Pancreatitis, Chronic

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 ...