Update on the Diagnosis and Management of Refractory Coeliac Disease

Abstract

A small subset of coeliac disease (CD) patients experiences persisting or recurring symptoms despite strict adherence to a gluten-free diet (GFD). When other causes of villous atrophy have been excluded, these patients are referred to as refractory celiac disease (RCD) patients. RCD can be divided in two types based on the absence (type I) or presence (type II) of an, usually clonal, intraepithelial lymphocyte population with aberrant phenotype. RCDI usually runs a benign course and may be difficult to be differentiated from uncomplicated, slow responding CD. In contrast, RCDII can be defined as low-grade intraepithelial lymphoma and frequently transforms into an aggressive enteropathy associated T-cell lymphoma with dismal prognosis. This paper describes the clinical characteristics of RCDI and RCDII, diagnostic approach, and the latest insights in treatment options.

Autoimmunity of Gastrointestinal Tract

“…Another therapy proposed is mesenchymal cell application (MSC)-used intraperitoneally, intravenously. However, the results are not conclusive, although a decrease of pro-inflammatory cytokines in mucous biopsy was noted [51,52].…”

“…These patients are candidates for HSCT, even in poor clinical condition, because only this therapy offers possibility to cure the disease and save their life [51]. As alternative to HSCT from unrelated donor, autoHSCT was used for such patients with success in five out of eight patients with five-year survival in 66% [52].…”

“…Celiac disease is included into diseases treated with monoclonal antibodies only in the case of GFD resistance and progression of malabsorption syndrome [28,52]. The good effect of elimination of TNF is noted only in refractory celiac disease type 1.…”

Preventing complications in celiac disease: Our experience with managing adult celiac disease

“…We divided since 2001 into two types based on the absence (Type I) or presence (type II) of an, usually clonal, intraepithelial lymphocyte population with aberrant phenotype [33]. Our diagnostic approach and the latest insights in treatment options are easily accessible in literature [4].…”

“…However, mucosal recovery may last years after the start of a GFD [3]. CD occurs only in patients who express HLA-DQ2 and/or DQ8 molecules [4]. The prevalence of CD in adults varies between one in 100 and one in 300 in most parts of the world [5].…”