Lymphoplasmacytic Sclerosing Pancreatitis (Autoimmune Type 1)
Clinical
- Age range 17-80 years, mean 60-65 years
- 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
