Journal articles: 'Refractory celiac disease type 2'

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Soldera, Jonathan, Karina Salgado, and Karla Lais Pêgas. "Refractory celiac disease type 2: how to diagnose and treat?" Revista da Associação Médica Brasileira 67, no. 2 (February 2021): 168–72. http://dx.doi.org/10.1590/1806-9282.67.02.20200618.

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Bianchi, Nicoletta, Luisa Doneda, Luca Elli, Cristian Taccioli, Valentina Vaira, Alice Scricciolo, Vincenza Lombardo, et al. "Circulating microRNAs Suggest Networks Associated with Biological Functions in Aggressive Refractory Type 2 Celiac Disease." Biomedicines 10, no. 6 (June 14, 2022): 1408. http://dx.doi.org/10.3390/biomedicines10061408.

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Despite following a gluten-free diet, which is currently the only effective therapy for celiac disease, about 5% of patients can develop serious complications, which in the case of refractory type 2 could evolve towards intestinal lymphoma. In this study, we have identified a set of 15 microRNAs in serum discriminating between the two types of refractory disease. Upregulated miR-770-5p, miR-181b-2-3p, miR-1193, and miR-1226-3p could be useful for the better stratification of patients and the monitoring of disease development, while miR-490-3p was found to be dysregulated in patients with refractory type 1. Finally, by using bioinformatic tools applied to the analysis of the targets of dysregulated microRNAs, we have completed a more precise assessment of their functions. These mainly include the pathway of response to Transforming Growth Factor β cell–cell signaling by Wnt; epigenetic regulation, especially novel networks associated with transcriptional and post-transcriptional alterations; and the well-known inflammatory profiles.

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Malamut, Georgia, and Christophe Cellier. "Refractory Celiac Disease: Epidemiology and Clinical Manifestations." Digestive Diseases 33, no. 2 (2015): 221–26. http://dx.doi.org/10.1159/000369519.

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A small subset of celiac disease (CD) patients becomes refractory to a gluten-free diet with persistent malabsorption and intestinal villous atrophy. This is a rare (probably less than 2% of adult CD patients), but serious disorder, with a high risk of progression to an overt T-cell lymphoma. Diagnosis of this condition defined as refractory CD (RCD) is made after exclusion of other small bowel diseases with villous atrophy. RCD has been subdivided into two subgroups according to the normal (RCDI) or abnormal phenotype of intraepithelial lymphocytes (RCDII). Whereas RCDI is hardly distinguishable from active noncompliant CD, RCDII has a severe clinical presentation and a very poor prognosis. We precisely describe below the different types of RCD and propose diagnostic and therapeutic guidelines for its clinical management.

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Nasr, I., S. Donnelly, C. Ho-Yen, T. Mitchell, F. Chang, and P. Ciclitira. "PTH-112 A Single Centre Experience Of Treatment Of Refractory Celiac Disease Type 2." Gut 63, Suppl 1 (June 2014): A260.2—A261. http://dx.doi.org/10.1136/gutjnl-2014-307263.558.

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Tack, Greetje J., Marielle J. Wondergem, Abdul Al-Toma, Wieke H. Verbeek, Alexander Schmittel, Mariana V. Machado, Francesco Perri, et al. "S2024 Refractory Celiac Disease Type 2: Haematopoietic Autologous Stem Cell Transplantation Does Improve Clinical Outcome." Gastroenterology 138, no. 5 (May 2010): S—303—S—304. http://dx.doi.org/10.1016/s0016-5085(10)61396-0.

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Vaira, Valentina, Gabriella Gaudioso, Maria Antonella Laginestra, Andrea Terrasi, Claudio Agostinelli, Silvano Bosari, Antonio Di Sabatino, et al. "Deregulation of miRNAs-cMYC circuits is a key event in refractory celiac disease type-2 lymphomagenesis." Clinical Science 134, no. 10 (May 2020): 1151–66. http://dx.doi.org/10.1042/cs20200032.

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Abstract A percentage of celiac disease (CD) patients develop refractory type-2 disease (RCD2), a condition associated with increased risk of enteropathy-associated T-cell-lymphoma (EATL) and without therapeutic option. Therefore, we profiled the miRNome in series of peripheral T-cell lymphomas (PTCLs), CD, RCD1 or 2 and in the murine interleukin-15 (IL15)-transgenic (TG) model of RCD. The transcriptome was analyzed in 18 intestinal T-cell lymphomas (ITLs). Bioinformatics pipelines provided significant microRNA (miRNA) lists and predicted targets that were confirmed in a second set of patients. Our data show that ITLs have a unique miRNA profile with respect to other PTCLs. The c-MYC regulated miR-17/92 cluster distinguishes monomorphic epitheliotropic ITL (MEITL) from EATL and prognosticates EATL outcome. These miRNAs are decreased in IL15-TG mice upon Janus kinase (JAK) inhibition. The random forest algorithm identified a signature of 38 classifier miRNAs, among which, the miR-200 and miR-192/215 families were progressively lost in RCD2 and ITL-CD, whereas miR-17/92 and C19MC miRNAs were up-regulated. Accordingly, SMAD3, MDM2, c-Myc and activated-STAT3 were increased in RCD2 and EATL tissues while JAK inhibition in IL15-TG mice restored their levels to baseline. Our data suggest that miRNAs circuit supports activation of STAT3 and c-Myc oncogenic signaling in RCD2, thus contributing to lymphomagenesis. This novel understanding might pave the way to personalized medicine approaches for RCD and EATL.

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Le Mouel, Jean-Philippe, Mathurin Fumery, Sami Hakim, Clara Yzet, Alexandra Dervaux, Xavier Dray, and Eric Nguyen-Khac. "Type 2 refractory celiac disease on third-generation capsule endoscopy and enteroscopy: typical appearance of ulcerative jejunitis." Endoscopy 52, no. 06 (December 13, 2019): E195—E197. http://dx.doi.org/10.1055/a-1046-1593.

