title: Epidemiologic and therapeutic aspects of refractory coeliac disease - a systematic review.
abstract: BACKGROUND
  Refractory coeliac disease (RCD) is a rare and severe malabsorptive disease. The condition has two subtypes: RCDI and RCDII. Different treatments have been tested: and because RCD has a poor prognosis due to progress to enteropathy-associated T-cell lymphoma, the aim was to review the epidemiologic aspects and the therapeutic options for RCD.
  
  
  METHODS
  A systematic literature search was performed in 18 databases, and 122 records were identified. Incidence, prevalence, treatment methods and their efficacy were evaluated.
  
  
  RESULTS
  Among coeliac disease patients, the cumulative incidence of RCD is 1-4% per ten-year period and the prevalence is 0.31-0.38%. In the general population, the prevalence of RCD is 0.002%. Treatment of RCDI is azathioprine (effect 100%), mesalamine (effect 60%) or tioguanine (effect 83%). Treatment for RCDII is the antimetabolite cladribine (effect 81%) and autologous haematopoetic stem cell transplantation (effect 85%).
  
  
  CONCLUSIONS
  RCD is a very rare disease. The current evidence for RCDI treatment includes prednisolone in combination with the immunosuppressants azathioprine, mesalamine or tioguanine. The current evidence for RCDII treatment documents use of the antimetabolite cladribine, and if there is no effect, autologous haematopoetic stem cell transplantation may be attempted. In the future, there is a need for more effective treatments which will also prevent further progression to enteropathy-associated T-cell lymphoma.
authors.affiliation: sara.anna.rowinski@gmail.com.
authors.forename: Sara Anna
authors.initials: SA
authors.lastname: Rowinski
authors.forename: Erik
authors.initials: E
authors.lastname: Christensen
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D002446:Celiac Disease / Q000188:drug therapy* / Q000453:epidemiology* / Q000628:therapy; D017338:Cladribine / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D015994:Incidence; D019804:Mesalamine / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D015995:Prevalence; D013866:Thioguanine / Q000627:therapeutic use
chemical_list: D000893:Anti-Inflammatory Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal; D007166:Immunosuppressive Agents; D017338:Cladribine; D019804:Mesalamine; D011239:Prednisolone; D013866:Thioguanine; D001379:Azathioprine