Celiac Sprue Disease: Overview and Insights

Definition

• Celiac Disease: A chronic intolerance to gluten causing intestinal injury in genetically predisposed individuals. Also known as Nontropical Sprue or Gluten-Sensitive Enteropathy.

Key Facts

• Prevalence: Affects ~1% of the U.S. population; common in Ireland and Northern Europe but rare in Africa, China, and Japan.
• Age Groups: Peaks at age 2 and again in the 3rd to 4th decades.
• Symptoms:
  • Malabsorption and steatorrhea.
  • Abdominal distension, flatulence, diarrhea.
  • Weight loss, anemia, glossitis.

Imaging Findings

1. CT Findings:

   • Thickened or thinned small bowel wall.
   • Submucosal edema or fat, hyperenhancement of mucosa.
   • Reversal of jejunoileal fold patterns (atrophied jejunal, thickened ileal folds).
   • Small bowel intussusception (non-obstructing).
   • Mesenteric lymphadenopathy (cavitated nodes in severe cases).

2. Barium Studies:

   • Dilated jejunum (>3 cm).
   • Flocculation and segmentation of barium.
   • Jejunal fold atrophy; jejunization of ileum.

3. Ultrasound:

   • Enlarged mesenteric lymph nodes.
   • Increased gallbladder volume (occasionally).

4. Best Imaging Tool:

   • CT Enterography for fold pattern reversals and multifocal intussusceptions.

Differential Diagnosis

• Whipple Disease: Lymphadenopathy and mesenteric thickening.
• Crohn Disease: Involvement of distal ileum and colon; no malabsorption pattern.
• Opportunistic Infections: Common in immunocompromised patients (e.g., AIDS-related enteritis).
• Ischemic Enteritis: Mucosal hyperenhancement with submucosal edema or gas.

Pathology

• Etiology:
  • T-cell-mediated autoimmune response to gluten.
  • Genetic predisposition linked to HLA-DQ2 and HLA-DQ8.
• Microscopic Features:
  • Villous atrophy, crypt hyperplasia, and increased cellular infiltrate.

Clinical Issues

• Diagnostic Tools:
  • Duodenojejunal mucosal biopsy.
  • Antibody tests: IgG antigliadin, IgA antiendomysial.
  • Response to gluten-free diet.
• Complications:
  • Refractory disease with enteritis unresponsive to diet.
  • Increased risks of T-cell lymphoma, adenocarcinoma, and metabolic bone disease.

Management

• Celiac Disease: Lifelong adherence to a gluten-free diet.
• Tropical Sprue: Antibiotics (e.g., tetracycline) and folates.