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Chandesris, Marie-Olivia, Georgia Malamut, Virginie Verkarre, Bertrand Meresse, Gabriel Rahmi, Elizabeth Macintyre, Nicole Brousse, Nadine Cerf-Bensussan, Christophe Cellier, and Olivier Hermine. "The Type of Enteropathy Is a Prognostic Factor in Enteropathy-Associated T-Cell Lymphoma." Blood 114, no. 22 (November 20, 2009): 2937. http://dx.doi.org/10.1182/blood.v114.22.2937.2937.

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Abstract Abstract 2937 Poster Board II-913 Enteropathy-associated T-cell Lymphoma (EATL) may complicate celiac disease (CD), refractory celiac disease type I (RCD I) characterized by normal intraepithelial lymphocytes (IEL) or refractory celiac disease type II (RCD II) defined by abnormal IEL (CD3s-,CD3i+ and CD8-) and a clonal rearrangement of the gamma or delta chain of the T cell receptor (TCRγ/δ). It remains unknown whether the type of the associated enteropathy, non clonal (CD or RCD I) or clonal (RCD II), may be a prognostic factor in EATL. We aimed to assess the prognosis of EATL according to the type of the associated enteropathy. Medical files of 29 patients with EATL were retrospectively studied. The type of associated enteropathy was confirmed by immunohistochemistry analysis in all cases and by flow cytometry phenotyping analysis of freshly isolated IEL and search for a TCR γ or δ clonal rearrangement by Multiplex PCR whenever possible. Kaplan-Meier curves and Logrank test were used to compare survival of EATL in the 2 groups of enteropathy (clonal or not). Mean age at EATL (13 women / 16 men) onset was 57 years. The associated enteropathy was CD (n=10) or RCD I (n=2) in 12 patients and RCD II in 17 patients. No statistical difference was found in lymphoma staging with localized (IE and IIE) versus disseminated stages (IV) found in 42% and 58% of patients with CD or RCD I and in 53% and 47% of patients with RCD II, respectively. Diagnostic or therapeutic surgery was practiced in 83% and 47% of patients with CD or RCD I and with RCD II, respectively and chemotherapy in 92% and 82% of the same groups of patients, respectively (n.s). Considering all the EATL, the two-year and five-year survival rates of EATL were 34.1% and 20.2%, respectively. In subgroup analysis, the two-year and five-year survival rates of EATL were 66.7% and 53.3% in case of CD and/or RCD I and 11.8% and 0% in case of RCD II, respectively (p=0.0007). In conclusion, the type of enteropathy significantly impacts the prognosis of EATL with a particular short survival in case of associated RCD II enteropathy. Disclosures: No relevant conflicts of interest to declare.

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Boutrid, N., and H. Rahmoune. "Tofacitinib, celiac disease and the elderly: mind the gut!" Acta Gastro Enterologica Belgica 86, no. 3 (September 2023): 502. http://dx.doi.org/10.51821/86.3.12207.

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To the Editor, We read with great attention the interesting case report by Lenfant et al. narrating the successful use of tofacitinib in a patient with microscopic colitis and celiac disease (1), and we would point some insights about this peculiar situation. In fact, tofacitinib depicts potential side effects, including a higher risk of malignancies, and the FDA has even issued a warning about this drug’s hazard (2). Actually, a randomized open-label trial published in the New England Journal of Medicine in 2021 found that patients with rheumatoid arthritis who took tofacitinib had a higher risk of developing cancer than those who took a tumor necrosis factor (TNF) inhibitor (3). Recently, two recent randmoized controlled trials from the ORAL Surveillance Trial and published in the Annals of the Rheumatic Diseases in 2023 also contributed to shed the light on this potential risk : that patients with rheumatoid arthritis aged > 50 with cardiovascular risk who took tofacitinib had a higher risk of developing any type of cancer than those who took a TNF inhibitor (4), and secondary stratification found that they were more likely to develop cancer if they were over the age of 65 years (5). Adding insult to injury, the maligancies are also driven by the two peculiar forms of celiac disease : seronegative and refractory celiac disease (RCD), and this risk is also increased in CD diagnosed at adulthood : particularly, elder patients are prone to present a RCD, and giving immune checkpoint therapy might increase this risk (6). In conclusion, RCD ought to be definetly ruled out before starting JAK inhibitor therapy, especially in aged population with seronegative celiac disease and microscopic colitis, two well-known conditions associated with RCD.

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Al-Toma, Abdul, and Harry R. Koene. "Hematopoietic Stem Cell Transplantation in Refractory Celiac Disease: An Overview with Focus on Infectious Complications." OBM Transplantation 04, no. 01 (February 18, 2020): 1–17. https://doi.org/10.21926/obm.transplant.2001101.

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Refractory celiac disease (RCD) is a rare condition in which a known celiac patient, usually an adult, suffers from persistence or recurrence of gluten-related symptomatology, laboratory abnormalities, and inflammatory enteropathy despite following an optimal dietary therapy with gluten-free diet (GFD). Arbitrarily, a duration of at least 12 months of GFD has been recommended prior to establishing such a diagnosis. Furthermore, exclusion of the other possible causes of non-celiac villous atrophy, particularly enteropathy associated T-cell lymphoma (EATL), is a prerequisite for establishing a diagnosis of RCD. RCD is subdivided into two types, depending on the percentage of immunophenotypically aberrant intraepithelial lymphocytes (IEL). The refractory patients having a high percentage of abnormal ‘aberrant’ IEL (RCD-II) are regarded as having pre-lymphoma due to the high probability of developing EATL. In addition, they are at high risk for infection owing to the impaired immunity resulting from malnutrition, bacterial overgrowth and translocation in the small intestine, and the presence of hyposplenism (functional asplenia). The RCD-II patients are generally non-responsive to the currently available pharmacological treatments. However, both clinical and histopathological remissions have been achieved using the purine analog cladribine (2-CDA). Autologous hematopoietic stem cell transplantation (auto-HSCT) appears to be an effective therapy for these patients as it is well tolerated and has a low risk of post-transplant infections or other complications. The present review provides an overview of the application of auto-HSCT for the treatment of patients with RCD-II, which is a classic example of an autoimmune disorder. The focus is particularly on the infectious complications developing after the application of auto-HSCT.

