Celiac crisis, a rare and profound presentation of celiac disease: a case report.

BACKGROUND

Celiac crisis is a life-threatening manifestation of celiac disease and is rare in adults, with only a handful of cases documented worldwide and mostly in children.

CASE PRESENTATION: A profoundly emaciated 43-year-old female presented with profuse diarrhoea, shortness of breath, left leg swelling with ulceration and immobility (Body Mass Index (BMI) = 14.7 kg/m2). The patient had normal anion-gap metabolic acidosis (pH = 7.16) with persisting hypokalemia, hyponatremia, hypomagnesemia and hypophosphatemia. In addition, severe vitamin deficiencies and coagulopathy were present. A computed tomography pulmonary angiogram (CT-PA) revealed bilateral massive pulmonary embolism causing infarction, arising from a left lower limb extensive deep vein thrombosis (DVT). Bone marrow suppression was seen on aspirate. The patient developed severe urosepsis in her immunocompromised state. Prolonged lower limb weakness despite supportive therapy, rehabilitation and strict adherence to a gluten-free diet prompted the magnetic resonance imaging (MRI) confirmed diagnosis of subacute combined degeneration of the spinal cord due to Vitamin-B12 deficiency.

CONCLUSIONS

Celiac crisis is a rare and potentially life-threatening presentation of celiac disease, often a diagnosis of exclusion. Subacute combined degeneration of the spinal cord should be considered in patients with chronic Vitamin B12 deficiency presenting with neurological symptoms.