Crohn Disease

BASICS

DESCRIPTION

  • A chronic, progressive inflammatory GI tract disorder, most commonly involving the terminal ileum (80%)
  • Hallmark features of Crohn Disease (CD):
    • Transmural inflammation that can result in fibrotic strictures, fistulas, fissures, or abscesses; noncaseating granulomas (30%); skip lesions: diseased mucosa interspersed with normal mucosa; can be continuous, mimicking ulcerative colitis (UC); rectal sparing; diverse presentations: ileitis (1/3), ileocolitis (1/3); isolated colitis (1/3)

EPIDEMIOLOGY

Incidence

3 to 20 cases per 100,000 person-years in North America; incidence is rising globally. Women are slightly more affected than men; increased incidence in northern climates

Prevalence

247 cases per 100,000 persons

ETIOLOGY AND PATHOPHYSIOLOGY

  • Genetics, environmental triggers, commensal microbial antigens, and immunologic abnormalities result in activation of inflammatory cells and subsequent tissue injury.

Genetics

  • 15% of CD patients have a first-degree relative with inflammatory bowel disease (IBD); first-degree relative of an IBD patient has 3- to 30-fold increased risk of developing IBD by age 28 years.
  • Associated genetic syndromes: Turner and Hermansky-Pudlak syndromes, glycogen storage disease type 1b

RISK FACTORS

Environmental factors:

  • Cigarette smoking doubles the risk of CD; tobacco cessation reduces flares and relapses.
  • Dietary factors: higher incidence if diet high in refined sugars, animal fat or protein, processed or ultraprocessed foods

COMMONLY ASSOCIATED CONDITIONS

  • Extraintestinal manifestations
    • Arthritis (20%): seronegative, small and large joints (ankylosing spondylitis [AS] or sacroiliitis [SI], associated with HLA-B27); skin disorders (10%): erythema nodosum, pyoderma gangrenosum, psoriasis; ocular disease (5%): uveitis (associated with HLA-B27), iritis, episcleritis
    • Kidney stones: calcium oxalate stones (from steatorrhea and diarrhea) or uric acid stones (from dehydration and metabolic acidosis); osteopenia and osteoporosis; hypocalcemia; hypercoagulability: venous thromboembolism prophylaxis essential in hospitalized patients; gallstones: cholesterol stones resulting from impaired bile acid reabsorption; primary sclerosing cholangitis (PSC) (5%)
  • Conditions that parallel disease activity
    • Peripheral arthropathy (not SI and AS); episcleritis (not uveitis); oral aphthous ulcers and erythema nodosum

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