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Sibon, David, Georgia Malamut, Virginie Verkarre, Coralie Derrieux, Isabelle Radford, Bertrand Meresse, Elizabeth Macintyre, et al. "Enteropathy-Associated T-Cell Lymphoma Type I, But Not Refractory Celiac Disease, Strongly Expresses CD30 and Might Benefit From Brentuximab Vedotin." Blood 122, no. 21 (November 15, 2013): 4252. http://dx.doi.org/10.1182/blood.v122.21.4252.4252.

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Abstract Introduction Enteropathy-associated T-cell lymphoma (EATL) is an intestinal tumor of intraepithelial T lymphocytes. The 2008 WHO classification distinguishes two types of EATL: the first type of EATL (type I), the most frequent (80-90%) is strongly associated with celiac disease (CD) and the HLA-DQ2/DQ8 haplotypes. The tumor cells are CD3+CD8-/+CD4-CD56- and contain cytotoxic granules. In almost all cases, a varying proportion of the tumor cells express CD30. The second type of EATL (type II), the monomorphic form, has a distinct immunophenotype (CD3+CD4-CD8+CD56+). In two types, TCRgamma genes are often clonally rearranged. EATL may be preceded by refractory celiac disease (RCD), corresponding to CD refractory to gluten free diet (GFD). RCD is divided in two types based on the absence (type I) or presence (type II) of abnormal intraepithelial lymphocytes (IEL) showing down-regulation of CD8 and often TCRgamma genes rearrangement. RCD II is now considered as a small cell intraepithelial T-cell lymphoma that could be an intermediate stage between CD and EATL. The aim of the present study was to establish the pattern of CD30 expression in EATL. This could have therapeutic implications with the use of anti-CD30 monoclonal antibody like brentuximab vedotin (BV). Methods Consecutive adult patients (pts) diagnosed with EATL between 2007 and 2013 in two university hospitals in Paris (Necker University Hospital and Georges Pompidou European Hospital) were eligible for this study. Diagnosis was confirmed after histopathologic and immunohistochemical review. For the purpose of the present study, two expert hematopathologists (V.V. and N.B.) reviewed all EATL and extended the phenotypic analysis to reclassify them according to the 2008 WHO classification. A panel of antibodies directed against CD20, CD3, CD4, CD5, CD8, CD56, granzyme B and ALK1 was used. CD30 staining was performed with Monoclonal Mouse Anti-Human CD30, Clone Ber-H2 (Dako). Consecutive RCDI and RCDII cases with complete phenotype and clonality analyses diagnosed in the same period were used as control. Diagnosis of CD was based on HLA-DQ2/8 typing, detection of celiac specific antibodies and of villous atrophy with increased counts of IEL on normal diet. Pts were further classified in RCDI or II depending on their clinical and histological response to a GFD and the presence of abnormal IEL. Results Twenty five adults were diagnosed with EATL on consensus review (median age 53 years [range 34-76], M/F ratio 12/13). Twenty five RCDI pts (median age 51 years [range 16-75], M/F ratio 6/19) and 20 RCDII pts (median age 62 years [range 29-81], M/F ratio 7/13) were used as control. A clinical history of CD was found in 17/20 (85%) evaluable EATL pts. Histological features of CD/RCD were seen in all cases (20/20) of EATL in which the mucosa adjacent to the tumor could be investigated (half of these were RCDII). Primary sites of EATL were small intestine (20/25), mesenteric lymph nodes (3/25), peritoneal nodules (1/25) and spleen (1/25). Phenotypic analysis showed that EATL cases were all WHO type I (25/25). ALK1 was constantly negative. IEL were CD3+ in all 70 cases. CD8 was normally expressed in all RCDI IEL and downregulated in all RCDII IEL and 35% of EATL. In all cases of EATL (25/25), CD30 was strongly expressed by all large tumor cells. In RCDI and II, CD30 was negative in most cases (∼90%), and was rarely expressed by dispersed atypical lymphocytes (IEL or in lamina propria) in some cases. TCRgamma genes were clonally rearranged in 11/14 (79%) EATL, 3/25 (12%) RCDI and 18/20 (90%) RCDII. Based on these results, we initiated in 2012 a pilot study combining BV with chemotherapy followed by autologous stem-cell transplantation (ASCT) as frontline treatment of EATL. Five pts have currently been treated. The associated chemotherapy regimen was IVE/MTX (Sieniawski M, Blood 2010) for the first two pts. After presentation at ASH 2012 Annual Meeting of preliminary results of a phase 1 study combining BV with CHP regimen as frontline treatment of systemic ALCL and other CD30-positive mature T–cell and NK–cell lymphomas (Fanale MA, Abstract #60), we replaced IVE/MTX by CHP regimen, and treated 3 other pts. The treatment was well tolerated, and the 5 pts reached CR and underwent ASCT. Conclusion CD30 is strongly expressed in EATL type I. Promising results of the combination of BV with CHP led us to plan a phase 2 study of BV and CHP followed by ASCT as frontline treatment of EATL. Disclosures: Off Label Use: Brentuximab vedotin was used in enteropathy-associated T-cell lymphoma (EATL).

