Coeliac Crisis

Coeliac crisis is a rare, life threatening presentation of coeliac disease with profuse diarrhoea, severe dehydration, electrolyte disturbance, metabolic acidosis, hypoalbuminaemia and weight loss. It occurs in untreated or undiagnosed disease and needs intensive support.

Fewer than 100 cases have been reported, mostly in young children but also in adults. It accounts for a very small part of coeliac presentations.

Precipitants are infection, surgery, pregnancy, medication, and prolonged untreated disease.

Pathophysiology.

Severe villous atrophy causes massive malabsorption and secretory loss of fluid and electrolytes. Protein loss lowers oncotic pressure, causing oedema, and calcium and magnesium malabsorption cause tetany.

Profuse watery diarrhoea, vomiting, abdominal distension, marked weight loss, hypotension, peripheral oedema, tetany or muscle weakness, bleeding tendency (vitamin K deficiency), and altered consciousness.

Red flags.

• Hypotension or shock.
• Potassium under 3.0 mmol/L or cardiac arrhythmia.
• Tetany or seizures.
• Albumin under 25 g/L.
• Bleeding or raised INR.

Send tissue transglutaminase IgA with total IgA (or deamidated gliadin IgG in IgA deficiency), endomysial antibodies and duodenal biopsies once stable (villous atrophy). Test electrolytes (potassium, sodium, magnesium, calcium, phosphate), blood gas, urea, creatinine, albumin, INR, full blood count, iron studies, folate and vitamin B12, and stool culture. Exclude other causes of severe diarrhoea.

Differential diagnosis.

• Infective gastroenteritis.
• Inflammatory bowel disease.
• Autoimmune enteropathy.
• Refractory coeliac disease.
• Tropical sprue.
• Hormone secreting tumours.
• Protein losing enteropathy.

Resuscitate with intravenous crystalloid and correct electrolytes while starting a strict gluten free diet.

• Potassium chloride: 10 mmol/h IV through a peripheral line (20 mmol/h through a central line with continuous ECG monitoring); until potassium is 4.0 to 5.0 mmol/L; maximum 2 to 3 mmol/kg daily; monitor potassium every 4 to 6 hours, ECG and renal function; reduce in renal impairment.
• Magnesium sulphate: 2 g (8 mmol) IV over 20 minutes, repeated as needed to keep magnesium 1.0 mmol/L or more; maximum 20 mmol in 24 hours without repeat levels; monitor reflexes, respiratory rate and blood pressure; reduce by half when creatinine clearance is under 30 mL/min.
• Calcium gluconate 10% (symptomatic hypocalcaemia): 10 to 20 mL IV over 10 minutes, repeated once if tetany persists, then an infusion adjusted to keep corrected calcium at 2.1 mmol/L or more; monitor ECG and calcium every 4 to 6 hours; avoid mixing with bicarbonate or phosphate; reduce with digoxin.
• Albumin 20% infusion for symptomatic hypoalbuminaemia or refractory oedema: 100 mL IV over 1 to 2 hours, repeated once daily as needed; maximum 2 mL/kg daily; monitor fluid status; avoid in heart failure.
• Vitamin K: 10 mg IV slowly once daily for 3 days if INR is raised; maximum 10 mg daily; monitor INR; use caution for anaphylactoid reactions.
• Corticosteroid when there is no response to fluids and diet within 24 to 48 hours (limited evidence): hydrocortisone 100 mg IV every 6 hours, or methylprednisolone 1 mg/kg IV once daily (maximum 80 mg), for 3 to 5 days then taper over 1 to 2 weeks; maximum hydrocortisone 400 mg daily; monitor glucose, blood pressure and infection; avoid in uncontrolled sepsis.

Parenteral nutrition when enteral feeding fails; replace thiamine before glucose (thiamine 100 mg IV once daily for 3 to 5 days). Treat iron, folate and vitamin B12 deficiency.

Monitoring. Electrolytes every 4 to 6 hours initially, daily weights, fluid balance, ECG and INR.

Referral and escalation. Critical care for shock or arrhythmia; gastroenterology and dietitian for long term gluten exclusion; paediatric gastroenterology for children.

Special groups. Children need weight based fluids and electrolytes. Pregnancy needs obstetric input and early nutritional support.