title: The incidence and clinical spectrum of refractory celiac disease in a north american referral center.
abstract: OBJECTIVE
  Refractory celiac disease (RCD) is one of the most serious causes of persistent symptoms in patients with celiac disease (CD). Published reports suggest that approximately half of patients in Europe are RCD type II, which carries a poor prognosis with a 5-year survival rate of ~50% compared with ~90% for RCD type I. However, disease patterns may be different in North America. The aim of this study was to explore the clinical spectrum of RCD in a North American population.
  
  
  METHODS
  Medical records of patients with biopsy-proven CD presenting to our institution were reviewed for a diagnosis of RCD. Demographic data, clinical characteristics, and mortality were evaluated and compared with our general CD population.
  
  
  RESULTS
  In all, 34 out of 844 (4.0%) CD patients had RCD. The cumulative incidence of RCD for patients diagnosed with CD at our center was 1.5%. Unintentional weight loss at diagnosis of RCD was found in 76.5% (n=26) compared with 16.7% (n=141) at diagnosis of CD (P<0.0001) and diarrhea at diagnosis of RCD was found in 79.4% (n=27) compared with 40.5% (342) at diagnosis of CD (P<0.0001). Five patients (14.7%) were diagnosed with RCD type II and of these, two died of enteropathy-associated lymphoma within 24 months of diagnosis of CD (observed mortality rate 5.9%).
  
  
  CONCLUSIONS
  Although RCD is a serious condition with significant morbidity; the observed mortality rates are low in our population. This study suggests that RCD may be less severe in North American vs. European populations.
authors.affiliation: Department of Gastroenterology, Celiac Center, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA. bakhtroshan@gmail.com
authors.forename: Bakht
authors.initials: B
authors.lastname: Roshan
authors.forename: Daniel A
authors.initials: DA
authors.lastname: Leffler
authors.forename: Shailaja
authors.initials: S
authors.lastname: Jamma
authors.forename: Melinda
authors.initials: M
authors.lastname: Dennis
authors.forename: Sunil
authors.initials: S
authors.lastname: Sheth
authors.forename: Kenneth
authors.initials: K
authors.lastname: Falchuk
authors.forename: Robert
authors.initials: R
authors.lastname: Najarian
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Goldsmith
authors.forename: Sohaib
authors.initials: S
authors.lastname: Tariq
authors.forename: Detlef
authors.initials: D
authors.lastname: Schuppan
authors.forename: Ciaran P
authors.initials: CP
authors.lastname: Kelly
mesh_terms: D001707:Biopsy, Needle; D002446:Celiac Disease / Q000145:classification / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D004386:Duodenum / Q000473:pathology; D005060:Europe; D006801:Humans; D016130:Immunophenotyping; D015994:Incidence; D011379:Prognosis; D015996:Survival Rate; D014481:United States