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Tjon, Jennifer M. L., Wieke H. M. Verbeek, Yvonne M. C. Kooy-Winkelaar, Binh H. Nguyen, Arno R. van der Slik, Allan Thompson, Mirjam H. M. Heemskerk, et al. "Defective synthesis or association of T-cell receptor chains underlies loss of surface T-cell receptor–CD3 expression in enteropathy-associated T-cell lymphoma." Blood 112, no. 13 (December 15, 2008): 5103–10. http://dx.doi.org/10.1182/blood-2008-04-150748.

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Abstract Enteropathy-associated T-cell lymphoma, an often fatal complication of celiac disease, can result from expansion of aberrant intraepithelial lymphocytes in refractory celiac disease type II (RCD II). Aberrant intraepithelial lymphocytes and lymphoma cells are intracellularly CD3ϵ+ but lack expression of the T-cell receptor (TCR)–CD3 complex on the cell surface. It is unknown what causes the loss of TCR-CD3 expression. We report the isolation of a cell line from an RCD II patient with the characteristic phenotype of enteropathy-associated T-cell lymphoma. We demonstrate that in this cell line the TCR-α and -β chains as well as the CD3γ, CD3δ, CD3ϵ, and ζ-chains are present intracellularly and that assembly of the CD3γϵ, CD3δϵ, and ζζ-dimers is normal. However, dimerization of the TCR chains and proper assembly of the TCR-CD3 complex are defective. On introduction of exogenous TCR-β chains, but not of TCR-α chains, assembly and functional cell surface expression of the TCR-CD3 complex were restored. Defective synthesis of both TCR chains was found to underlie loss of TCR expression in similar cell lines isolated from 2 additional patients. (Pre)malignant transformation in RCD II thus correlates with defective synthesis or defective association of the TCR chains, resulting in loss of surface TCR-CD3 expression.

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Meresse, Bertrand, Natalia Korneychuk, Georgia Malamut, and Nadine Cerf-Bensussan. "Interleukin-15, a Master Piece in the Immunological Jigsaw of Celiac Disease." Digestive Diseases 33, no. 2 (2015): 122–30. http://dx.doi.org/10.1159/000369521.

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Background: The immune response causing celiac disease (CD) depends on the activation of intestinal CD4+ T cells by gluten-derived peptides presented by HLA-DQ2 or HLA-DQ8 molecules, the main genetic risk factor. However, additional factors are necessary to impair immune tolerance to dietary gluten, to stimulate intraepithelial lymphocytes (IEL) and to induce intestinal damage. Key Messages: Current data point to a central role of interleukin-15 (IL-15). In situ and ex vivo studies indicate that IL-15 stimulates the accumulation and cytotoxic activation of CD8+ T IEL in active CD, and that of the malignant innate-like IEL in type II refractory CD (RCDII). Other studies show that IL-15 impairs the immunoregulatory control of effector T cells, notably CD8+. Recently, animal models have been designed to investigate the respective role of CD4+ T cells and IL-15 in CD. We discuss more particularly our results in such a model, which shows that IL-15 produced in excess in the intestine can cooperate with CD4+ T cells specific for a dietary antigen to trigger a celiac-like enteropathy. In this mouse model, CD4+ T cells activated by dietary ovalbumin secreted IL-2 which, along with IL-15, stimulated the expansion of noncognate intestinal cytotoxic CD8+ T cells containing large amounts of granzyme B. In the presence of IL-15, the latter cells did not respond to regulatory T cells, and accumulated in the intestine close to epithelial damage. Conclusion: On the basis of these data, we propose that, in CD, gluten-specific CD4+ T cells synthesize cytokines that synergize with IL-15 to license the expansion and activation of cytotoxic IEL, which drive tissue damage. We suggest that IL-15 is a meaningful therapeutic target, notably in patients with RCDII in which malignant IEL can respond to IL-15 independently of signals provided by CD4+ T cells.

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Kooy-Winkelaar, Yvonne M. C., Dagmar Bouwer, George M. C. Janssen, Allan Thompson, Martijn H. Brugman, Frederike Schmitz, Arnoud H. de Ru, et al. "CD4 T-cell cytokines synergize to induce proliferation of malignant and nonmalignant innate intraepithelial lymphocytes." Proceedings of the National Academy of Sciences 114, no. 6 (January 3, 2017): E980—E989. http://dx.doi.org/10.1073/pnas.1620036114.

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Refractory celiac disease type II (RCDII) is a severe complication of celiac disease (CD) characterized by the presence of an enlarged clonal population of innate intraepithelial lymphocytes (IELs) lacking classical B-, T-, and natural killer (NK)-cell lineage markers (Lin−IELs) in the duodenum. In ∼50% of patients with RCDII, these Lin−IELs develop into a lymphoma for which no effective treatment is available. Current evidence indicates that the survival and expansion of these malignant Lin−IELs is driven by epithelial cell-derived IL-15. Like CD, RCDII is strongly associated with HLA-DQ2, suggesting the involvement of HLA-DQ2–restricted gluten-specific CD4+ T cells. We now show that gluten-specific CD4+ T cells isolated from CD duodenal biopsy specimens produce cytokines able to trigger proliferation of malignant Lin−IEL lines as powerfully as IL-15. Furthermore, we identify TNF, IL-2, and IL-21 as CD4+ T-cell cytokines that synergistically mediate this effect. Like IL-15, these cytokines were found to increase the phosphorylation of STAT5 and Akt and transcription of antiapoptotic mediator bcl-xL. Several small-molecule inhibitors targeting the JAK/STAT pathway blocked proliferation elicited by IL-2 and IL-15, but only an inhibitor targeting the PI3K/Akt/mTOR pathway blocked proliferation induced by IL-15 as well as the CD4+ T-cell cytokines. Confirming and extending these findings, TNF, IL-2, and IL-21 also synergistically triggered the proliferation of freshly isolated Lin−IELs and CD3−CD56+ IELs (NK-IELs) from RCDII as well as non-RCDII duodenal biopsy specimens. These data provide evidence implicating CD4+ T-cell cytokines in the pathogenesis of RCDII. More broadly, they suggest that adaptive immune responses can contribute to innate IEL activation during mucosal inflammation.

