Refractory (non-responsive) CD (RCD)

[kickingfrog]

Refractory Coeliac Disease (RCD)....

Posted by Charlotte, Oxford on 23/1/2006

....Best Pract Res Clin Gastroenterol. 2005 Jun;19(3):413-24.

Refractory coeliac disease.

Daum S, Cellier C, Mulder CJ.

Department of Medicine I, Gastroenterology, Infectious Diseases and Rheumatology, Charite, Campus Benjamin Franklin, Berlin, Germany.
[e-mail]

A small proportion of coeliac disease (CD) patients fail to improve after a gluten-free diet (GFD) and may be considered as atypical regarding their outcome (refractory coeliac disease).
The aim of this study is to diagnose and manage patients with CD who fail to improve after a GFD.

Refractory coeliac disease (RCD) is a malabsorption syndrome defined by persisting villous atrophy with, usually, an increase of intraepithelial lymphocytes (IELs) in the small bowel in spite of a strict GFD and comprises a heterogenous group of diseases.

Some of these diseases have to be excluded and can be treated by specific therapies like antibiotics in tropical sprue and giardiasis and immune globulin substitution in common variable immunodeficiency, while other malabsorption syndromes are less well defined and may require immunosuppressive therapy.
Standardized treatment, however, has not been evaluated in such patients so far.

In a subgroup of patients with RCD, an abnormal intraepithelial lymphocyte (IEL) population may be observed with the lack of surface expression of usual T-cell markers (CD3-CD8 and/or the T-cell receptor (TCR)) on IELs associated with T-cell clonality pattern suggest the presence of an early enteropathy-associated T-cell lymphoma (EATL) in a subgroup of patients with RCD.
This hypothesis has been supported by studies, which revealed progression into overt intestinal T-cell lymphomas in a subgroup of RCD.
Steroid treatment has been reported effective even in patients with underlying early EATL.
However, long-term results are unsatisfactory in most of these patients with RCD and parenteral nutrition has to be applied in some of these cases.
First results with more aggressive chemotherapies and use of cytokines are under way.
Due to the difficulty of diagnostic and therapeutic regimens patients should be referred to tertiary centres for coeliac disease.


PMID: 15925846 [PubMed - indexed for MEDLINE]

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Refractory coeliac disease: remission with infliximab and immunomodulators.

Turner SM, Moorghen M, Probert CS.

Department of Gastroenterology, Bristol Royal Infirmary, Marlborough Street, Bristol, UK.
[e-mail]

Coeliac disease is a T-cell-mediated enteropathy induced by gluten.
A minority of patients who fail to respond to a gluten-free diet may require intervention with immunomodulating drugs.
We report a case of refractory coeliac disease where remission was induced by the anti-tumour necrosis factor-alpha antibody infliximab and was maintained with prednisolone and azathioprine


www.ncbi.nlm.nih.gov/pubmed/15925846?dopt=Abstract


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Non responsive coeliac disease

From Helen on GF board


I had non responsive coeliac disease too and actually had 9 months on prednisolone followed by a couple of months on Budesonide which is another steroid usually used for Krohns apparently.
I was told the next alternatives were either chemo or immunosuppressants for life but luckily eventually the steroids work however I wish I'd stopped them sooner and tried something else.
Long term steroids are not good for you and you need to weigh up the options.
My advice would be to research Pred if that's what you are on and its side effects so you can discuss them with your doctors and be ready to look at both Chemo and Immunosuppressants (Azathioprine was the one mentioned)
I have steroid induced cataracts now and I have another friend who has Avascular necrosis because of steroid use. Take this with a good look at how long I was on them though!!!
My gastro said that to have to treat CD with steroids is very rare and to have them not respond is practically unheard ....

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unresponsive CD & azathioprine

Posted by eli on 29/1/2006
GF board


.... someone was having trouble with CD and was put on steroids, with azathioprine also being mentioned as a possibility?
I have been on the aza since the beginning of November now and my stomach is brilliant! Within a couple of weeks I felt the difference and now I reckon I have the best digestion in my family - fingers crossed it lasts.
...... I am now beginning to put on weight, I can even tolerate some dairy which I couldnt before (cadburys roses and clotted cream )
I am on steroids full time anyway, and at first they also helped my stomach a bit, but soon wore off.The aza, however is now at the end of the 3rd month and still working.
It seemed like a big scarey drug to take at the beginning and I had to think very hard before taking it but Im glad I have now.

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Refractory CD?

Posted by Vee Durham on 18/10/2013

...

...I was diagnosed with RCD 2 over twelve years ago and am being monitored by my consultant on an annual basis. This is on the understanding that if I notice 'changes' in the interim period - whatever they might be - I will contact him.

RCD is diagnosed by dry biopsy and shows abnormality of the T cells. Type 2 is a precursor to cancer. My villi (which had totally disappeared) now have stunted growth but are still able to absorb all the nutrients and vitamins I need for a healthy life. I no longer take vitamins or iron and my blood tests continue to show as healthy.

From time to time I have discomfort in my upper abdomen,... and this has been diagnosed as gastritis. I give my body a rest from the things that irritate it, such as alcohol!!! ha ha, then it recovers.

Video endoscopy still shows inflammation in the gut but I am so much healthier than when first diagnosed. I also have hiatus hernia. I no longer worry about the prognosis of RCD2. ....

