HEMOLYTIC UREMIC SYNDROME COLITIS
 
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HEMOLYTIC UREMIC SYNDROME COLITIS COLITIS

 

DEFINITION:

A systemic disease (vasculitis) involving the colon and which may begin as an intestinal inflammatory disorder.

EPIDEMIOLOGY:

  • see HEMOLYTIC UREMIC SYNDROME COLITIS (HUS)

PATHOGENESIS:

1. Background

  • in HUS, an inherited or acquired disturbance in prostacyclin metabolism (prostacyclin is needed for endothelial cell repair) causes endothelial injury and subsequent generalized vasculitis with systemic manifestations

2. Etiology

  • the acquired disturbances in prostacyclin metabolism associated with HUS often arise from an infectious etiology and in particular:

1. Shigella Dysentery

2. E. Coli Colitis

  • verotoxin-producing strains (0157:H7, 026, 0111, 0121, 0145)

3. Campylobacter Enterocolitis

  • the shigatoxin released by Shigella and the Enterohemorrhagic and Enteroinvasive strains of E. coli eventually damages endothelial cells causing:
    • release vWF multimers from damaged endothelial cells capable of aggregating platelets (elevated vWF multimers and antigen titres may be present in HUS)
    • decrease prostacyclin synthesis by endothelial cells with a subsequent decreased endothelial repair mechanism - both mechanisms initiate an intravascular coagulopathy
    • -> HUS
  • an elevated inflammatory response in the colon related to an increased absorption of shigatoxin may increase the risk of complications outside of the GI tract
  • there may be a correlation between the severity of the GI prodrome in HUS (severe colitis and rectal prolapse) and:
    • extraintestinal disease
      • seizures, edema, severe thrombocytopenia, prolonged anuria
    • long-term recovery of renal function

CLINICAL FEATURES:

1. Gastrointestinal Prodrome

  • diarrhea (watery -> grossly bloody diarrhea)
  • mild gastroenteritis to severe colitis
  • rectal prolapse

2. Extraintestinal Manifestations (HUS)

1. Microangiopathic

  • hemolytic anemia
  • thrombocytopenia

2. Renal

  • hypertension
  • edema
  • acute renal failure

3. Neurologic

  • seizures

INVESTIGATIONS:

1. Colonoscopy

  • inflammatory changes in the colon

2. Serum

  • dehydration, electrolyte and metabolic disturbances
  • hemolytic anemia, thrombocytopenia
  • acute renal failure

3. Imaging Studies

1. Barium Enema

  • may initially show spasm with transient early filling defects but may progress to stenosis

MANAGEMENT:

1. Supportive

  • oral rehydration fluid (ORF) or intravenous rehydration

 

 

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