Lymphoplasmacytic sclerosing (autoimmune) pancreatitis
David S Klimstra1, N Volkan Adsay
1Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA. klimstrd@mskcc.org
Seminars in Diagnostic Pathology
|November 9, 2005
Summary
Lymphoplasmacytic sclerosing pancreatitis (LPSP), a distinct form of autoimmune pancreatitis, affects middle-aged individuals without typical risk factors. Early diagnosis and steroid therapy offer an excellent prognosis for this condition.
Area of Science:
- Gastroenterology
- Pathology
- Immunology
Background:
- Lymphoplasmacytic sclerosing pancreatitis (LPSP), also known as autoimmune pancreatitis, is increasingly recognized.
- It affects middle-aged patients lacking typical chronic pancreatitis risk factors like alcohol abuse.
- LPSP can mimic pancreatic cancer clinically and radiographically, often presenting with pancreatic masses and bile duct strictures.
Purpose of the Study:
- To review the defining clinical and pathologic features of LPSP.
- To discuss ongoing efforts in understanding its pathogenesis.
- To highlight diagnostic and therapeutic considerations.
Main Methods:
- Review of clinical presentations and radiographic findings.
- Pathologic examination of resected LPSP cases.
- Analysis of serum IgG4 levels as a diagnostic marker.
Main Results:
- LPSP cases show dense periductal lymphoplasmacytic inflammation, fibrosis, and obliterative venulitis.
- Elevated serum IgG4 levels aid in differentiating LPSP from pancreatic carcinoma.
- Reactive fibroinflammatory pseudotumors can occur, extending beyond the pancreas.
Conclusions:
- LPSP is a distinct entity with a generally excellent prognosis.
- Steroid therapy is an effective treatment option.
- Further research is needed for improved preoperative diagnostics and understanding disease variants.
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