[kickingfrog]

Titre du document / Document title
Defining gluten refractory enteropathy
Auteur(s) / Author(s)

BIAGI Federico (1) ; CORAZZC Gino R. (1) ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

(1) Gastroenterology Unit, University of Pavia, IRCCS Policlinico San Matteo, Pavia, ITALIE

Résumé / Abstract
Objective The diagnosis of coeliac disease (CD) is based on the responsiveness of the enteropathy to a gluten-free diet (GFD). This implies that terms such as 'non-responsive CD' and 'refractory CD' are almost paradoxical. In spite of this, these terms are commonly used in the literature, often with different and confusing meanings.

Methods On the basis of both a review of the literature and our clinical experience, we propose the following classification.
A condition characterized by a refractory enteropathy, not due to lymphoma, ulcerative jejunoileitis or collagenous sprue, but in which gluten sensitivity has been shown previously or could be shown while the patients were on an immunosuppressive therapy should be indicated as refractory CD.
Those patients in whom gluten sensitivity can be excluded should be considered to be affected by non-coeliac refractory sprue.
Finally, patients in whom the presence of CD cannot be either confirmed or excluded should be considered to be affected by undefined sprue.

Results Twenty-four certain refractory patients are described in the literature.
The data suggest a diagnosis of refractory CD in 13 patients, non-coeliac refractory sprue in three patients, and undefined sprue in eight patients

Conclusions We define refractory CD as a form of CD that no longer responds to a GFD.
Non-coeliac refractory sprue is a condition unrelated to CD. It could be either an independent condition or a common end point of different enteropathies.

Revue / Journal Title
European journal of gastroenterology & hepatology ISSN 0954-691X
Source / Source
2001, vol. 13, no5, pp. 561-565 (39 ref.)
Langue / Language
Anglais
Editeur / Publisher
Lippincott Williams & Wilkins, Hagerstown, MD, ETATS-UNIS (1989) (Revue)



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[kickingfrog]

Refractory coeliac disease: remission with infliximab and immunomodulators
/ Turner, SM; Moorghen, M; Probert, C.
In: Eur J Gastroenterol Hepatol, Vol. 17(6), 06.2005, p. 667 - 669.


Abstract

Coeliac disease is a T-cell-mediated enteropathy induced by gluten. A minority of patients who fail to respond to a gluten-free diet may require intervention with immunomodulating drugs. We report a case of refractory coeliac disease where remission was induced by the anti-tumour necrosis factor-alpha antibody infliximab and was maintained with prednisolone and azathioprine.
...



research-information.bristol.ac.uk/en/publications/refractory-coeliac-disea
se-remission-with-infliximab-and-immunomodulators(1e28cb0f-5a3f-4db8-b71f-231b0231aed2)/export.html

[kickingfrog]

Also please see:

coeliac.proboards.com/thread/1316/steroid-treatment-cd

[kickingfrog]

From CUK:

...Non responsive and refractory coeliac disease

The majority of people with coeliac disease start to feel much better after starting a gluten-free diet and symptoms usually improve within a few weeks. However, a small number of people with coeliac disease do not respond to the gluten-free diet or may initially respond to the diet and then start to experience symptoms again.

In most cases, the reason why people do not feel better after starting a gluten-free diet is because they are still eating foods containing gluten without knowing it or they have another condition that may be causing the symptoms, such as irritable bowel syndrome or lactose intolerance. For most people with non responsive coeliac disease, the first step is a thorough dietary review with a dietitian to identify potential sources of gluten contamination. During the dietary review, the dietitian may discuss avoiding certain foods which may be causing sensitivity. After making sure you are following a strict gluten-free diet, if you are still experiencing symptoms, your healthcare team may do further tests to eliminate other potential causes of your symptoms.

Refractory coeliac disease

Refractory coeliac disease is a very rare complication of coeliac disease with an increased risk of certain types of cancer.

Symptoms of refractory coeliac disease include:

ongoing severe diarrhoea
stomach pain
sudden unexpected weight loss.
Refractory coeliac disease is estimated to affect around two to five percent of people with coeliac disease. In most cases, refractory coeliac disease is diagnosed in people over the age of 50.

Diagnosis of refractory coeliac disease should be conducted in a specialist centre where further tests will be conducted.

It’s likely that you’ll have to have further endoscopy investigations to look at your gut lining again. In people with refractory coeliac disease, the lining of the gut remains damaged and little or no improvement is seen from when you started the diet.

Treatment
Because refractory coeliac disease is so rare, there have been limited studies on it. In addition to the gluten-free diet, drug treatments may be needed such as steroids, immunosuppressive drugs, chemotherapy or a combination of these.

www.coeliac.org.uk/coeliac-disease/about-coeliac-disease-and-dermatitis-herpetiformis/refractory-coeliac-disease/

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Management of Refractory Celiac Disease

Karoly Horvath, MD, PhD, Alessio Fasano, MD, Division of Pediatric Gastroenterology and Nutrition,
Department of Pediatrics, University of Maryland School of Medicine, Baltimore, Maryland.

Abstract and Introduction

Abstract
A small percentage of patients diagnosed with celiac disease fail to have a long-term favorable response
to a strict gluten-free diet.
These individuals continue to have symptoms and histologic abnormalities or have symptomatic relapse despite treatment with gluten-free diet and ultimately develop a potentially life-threatening disease known as refractory sprue. Based on data reported in recent studies, the majority of patients with refractory sprue have abnormal intraepithelial lymphocytes and a poor prognosis. The current hypothesis is that refractory sprue represents a transition state to intestinal lymphoma. The true prevalence of this disease entity is unknown. Future multicenter trials are therefore warranted to estimate its prevalence and establish clear therapeutic guidelines.

www.medscape.com/viewarticle/414940

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