Description
- Malabsorption syndrome related to immune reaction to gluten in diet. Also referred to as celiac sprue or gluten intolerance.
ICD-9 codes
- 579.0 celiac disease
Epidemiology
- Celiac disease may be more common than recognized.
- Almost 1% of the Western population is estimated to have celiac disease.
- Peak incidence occurs in adults who are aged 40-50 years.
- Celiac disease is more common in women than in men (3.33:1)
Causes
- Thought to be attributable to
– Genetics
– Toxic effect of gluten/gliadin (gliadin is a glycoprotein component of gluten)
– Immune reaction (antibody to gluten/gliadin)
Pathogenesis
- Gluten is found in most food products that contain wheat, barley, and rye, so it is widely prevalent in typical diets.
- Effects on intestinal mucosa
– Villi become blunted or flat
– Crypts hypertrophy
– Increased numbers of intraepithelial lymphocytes, plasma cells, and lymphocytes in lamina propria
Risk factors
- Family history of celiac disease
- Diabetes mellitus type 1
Complications
- Collagenous sprue
- Intestinal ulcers or strictures
- Nutritional complications
– Malabsorption
– Vitamin D deficiency
– Osteomalacia/rickets
: Short stature; iron-resistant anemia
.
- Delayed puberty
- Low bone mineral density and bone mineral content
- Malignancy (e.g. gastrointestinal cancer, non-Hogkin lymphoma)
Associated conditions
- Dermatologic conditions (e.g. dermatitis herpetiformis, xerosis, keratosis pilaris)
- Immunologic conditions
– Immunoglobulin (Ig)A deficiency
– Common variable immunodeficiency
- Rheumatologic conditions
– Sjögren syndrome
– Rheumatoid arthritis
- Gastrointestinal conditions
– Esophagitis
– Microscopic colitis and irritable bowel syndrome
– Association between celiac disease and primary biliary cirrhosis is controversial.
- Endocrine conditions
– Thyroid disease in children
– Osteoporosis
– Diabetes mellitus type 1
- Neurologic disorders (e.g. hypotonia, developmental delay, learning disorders, headache, attention-deficit hyperactivity disorder)
- Down syndrome
- IgA nephropathy
- Social phobia and depression
History
- Chronic diarrhea
- Foul-smelling stools (pale, bulky, frothy, floating)
- Abdominal bloating and pain
- Vomiting and weight loss
- Weakness, fatigue
