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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

General Considerations

  • Occurs most often with alcoholism (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 (autoimmune pancreatitis) is the pancreatic manifestation of IgG4-related disease and is characterized by

      • Lymphoplasmacytic sclerosing pancreatitis 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

      • Duct centric pancreatitis 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 fibrosis

Demographics

  • 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)

    • Gene for uridine 5'-diphosphate glucuronosyltransferase (possibly)

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 pancreas, mild muscle guarding, and ileus may be noted

  • Attacks may last only a few hours or as long as 2 weeks; pain may eventually be almost continuous

  • Steatorrhea (as indicated by bulky, foul, fatty stools) may occur late in the course

Differential Diagnosis

  • Cholelithiasis

  • Diabetes mellitus

  • Malabsorption due to other causes

  • Intractable duodenal ulcer

  • Pancreatic cancer

  • Irritable bowel syndrome

Diagnosis

Laboratory Tests

  • Serum amylase and lipase may be elevated during acute attacks; normal values do not exclude the diagnosis

  • Serum alkaline phosphatase and bilirubin may be elevated owing to compression of the bile duct

  • Glycosuria may be present

  • Excess fecal ...

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