/HPF) |
| Lymphoplasmacytic Sclerosing Pancreatitis (Autoimmune Type 1) | Primary Sclerosing Cholangitis |
|---|---|
| Overall pattern is mass lesion composed of lymphocytes and plasma cells, no duct dilation | Pattern is multifocal nodules of inflammation involving bile duct |
| Frequent obliterative venulitis | Lacks venous damage |
| IgG4 plasma cells >20-50/HPF | IgG4 plasma cells infrequent (<10/HPF) |
| Lymphoplasmacytic Sclerosing Pancreatitis (Autoimmune Type 1) | Alcoholic Pancreatitis |
|---|---|
| Overall pattern is mass lesion composed of lymphocytes and plasma cells, no duct dilation | Pattern is basically atrophic with duct dilation |
| Frequent obliterative venulitis | Lacks venous damage |
| Stones, fat necrosis and pseudocysts not seen | Stones, fat necrosis and pseudocysts common |
| IgG4 plasma cells >20-50/HPF | IgG4 plasma cells infrequent (<10/HPF) |
Bibliography
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