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Balitsky, Amaris, Anca Prica, Jan M. A. Delabie, Michael Crump, Vishal Kukreti, and John Kuruvilla. "Enteropathy-Associated T-Cell Lymphoma: A Single Centre Experience." Blood 126, no. 23 (December 3, 2015): 5057. http://dx.doi.org/10.1182/blood.v126.23.5057.5057.

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Abstract Introduction: Enteropathy-associated T-cell Lymphoma (EATL) is a rare lymphoma of the GI tract accounting for less than 1% of all non-Hodgkin's lymphomas (NHL). EATL type I is seen predominately in patients (pts) from parts of Europe associated with a high incidence of celiac disease; type II is less frequently associated with celiac disease and thus a more worldwide distribution. EATL is difficult to treat with very poor prognosis. The purpose of this study is to understand the epidemiology and outcomes of this rare lymphoma in a North American multicultural setting at a tertiary cancer care centre. Methods: All pts with EATL who were assessed and treated at Princess Margaret Cancer Centre in Toronto, Canada, between January 1, 1996 and January 30, 2015 were retrospectively reviewed with case ascertainmentfrom a pathology database as well as a prospectively populatedlymphoma database. Pathological diagnoses were made according to the WHO classification and central pathology review was undertaken when available. Staging procedures included CT scans and bone marrow biopsy. Simple descriptive statistics were calculated as well as progression-free survival (PFS) and overall survival (OS). Results: 4852 new NHLs were seen for primary treatment at our centre during this time period, of which 287 were T cell lymphomas; 11 had EATL (0.23% of all NHL cases and 3.8% of all T-cell lymphomas). Median age at presentation was 61 years (range 51-85) with the majority being male (64%) (Table 1). All 11 pts presented with abdominal pain from small bowel perforation or obstruction. The most common site of EATL was jejunum (55%). Four pts (36%) had EATL type 1 and three had positive celiac serology. None had a history of celiac disease prior to diagnosis. 46% and 36% of pts presented with a high Ann Arbour stage (stage III or IV) and high Lugano stage (stage III or IV) respectively. No pt had a high ECOG score at presentation. Central pathology review was available for 8 patients (table 1). Interestingly, only 36% of cases had an intermediate-high risk International Prognostic Index (IPI) score of 3 and no pts had a high risk score of 4 or 5. Only one pt had an elevated LDH. All type 1 pts were CD56-, while type 2 pts were all CD8+ and most (86%) were CD56+. Ten pts (91%) received CHOP chemotherapy (one with the addition of etoposide and one of an IL2 receptor antibody) and one pt received palliative therapy. Only 5 of 11 responded to therapy (45%), with 2 patients achieving complete remission. All patients have progressed through treatment or relapsed (6 pts primary refractory and 5 responders), with one late relapse 14 years post response. At relapse, patients often presented with another bowel perforation. Upon relapse, four (36%) pts received salvage chemotherapy and 1 patient received an autologous stem cell transplant but progressed 16 months later and died from his disease. Ten patients (91%) have died, all from lymphoma; one patient (the late relapser) is presently undergoing salvage chemotherapy. Both median PFS and OS for all pts were 7 months. Two year OS and PFS were 18% and 9% respectively. Table 1 provides PFS and OS for EATL type separately. Conclusions: EATL is an aggressive form of lymphoma with unique presentation and very poor prognosis despite frequently presenting with limited disease and low IPI score. In our centre, type II EATL was more common, potentially underlying the multicultural composition of our city. CHOP with or without etoposide remains inadequate therapy for this aggressive lymphoma, and alternative induction regimens are needed. Few patients are stem cell transplant candidates at relapse due to age and poor performance status. Table 1. Patient characteristics Feature EATL1 (N=4) EATL 2 (N=7) Age (yrs) median (range) 65 (55-71) 61 (51-85) Gender (%) male 1 (25) 6 (86) Celiac status (%) positive 3(75) 0 Pathology CD3+ 4(100) 7(100) CD7+ 3(75) 5(71) CD4+/CD8- 1(25) 0 CD4-/CD8+ 2(50) 7(100) CD4-/CD8- 1(25) 0 CD56+ 0 6(86) T-cell receptor clonal gene rearrangement 1/2(50) 4/4(100) Symptoms (%) abdominal pain 4(100) 7(100) constitutional symptoms 3(75) 1(17) lymphadenopathy 2(50) 1(14) bone marrow 1 (25) 0 Primary Location (%) duodenum 2 (50) 3 (43) jejunum 1 (25) 5 (71) ileum 3 (75) 0 Monofocal (%) yes 3 (75) 3 (43) Anne Arbor Staging (%) III or IV 2 (50) 3 (43) Lugano Staging (%) III or IV 2 (50) 2 (29) IPI (%) score 3-5 1(25) 3 (43) Response to tx CR/CRu/PR 1(25) 4 (57) PFS median (mo) 6 7 2 year PFS 25% 0% OS median (mo) 7 8 2 year OS 25% 14% Disclosures Prica: Janssen: Honoraria; Celgene: Honoraria. Crump:Celgene: Consultancy; Seattle Genetics: Consultancy; Roche Canada: Consultancy. Kukreti:Celgene: Honoraria; Roche: Honoraria; Lundbeck: Honoraria; Amgen: Honoraria; Janssen Ortho: Honoraria. Kuruvilla:Lundbeck: Honoraria; Karyopharm: Honoraria; Merck: Honoraria; Bristol-Myers Squibb: Honoraria; Janssen: Consultancy, Honoraria; Gilead: Consultancy; Seattle Genetics: Consultancy, Honoraria; Hoffmann LaRoche: Consultancy, Honoraria, Research Funding.

