title: Short article: Mortality and differential diagnoses of villous atrophy without coeliac antibodies.
abstract: OBJECTIVE
  Villous atrophy (VA) of the small bowel is mainly related to coeliac disease (CD), whose diagnosis is made on the basis of positive endomysial/tissue transglutaminase antibodies while on a gluten-containing diet in the vast majority of patients. However, VA can also occur in other conditions whose epidemiology is little known. Our aim was to study the epidemiology and clinical features of these rare enteropathies.
  
  
  METHODS
  Clinical and laboratory data of all the patients with VA directly diagnosed in our centre in the last 15 years were collected and statistically analysed.
  
  
  RESULTS
  Between September 1999 and June 2015, 274 patients were diagnosed with VA. A total of 260 patients were also positive to coeliac antibodies; the other 14 had VA, but no IgA endomysial antibodies: five had common variable immunodeficiency, three had dermatitis herpetiformis, two had IgA deficiency associated with CD, one had abdominal lymphoma, one had unclassified sprue, one had olmesartan-associated enteropathy and one had seronegative CD. Mortality was 6.0 deaths per 100 person years (95% confidence interval: 2.2-16) in patients with VA but negative coeliac antibodies, whereas only 0.2 deaths per 100 person years (95% confidence interval: 0.1-0.6) occurred in coeliac patients.
  
  
  CONCLUSIONS
  Patients with VA and negative endomysial antibodies are rare. However, these forms of VA identify specific causes that can be diagnosed. These patients are affected by a very high mortality.
authors.affiliation: aFirst Department of Internal Medicine, Coeliac Centre Departments of bClinical Biochemistry cBiometry and Clinical Epidemiology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
authors.forename: Annalisa
authors.initials: A
authors.lastname: Schiepatti
authors.forename: Federico
authors.initials: F
authors.lastname: Biagi
authors.forename: Giacomo
authors.initials: G
authors.lastname: Fraternale
authors.forename: Claudia
authors.initials: C
authors.lastname: Vattiato
authors.forename: Davide
authors.initials: D
authors.lastname: Balduzzi
authors.forename: Simona
authors.initials: S
authors.lastname: Agazzi
authors.forename: Claudia
authors.initials: C
authors.lastname: Alpini
authors.forename: Catherine
authors.initials: C
authors.lastname: Klersy
authors.forename: Gino R
authors.initials: GR
authors.lastname: Corazza
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001284:Atrophy / Q000209:etiology / Q000401:mortality / Q000473:pathology; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D002446:Celiac Disease / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000175:diagnosis / Q000401:mortality; D003937:Diagnosis, Differential; D005188:False Negative Reactions; D005260:Female; D019204:GTP-Binding Proteins / Q000276:immunology; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood; D007413:Intestinal Mucosa / Q000473:pathology*; D007421:Intestine, Small / Q000473:pathology*; D007558:Italy / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D000091345:Protein Glutamine gamma Glutamyltransferase 2; D012189:Retrospective Studies; D011503:Transglutaminases / Q000276:immunology; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D007070:Immunoglobulin A; D000091345:Protein Glutamine gamma Glutamyltransferase 2; D011503:Transglutaminases; D019204:GTP-Binding Proteins