title: [Gastrointestinal manifestations in patients with primary immunodeficiencies causing antibody deficiency].
abstract: BACKGROUND
  Primary immunodeficiencies can lead to gastrointestinal manifestations that are still not well defined.
  
  
  OBJECTIVE
  To analyze gastrointestinal manifestations associated with primary immunodeficiencies.
  
  
  METHODS
  We performed a retrospective study that included patients diagnosed with primary antibody deficiencies in a third-level hospital. The patients were divided into two groups: isolated IgA deficiency and common variable immunodeficiency syndrome (CVIS). The timing of presentation and type of gastrointestinal symptoms were analyzed.
  
  
  RESULTS
  There were 57 patients: 20 with CVIS (35%) and 37 with isolated IgA deficiency (65%). Diagnosis was made in the pediatric age in 17 patients, of whom 13 had isolated IgA deficiency. In 84% of the patients, diagnosis of immunodeficiency was made before the development of gastrointestinal manifestations. Digestive symptoms were found in 74% of the patients, the most frequent being diarrhea. In 46% of the patients, digestive disease was confirmed, mainly through endoscopy. Celiac-like lesions, chronic atrophic gastritis, ulcerative colitis-like disease and Crohn's disease were more common in CVIS. In isolated IgA deficiency, Helicobacter pylori-positive chronic gastritis predominated. Mean age was significantly higher (36 vs. 24 years, p=0.02) and IgA titer significantly lower (17 vs. 34UI/ml; p=0.008) in patients with associated gastrointestinal disease.
  
  
  CONCLUSIONS
  Gastrointestinal symptoms are frequent and lead to endoscopic diagnosis in half of patients with primary immunodeficiencies. Ulcerative colitis, and celiac- and Crohn's-like disease are atypical entities that occur in CVIS.
authors.affiliation: Sección de Aparato Digestivo, Hospital Universitario de León, León, España.
authors.forename: Rubén
authors.initials: R
authors.lastname: Díez
authors.forename: M Jesús
authors.initials: MJ
authors.lastname: García
authors.forename: Santiago
authors.initials: S
authors.lastname: Vivas
authors.forename: Laura
authors.initials: L
authors.lastname: Arias
authors.forename: Gabriela
authors.initials: G
authors.lastname: Rascarachi
authors.forename: Elvira del
authors.initials: Ed
authors.lastname: Pozo
authors.forename: Luis M
authors.initials: LM
authors.lastname: Vaquero
authors.forename: Aleida
authors.initials: A
authors.lastname: Miguel
authors.forename: Mónica
authors.initials: M
authors.lastname: Sierra
authors.forename: Sara
authors.initials: S
authors.lastname: Calleja
authors.forename: José M
authors.initials: JM
authors.lastname: Ruiz De Morales
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000150:complications*; D003967:Diarrhea / Q000209:etiology / Q000276:immunology; D016099:Endoscopy, Gastrointestinal; D005260:Female; D005756:Gastritis / Q000209:etiology / Q000276:immunology / Q000382:microbiology; D005767:Gastrointestinal Diseases / Q000209:etiology* / Q000276:immunology; D016481:Helicobacter Infections / Q000150:complications / Q000276:immunology; D016480:Helicobacter pylori / Q000302:isolation & purification; D006801:Humans; D017098:IgA Deficiency / Q000150:complications*; D007223:Infant; D015212:Inflammatory Bowel Diseases / Q000209:etiology / Q000276:immunology; D008286:Malabsorption Syndromes / Q000209:etiology / Q000276:immunology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult