Auteur Topic: Voll.art.: The management of refractory coeliac disease  (gelezen 2633 keer)

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Voll.art.: The management of refractory coeliac disease
« Gepost op: mei 04, 2013, 16:23:45 »
Volledig artikel.


Auteurgegevens:
Jeremy Woodward
Consultant Gastroenterologist, Addenbrooke’s Hospital, Cambridge CB2 0QQ, UK

Ther Adv Chronic Dis. 2013 March; 4(2): 77–90.
doi:  10.1177/2040622312473174



The management of refractory coeliac disease

Abstract.
A significant proportion of patients with coeliac disease are ‘nonresponsive’ to gluten withdrawal.
Most cases of nonresponsive coeliac disease are due to persisting gluten ingestion.
Refractory coeliac disease (RCD) is currently defined by persistent symptoms and signs of malabsorption after gluten exclusion for 12 months with ongoing intestinal villous atrophy.
Primary (without initial response to diet) and secondary (relapse following response to diet) RCD is recognized.
RCD is further classified as type I or type II based on the absence or presence of a population of aberrant intestinal lymphocytes.
Quality of dietetic advice and support is fundamental, and lack of objective corroboration of gluten exclusion may result in over-identification of RCD I, particularly in those cases with persisting antibody responses.
Over-reliance on lymphocyte clonality similarly may result in over-diagnosis of RCD II which requires careful quantification of aberrant lymphocyte populations.
Management of RCD should be undertaken in specialist centres.
It requires initial intensive dietary supervision, strict gluten exclusion and subsequent re-evaluation.
There is currently insufficient evidence to recommend specific treatments.
Steroids are often used in both RCD I and II (albeit with little objective evidence of benefit in RCD II), and azathioprine as steroid-sparing therapy in RCD I.
There is growing evidence for the use of cladribine in RCD II with autologous stem cell transplantation in nonresponders, but this requires further multicentre evaluation.
There remains considerable controversy regarding the diagnosis, treatment and surveillance of RCD:
international consensus in these areas is urgently required to facilitate future therapeutic advances.


Current understanding of coeliac disease


Definitions of refractory coeliac disease
- Symptomatically refractory coeliac disease (‘nonresponsive’ coeliac disease)


Histological RCD


Serological RCD


Classification of RCD


Epidemiology and natural history of RCD


Aetiology of RCD


Clinical presentation of RCD


Investigation of RCD


Management of RCD
- Table 1.
  Characteristics, therapeutic options and outcomes in refractory coeliac disease (RCD).
  http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3610261/table/table1-2040622312473174/

- Steroids
- Purine analogue antimetabolites
- Other immunosuppressant agents
- Autologous stem cell transplantation


Surveillance in RCD


Future prospects for the treatment of RCD


Conclusions
A significant proportion of patients with coeliac disease are ‘nonresponsive’ to gluten withdrawal.
Nonresponsive coeliac disease is due to persisting gluten ingestion rather than RCD in the majority of cases.

RCD is currently defined as the presence of persistent symptoms and signs of malabsorption after gluten exclusion for 12 months with ongoing intestinal villous atrophy.
Primary (without initial response to diet) and secondary (relapse following response to diet) RCD is recognized.
RCD is further classified as type I or type II based on the absence or presence of a population of aberrant lymphocytes in the intestinal epithelium.

The quality of dietetic advice and support is fundamental to the diagnosis of RCD, and lack of objective corroboration of gluten exclusion is likely to result in over-identification of RCD I, particularly in those cases with persisting antibody responses.
Over-reliance on lymphocyte clonality similarly may result in over-diagnosis of RCD II which requires careful quantification of aberrant lymphocyte populations.

Management of RCD should be undertaken in centres specializing in RCD and requires initial intensive dietary supervision with strict gluten exclusion and subsequent re-evaluation.
There is currently insufficient evidence to recommend specific therapeutic interventions.
Steroids are often used as first-line therapy in both RCD I and II (but with little objective evidence of benefit in RCD II), and Azathioprine is used as steroid-sparing therapy in RCD I.
There is growing evidence from one centre for the use of cladribine in RCD II with ASCT in nonresponders.
However, formal multicentre collaborative evaluation is required before such interventions can be universally recommended.

There remains considerable controversy regarding the diagnosis, treatment and surveillance of patients with RCD and international consensus in these areas is urgently required in order to facilitate future therapeutic advances.


Volledig artikel:
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3610261/