Celiac Sprue (Non-tropical Sprue)

Pathophysiology

1) disease of the gastrointestinal tract marked by malabsorption and lesions in the small intestine 2) prompt resolution of lesions when gluten is removed from diet

Signs and Symptoms

1) usually presents after cereal is introduced into diet 2) growth retardation owing to malabsorption 3) diarrhea 4) steatorrhea 5) weight loss 6) edema from protein loss 7) osteomalacia

Characteristic Test Findings

Laboratory – 1) anemia 2) decreased serum folic acid 3) decreased serum protein 4) decreased D-xylose test 5) increased serum testosterone 6) increased serum LH Small intestine biopsy – 7) diagnosis is by characteristic histology

Histology/Gross Pathology

1) “flat” intestinal wall, most pronounced in duodenum and proximal jejunum 2) blunting of microvilli and villi with crypt hyperplasia 3) proliferation of T lymphocytes in epithelium 4) infiltration of plasma cells in lamina propria

Associated Conditions

1) HLA-B8 or the class II antigens DR3 and DQw2 (90%) 2) gliadin (water-soluble alcoholic extract of wheat) has a significant homology with adenovirus 12 3) via a tissue transglutaminase, this virus may sensitize immunologically susceptible individuals who then react to the homologous gliadin with introduced in the diet

Inheritance/Epidemiology

1) occurs in 1/300 in some parts of Ireland 2) occurs in 1/3000 worldwide 3) affects all ethnic groups 4) female to male, 1.3:1 5) involved gene is DQB1*0201 6) occasionally spontaneous remissions occur in adolescence

Treatment

1) strict avoidance of foods containing gluten (wheat, barley, rye, oats) 2) if refractory to diet changes, a short course of steroids can be used

Tips for USMLE

1) hallmark of the disease is an abnormal small intestine biopsy that returns to normal after elimination of gluten from the diet 2) characteristic antibodies are antigliadin and antiendomysial 3) antiendomysial antibodies disappear after a period of gluten-free diet