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Sibon, David, Sherine Khater, Julie Bruneau, Chantal Brouzes, Ludovic Lhermitte, Thierry Jo Molina, Guillaume Cartron, et al. "The Eatl-001 Trial: Results of a Phase 2 Study of Brentuximab Vedotin and CHP Followed By Consolidation with High-Dose Therapy - Autologous Stem-Cell Transplantation (HDT-ASCT) in the Frontline Treatment of Patients with Enteropathy-Associated T-Cell Lymphoma." Blood 138, Supplement 1 (November 5, 2021): 136. http://dx.doi.org/10.1182/blood-2021-153709.

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Abstract Background Enteropathy-associated T-cell lymphoma (EATL), previously designated type 1 EATL, is a neoplasm of intraepithelial T cells that occurs in individuals with celiac disease (CD). It is a rare lymphoma, accounting for approximately 3% of all peripheral T-cell lymphomas (PTCLs). EATL may be preceded by refractory CD (RCD), defined as persistent or recurrent symptoms and signs of malabsorption with villous atrophy despite a strict gluten-free diet for more than 12 months. Currently, RCD is categorized into 2 types, based on immunophenotypic and molecular criteria. In RCD-II, intraepithelial lymphocytes (IELs) have an aberrant phenotype and a clonal TCR gene rearrangement. RCD-II is considered a low-grade lymphoma of intraepithelial T cells, with a high risk of transformation into EATL. CD or RCD may be diagnosed prior to or concomitant with EATL. EATL has a poor prognosis due to perforation or obstruction of the bowel, sepsis, malnutrition and treatment resistance, with 2-year OS of 20% (de Baaij, CCR 2015). An EATL prognostic index (EPI) has been developed, that can distinguish 3 risk groups (de Baaij, CCR 2015). Optimal treatment of EATL is an unmet need, and novel therapeutic approaches are required. Most EATLs are CD30+ and could be targeted by brentuximab vedotin (BV). Based on the encouraging activity and manageable safety profile of BV and CHP (cyclophosphamide, doxorubicin and prednisone) combination in CD30+ PTCLs, the EATL-001 phase 2 trial was initiated to assess the efficacy and safety of BV-CHP followed by HDT-ASCT for the frontline treatment of patients (pts) with EATL (ClinicalTrials.gov No. NCT03217643). Here we report the first results of the EATL-001 trial. Methods EATL-001 is an Investigator Initiated-Sponsored Research phase 2 study, on behalf of the CELAC (French NCI-labeled network of Centers of Expertise for Lymphomas Associated with Celiac disease). Key inclusion criteria were as follows: Newly diagnosed CD30+ (≥10% of neoplastic cells by central review) EATL (WHO 2016 criteria), 18-65 years, PS 0-3. Response was assessed according to the Lugano classification. Pts were scheduled to receive 4 cycles of BV+CHP as induction. Responding pts received 2 cycles of Etoposide (200 mg/m2) + Methotrexate (3 g/m2) followed by HDT-ASCT (BEAM conditioning regimen). The primary endpoint was 2-year PFS per investigator. Underlying CD/RCD diagnosis was based on uninvolved duodenal histology (including CMF and TCR gene rearrangement analysis of IEL), serology and HLA typing. Results A total of 14 pts were included between February 2018 and February 2021. The median age was 54 years (range, 34-65) and 64% were male. 11 pts (79%) had initial surgery for bowel obstruction (n=6) or jejunal perforation (n=5). All pts had CD, diagnosed prior to (n=4) or concomitant with (n=10) EATL. 9 pts (64%) had RCD-II. CD30 expression ranged from 10% to 100%, nine cases having 100% positivity. EPI was high-risk in 4 pts (29%), intermediate-risk in 6 pts (43%), and low-risk in 4 pts (29%). Preliminary results by investigator assessment show an overall response rate following completion of the induction of 79% (11/14) with 64% (9/14) achieving a complete response. 3 pts had primary progressive disease (all had high-risk EPI), of which 2 died of the lymphoma. The 11 responding pts, still in response before intensification, underwent HDT-ASCT. 2 pts died of septic shock during HDT-ASCT. With a median follow-up of 2.1 years, there was no relapse and the 2-year PFS and OS for all pts were 63% and 68%, respectively. The incidence of AEs was consistent with the known safety profiles of BV-CHP regimen. Conclusions EATL-001 is the first prospective phase 2 study dedicated to EATL. BV-CHP was well tolerated and induced high response rates, allowing the majority of patients to be transplanted. This novel therapeutic approach shows promising efficacy compared to historical controls. Disclosures Sibon: Takeda: Consultancy; Roche: Consultancy; Janssen: Consultancy; Abbvie: Consultancy; iQone: Consultancy. Cartron: Roche, Celgene-BMS: Consultancy; Danofi, Gilead, Novartis, Jansen, Roche, Celgene-BMS, Abbvie, Takeda: Honoraria. Morschhauser: Incyte: Membership on an entity's Board of Directors or advisory committees; Celgene: Membership on an entity's Board of Directors or advisory committees; Gilead: Consultancy, Membership on an entity's Board of Directors or advisory committees; Chugai: Honoraria; AstraZenenca: Membership on an entity's Board of Directors or advisory committees; F. Hoffmann-La Roche Ltd: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees; AbbVie: Consultancy, Membership on an entity's Board of Directors or advisory committees; Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees; Genentech, Inc.: Consultancy; Janssen: Honoraria; Roche: Consultancy, Speakers Bureau; Epizyme: Consultancy, Membership on an entity's Board of Directors or advisory committees; BMS: Consultancy, Membership on an entity's Board of Directors or advisory committees; Genmab: Membership on an entity's Board of Directors or advisory committees; Servier: Consultancy. Hermine: Takeda: Consultancy. OffLabel Disclosure: Brentuximab vedotin is not approved in Europe for EATL.

