Non-IBD Disorders of Intestinal Inflammation

Question 1

HSP

Answer 1

• Males > Females
• Peaks 5 years of age
• Late fall-Spring
• Genetic predisposition: HLA B35
• Infection, most often viral or Staphylococcus
• Mediated by IgA deposition in various tissue sites

Question 2

HSP Clinical Manifestations

Answer 2

• Purpura (painless palpable) in dependent areas, non pitting edema
• GI: small bowel, colicky pain, vomiting, bleeding intussusception, PLE, cholecystitis, pancreatitis
• Musculoskeletal: symmetrical arthralgia/arthritis or large joints in legs
• Renal: microscopic hematuria with or without proteinuria, may develop rapidly progressive glomerulonephritis or ESRD.
• Histology: Leukocytoclastic vasculitis affecting capillaries, venules, and arterioles
• IgA and C3 deposits

Question 3

HSP: treatment

Answer 3

• Steroids can be controversial in regards to effectiveness, but it does seem to help the pain. intussusception can lead to severe pain, possibel for moassive GI bleed
  Management=supportive care

Question 4

Bechet’s Syndrome: HLA-B51

Answer 4

Chronic relapsing multisystemic vasculitis
Starts ~2nd-4th decade of life
Increased among silk route population from Asia to Mediterranean
Diagnosis: clinical criteria:
- multiple recurrent painful oral and genital aphthous ulcers
- eyes: uveitis >retinal vasculitis, ocular pain, photophobia, redness, visual blurring and tearing
- vascular: systemic vasculitis (venous >arterial system), affecting all sized vessels
- thrombopblebitis, Budd-Chiari
- joints: nonerosive, nondeforming and monoarticular arthritis
-GI abdominal pain, bleeding, fever, weight loss, diarrhea
- Neuro: acute aseptic meningitis, cranial nerve palsies, seizures

Question 5

HUS: Hemolytic Uremic Syndrome

Answer 5

6 months to 5 years
triggered by intestinal infections: EHEC 0157:H7, immune complexes and drugs
Clinical manifestations: blood diarrhea, hemolytic anemia, thrombocytopenia, acute renal failure
Management: supportive, dialysis as needed, antihypertensive and antiseizure meds as needed. NO antibiotics given for EHEC.

Question 6

Lymphocytic colitis

Answer 6

Chronic, non-bloody diarrhea
Associated with celiac disease, drugs (PPI)
Biopsy diagnosis
Management: Budesonide, 5 ASA, Pepto-bismol