Celiac Crisis

Pathophysiology and Clinical Profile

Rare, dramatic, acute presentation of celiac disease.
Occurs predominantly in young children prior to formal diagnosis.
Autoimmune enteropathy triggered by gluten ingestion in genetically susceptible individuals expressing HLA-DQ2/DQ8 haplotypes.
T-cell-mediated chronic inflammatory response.
Intestinal villous atrophy decreases absorptive surface, causing severe malabsorption.
Sudden exacerbation precipitates massive secretory and osmotic fluid losses.

Category	Manifestations
Gastrointestinal	Explosive diarrhea.
Metabolic	Profound electrolyte abnormalities, metabolic acidosis.
Hemodynamic	Severe dehydration, lethargy, potential hypovolemic shock.
Nutritional	Protein-losing enteropathy, profound hypoalbuminemia, severe malnutrition, cachexia.

Serum electrolytes: Detect profound derangements, confirm metabolic acidosis.
Complete blood count: Identify associated anemia, predominantly iron-deficient.
Hepatic panel: Evaluate secondary hypertransaminasemia, assess serum albumin for protein-losing enteropathy.
Celiac serology: Assess tissue transglutaminase (tTG) IgA and endomysial antibodies (EMA) IgA.

Esophagogastroduodenoscopy with biopsy: Required for definitive diagnosis after initial stabilization.
Target biopsy sites: Minimum four biopsies from distal duodenum, one from duodenal bulb.
Characteristic findings: Villous atrophy, crypt hyperplasia, intraepithelial lymphocytosis.

Intravenous fluid resuscitation: Mandatory intervention for severe dehydration and explosive diarrhea.
Electrolyte replacement: Urgent correction of profound deficits.
Acid-base management: Intravenous bicarbonate administration for severe metabolic acidosis.

Intravenous albumin infusion: Indicated for symptomatic hypoalbuminemia secondary to protein-losing enteropathy.

Lifelong, strict adherence to gluten-free diet.
Complete elimination: Wheat, rye, barley, related derivatives (triticale, spelt, kamut, malt).
Safe grain alternatives: Rice, corn, buckwheat, millet, amaranth, quinoa.
Threshold limits: Daily gluten intake strictly maintained below 10 mg.
Professional consultation: Mandatory skilled dietician involvement for counseling and compliance monitoring.

Iron therapy: 2-5 mg/kg elemental iron for deficiency anemia correction.
Vitamin supplementation: Fat-soluble vitamins (A, D, E, K) for malabsorption.
Bone health: Calcium and vitamin D supplementation to reverse osteopenia/osteoporosis.
Pancreatic enzyme supplementation: Temporary administration for secondary pancreatic hypofunction facilitating weight gain.

Corticosteroids: Short course indicated for severe, refractory enteropathy or associated multiple nonpeptic duodenal ulcers.
Immunosuppressants: Azathioprine, 6-mercaptopurine, or cyclosporine reserved for nonresponsive or refractory celiac disease failing strict dietary elimination.

Clinical response: Track resolution of diarrhea, monitor weight velocity and linear growth.
Serologic monitoring: Serial measurement of tTG IgA levels at 6 months, then annually to confirm dietary compliance.
Comorbidity screening: Evaluate for associated autoimmune conditions including type 1 diabetes, autoimmune thyroiditis, and selective IgA deficiency.