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Malamut, Georgia, Nadine Cerf-Bensussan, and Christophe Cellier. "Deciphering the Different Types of Refractory Celiac Disease." International Journal of Celiac Disease 2, no. 1 (May 5, 2016): 4–5. http://dx.doi.org/10.12691/ijcd-2-1-2.

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Van Beurden, Yvette H., Tom Van Gils, Nienke A. Van Gils, Zain Kassam, Chris J. J. Mulder, and Nieves Aparicio-Pagés. "Serendipity in Refractory Celiac Disease: Full Recovery of Duodenal Villi and Clinical Symptoms after Fecal Microbiota Transfer." Journal of Gastrointestinal and Liver Diseases 25, no. 3 (September 1, 2016): 385–88. http://dx.doi.org/10.15403/jgld.2014.1121.253.cel.

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Treatment of refractory celiac disease type II (RCD II) and preventing the development of an enteropathy associated T-cell lymphoma in these patients is still difficult. In this case report, we describe a patient with RCD II who received fecal microbiota transfer as treatment for a recurrent Clostridium difficile infection, and remarkably showed a full recovery of duodenal villi and disappearance of celiac symptoms. This case suggests that altering the gut microbiota may hold promise in improving the clinical and histological consequences of celiac disease and/or RCD II. Abbreviations: CDI: Clostridium difficile infection; EATL : enteropathy associated T-cell lymphoma; FMT: fecal microbiota transfer; IEL: intraepithelial lymphocytes; RCD II: refractory celiac disease type II; TPN: total parenteral nutrition.

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Weber, Marko, Nina Wolf, Federica Branchi, Paul Tangermann, Alice Itzlinger, Lukas Poralla, Jan C. Preiß, et al. "Results from the German registry for refractory celiac disease." Zeitschrift für Gastroenterologie 59, no. 09 (September 2021): 944–53. http://dx.doi.org/10.1055/a-1540-7476.

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AbtractRefractory celiac disease (RCD) refers to a rare subgroup of patients with celiac disease who show clinical signs of malabsorption despite a gluten-free diet. RCD is divided into an autoimmune phenotype (RCD type I) and pre-lymphoma (RCD type II). To reflect the clinical reality in managing this disease in Germany, a national register was established based on a questionnaire developed specifically for this purpose. Between 2014 and 2020, a total of 53 patients were registered. The diagnosis of RCD was confirmed in 46 cases (87%). This included 27 patients (59%) with RCD type I and 19 patients (41%) with RCD type II. A wide range of diagnostic and therapeutic measures was used. Therapeutically, budesonide was used in 59% of the RCD patients regardless of the subtype. Nutritional therapy was used in only 5 patients (11%). Overall mortality was 26% (12 patients) with a clear dominance in patients with RCD type II (9 patients, 47%). In summary, RCD needs to become a focus of national guidelines to increase awareness, establish standards, and thus enable the treating physician to make the correct diagnosis in a timely manner. Moreover, we concluded that when treating such patients, contacting a specialized center is recommended to ensure sufficient management.

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Tack, Greetje J. "Evaluation of Cladribine treatment in refractory celiac disease type II." World Journal of Gastroenterology 17, no. 4 (2011): 506. http://dx.doi.org/10.3748/wjg.v17.i4.506.

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Verdelho Machado, Mariana. "Refractory Celiac Disease: What the Gastroenterologist Should Know." International Journal of Molecular Sciences 25, no. 19 (September 26, 2024): 10383. http://dx.doi.org/10.3390/ijms251910383.

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Fewer than 1% of patients with celiac disease (CD) will develop refractory CD (RCD). As such, most gastroenterologists might never need to manage patients with RCD. However, all gastroenterologists must be familiarized with the basic concepts of RCD and non-responsive CD (NRCD), since it can present as a severe disease with high mortality, not only due to intestinal failure, but also due to progression to enteropathy-associated T cell lymphoma (EATL) and a higher susceptibility to life-threatening infections. The diagnostic workup and differential diagnosis with other causes of gastrointestinal symptoms and villous atrophy, as well as the differentiation between type I and II RCD, are complex, and may require specialized laboratories and reference hospitals. Immunosuppression is efficient in the milder RCDI; however, the treatment of RCDII falls short, with current options probably only providing transient clinical improvement and delaying EATL development. This review summarizes the current diagnostic and therapeutic approach for patients with RCD that all doctors that manage patients with CD should know.

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Schumann, Michael, Christian Bojarski, Verena Moos, and Severin Daum. "Refractory Celiac Disease New Diagnostic Approaches and Current Treatment." International Journal of Celiac Disease 2, no. 1 (May 5, 2016): 33–37. http://dx.doi.org/10.12691/ijcd-2-1-10.

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OʼMalley, Dennis P., Margaret L. Gulley, and Peter M. Banks. "Enteropathy-Type T-cell Lymphoma: Its Relationship to Refractory Celiac Disease." Pathology Case Reviews 7, no. 3 (May 2002): 110–16. http://dx.doi.org/10.1097/01.pcr.0000015747.87813.27.

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O’Malley, Dennis P., Margaret L. Gulley, and Peter M. Banks. "Enteropathy-Type T-cell Lymphoma: Its Relationship to Refractory Celiac Disease." Pathology Case Reviews 7, no. 3 (May 2002): 110–16. http://dx.doi.org/10.1097/00132583-200205000-00006.

