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For further information, see CMDT Part 16-27: Chronic Pancreatitis

Key Features

Essentials of Diagnosis

  • Epigastric pain

  • Steatorrhea

  • Weight loss

  • Abnormal pancreatic imaging

  • A mnemonic for predisposing factors is TIGAR-O

    • Toxic-metabolic

    • Idiopathic

    • Genetic

    • Autoimmune

    • Recurrent and severe acute pancreatitis

    • Obstructive

  • Another multi-risk factor classification system that provides criteria for etiology, clinical and diagnostic stage, and severity is M-ANNHEIM

    • Multiple risk factors

    • Alcohol consumption

    • Nicotine consumption

    • Nutritional factors

    • Hereditary factors

    • Efferent duct factors

    • Immunologic factors

    • Miscellaneous and rare metabolic factors system

General Considerations

  • Occurs most often with alcohol use disorder (45–80% of all cases)

  • Risk of chronic pancreatitis increases with duration and amount of alcohol consumed, but it develops in only 5–10% of heavy drinkers

  • Tobacco smoking is a risk factor for idiopathic chronic pancreatitis and may accelerate progression of alcoholic chronic pancreatitis

  • Pancreatitis develops in about 2% of patients with hyperparathyroidism

  • In tropical Africa and Asia, tropical pancreatitis, related in part to malnutrition, is most common cause of chronic pancreatitis

  • A stricture, stone, or tumor obstructing the pancreas can lead to obstructive chronic pancreatitis

  • Autoimmune pancreatitis is associated with hypergammaglobulinemia and is responsive to corticosteroids

    • Type 1 autoimmune pancreatitis (lymphoplasmacytic sclerosing pancreatitis, or simply "autoimmune pancreatitis") is the pancreatic manifestation of IgG4-related disease and is characterized by

      • Lymphoplasmacytic infiltration and fibrosis on biopsy

      • Associated bile duct strictures

      • Retroperitoneal fibrosis

      • Renal and salivary gland lesions

      • High rate of relapse after treatment

    • Type 2 (idiopathic duct-centric chronic pancreatitis) is characterized by

      • Intense duct-centric lymphoplasmacytic infiltration on biopsy

      • Lack of systemic IgG4 involvement

      • Association with inflammatory bowel disease in 25% of cases

      • Often a tumor-like mass

      • Low rate of relapse after treatment

    • Affected persons are at increased risk for various cancers

  • Between 10% and 30% of cases are idiopathic

  • The pathogenesis of chronic pancreatitis may be explained by the SAPE (sentinel acute pancreatitis event) hypothesis; a first episode of acute pancreatitis initiates an inflammatory process that results in injury-related fibrosis

Demographics

  • Prevalence in the United States is 25–99 per 100,000 population with a peak in persons aged 46–55 years

  • Type 1 typically occurs in a patient over age 60

  • Type 2 typically occurs in a patient aged 40–50 years

  • Genetic factors may predispose to chronic pancreatitis in some cases; for example, mutations of the

    • Cystic fibrosis transmembrane conductance regulator (CFTR) gene

    • Pancreatic secretory trypsin inhibitor gene (PSTI, serine protease inhibitor, SPINK 1)

    • Chymotrypsin-C (CTRC) gene

    • Genes for carboxypeptidase A1 (CPA1) and possibly uridine 5′-diphosphate glucuronosyltransferase (UGT1A7)

Clinical Findings

Symptoms and Signs

  • Persistent or recurrent episodes of epigastric and left upper quadrant pain with referral to the left upper lumbar region are typical

  • Anorexia, nausea, vomiting, constipation, flatulence, and weight loss are common

  • During attacks, tenderness over the ...

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