• Chronic granulomatous inflammatory GI disease characterised by transmural granulomatous inflammation
  • May occur in any part of the gut but favours the terminal ileum and proximal colon
  • Unlike UC there are unaffected bits in between active areas – skip lesions
  • Associated with mutations of the NOD2/CARD15 gene
  • Symptoms

    • Diarrhoea, abdo pain and weight loss
    • FTT in children
    • In active disease there can be fever, malaise and anorexia

    Signs

    • Apthous ulcers
    • Abdo tenderness
    • Right iliac fossa mass
    • Perianal abscess/fissure/skin tags
    • Anal/rectal strictures
    • Extraintestinal signs;
      • Clubbing
      • Erythema nodosum
      • Pyoderma gangrenosum
      • Conjunctivis
      • Episcleritis
      • Iritis
      • Large joint arthritis
      • Sacroilitis
      • AS
      • Fatty liver
      • PSC
      • Cholangiocarcinoma
      • Renal stones
      • Osteomalacia
      • Malnutrition
      • Amyloidosis

    Complications

    • Small bowel obstruction
    • Toxic dilation
    • Abscess formation
    • Fistulae
    • Perforation
    • Rectal haemorrhage
    • Colonic carcinoma (rarer than in UC)

    Tests

    • Blood – FRB, ESR, CRP, U&Es, LFT, blood cultures, serum iron, B12, red cell folate in anaemic
    • Markers of activity – reduced Hb, raised ESR, CRP, WCC, reduced albumin
    • Stool culture
    • Barium enema – may show cobble stoning, ‘rose thorn’ ulcers and colon strictures with rectal sparing
    • Colonoscopy

    Management

    • Prednisolone – milder attacks
    • Azothioprine can be useful as a steroid sparing agent
    • Severe attacks;
      • Admit, IV steroids, nil by mouth, IV hydration
      • Hydrocortisone
      • Treat rectal disease with topical steroids
      • Metronidazole
      • Consider blood transfusion
      • If no response consider surgical opinion
        • 50-80% need an operation at some point
        • Indications for surgery are;
          • Failure to respond to medication
          • Intestinal obstruction from strictures
          • Intestinal perforation
          • Local complications e.g. abscess formation, fistulae

    Perianal disease

    • Occurs in 50%
    • MRI and examination under anaesthesia are important part of assessment
    • Treat with oral antibiotics, immunosuppressant therapy +/- infliximab

    Causes of erythema nodosum (painful purple patches on shins)

    • UC
    • Crohn’s
    • Sarcoidosis
    • Drugs
    • Streptococcal infection
    • TB
     

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