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Aleanzi, Mabel, Ana María Demonte, Cecilia Esper, Silvia Garcilazo, and Marta Waggener. "Celiac Disease." Clinical Chemistry 47, no. 11 (November 1, 2001): 2023–28. http://dx.doi.org/10.1093/clinchem/47.11.2023.

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Abstract Background: Selective deamidation of glutamine residues by tissue transglutaminase (tTG) turns gliadin peptides into stronger activators of T cells from celiac disease (CD) patients. We examined the possibility that these modified peptides could be more specific epitopes for circulating antibodies than are native peptides. Methods: Two native synthetic peptides and their respective modified sequences were used as antigens for ELISA assays: peptide-1, with residues 56–75 of α-type gliadin; and peptide-2, with residues 134–153 of γ-type gliadin. We examined 40 CD patients [31 not being treated with a gluten-free diet (GFD) and 9 being treated with a GFD] and 30 non-CD patients. Results: An enhanced response against deamidated peptides was observed in 4 (IgA) and 22 (IgG) of 31 untreated CD patients for peptide-1 and in 25 (IgA) and 29 (IgG) patients for peptide-2. Higher anti-gliadin antibody and anti-tTG IgA concentrations correlated with increased IgA reactivity to modified peptides. Among the nine treated CD patients, eight also displayed an improved IgG signal for the deamidated sequence. Deamidation of peptides did not increase the reactivity of non-CD sera. Conclusions: Selective deamidation specifically increases circulating antibody recognition of gliadin peptides in CD patients. This suggests that deamidated gliadin peptides are more specific CD B-cell epitopes than native peptides; this finding may be relevant for designing improved diagnostic tests.

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Haddad, Philip A., and Sai Malireddy. "Abstract 4411: Clinicopathologic determinants of survival in enteropathy-associated T-cell lymphoma (EATL): analysis of a pooled database." Cancer Research 83, no. 7_Supplement (April 4, 2023): 4411. http://dx.doi.org/10.1158/1538-7445.am2023-4411.

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Abstract Background: EATL is a rare and rapidly progressive extranodal T-cell lymphoma which arises from the intestinal intraepithelial T lymphocytes. It often occurs in the context of untreated or long-standing Celiac disease (CD) or the development of refractory sprue. EATL tends to be less responsive to conventional chemotherapy, often with poor clinical outcomes. We conducted this analysis to explore the clinicopathologic determinants of survival in this rare extranodal T-cell entity. Methods: To study the demographic characteristics, molecular and immunohistochemical signatures, therapeutic interventions, survival, and prognostic factors, we compiled a pooled database of 220 cases. Kaplan-Meier survival curves were constructed. Cox proportional-hazards model and Log-rank tests were used to assess the influence of demographic and clinicopathologic factors on overall survival (OS). Results: A total of 220 patients with confirmed EATL were identified. The median age was 60, with a peak incidence between 64 and 75. There was a slight female predominance with F:M ratio of 1.1. The jejunum was the most involved site (47%). The median OS of the whole group was 10 months. The most common presentations were abdominal pain, followed by perforation, diarrhea, obstruction, and gastrointestinal bleeding. The majority presented at stage I&II (53%). The median duration of symptoms before diagnosis was 2 months. Younger patients (p=0.02) and HLH (p=0.006) had worse OS. The time interval between diagnosing CD and EATL impacted OS, with <1 yr and >20yrs having worse outcomes (p=0.04). Combination chemotherapy and stem cell transplant (SCT) were statistically superior to no treatment, with a median OS of 11, 22, and 0.75 months respectively (p=0.002). Further analysis revealed that surgical resection imparted a survival advantage in combination with chemotherapy and SCT. When surgical resection was incorporated into the analysis, median OS amounted to 0.75, 9, 16, 8, 69 months for surgery alone, chemotherapy, surgery+chemotherapy, SCT, and surgery+SCT, respectively (p=0.003). Combinations that used anthracyclines (p=0.02) and etoposide (p=0.05) had better OS. The quality of response to treatment also seemed to impact the outcome (p=0.0001) with median OS of 9, 9, and 69 months for <PR, PR, and CR, respectively. OS was not impacted by stage, constitutional symptoms, presentation with obstruction or perforation, or CD30+. While male sex, colon and visceral involvement, and null TCR type seemed to impact OS negatively, they did not reach statistical significance. Conclusions: This study presents updated clinicopathologic data from a pooled cohort of patients with EATL. It identifies age, CD duration, HLH, treatment modalities, and quality of response as major determinants of OS in this rare disease. Citation Format: Philip A. Haddad, Sai Malireddy. Clinicopathologic determinants of survival in enteropathy-associated T-cell lymphoma (EATL): analysis of a pooled database. [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2023; Part 1 (Regular and Invited Abstracts); 2023 Apr 14-19; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2023;83(7_Suppl):Abstract nr 4411.

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Gross, Sascha, Sjoerd F. Bakker, Adriaan A. Van Bodegraven, Ingrid M. W. Van Hoogstraten, Kyra A. Gelderman, Gerd Bouma, Chris J. Mulder, B. Mary E. Von Blomberg, and Hetty J. Bontkes. "Increased IgA Glycoprotein-2 Specic Antibody Titres in Refractory Celiac Disease." Journal of Gastrointestinal and Liver Diseases 23, no. 2 (June 1, 2014): 127–33. http://dx.doi.org/10.15403/jgld.2014.1121.232.sg1.

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Background & Aims: In most cases celiac disease (CD) is successfully treated with a gluten-free diet (GFD). However, some patients become refractory to the GFD. Refractory CD (RCD) patients have an increased risk for developing enteropathy associated T-cell lymphoma and early diagnosis is therefore of importance. Currently, RCD diagnosis relies on endoscopy and adequate serological markers are lacking. Antibodies against glycoprotein-2 (GP2A) were described in Crohn's disease (CrD) and active CD but not in ulcerative colitis (UC), suggesting this is a specific marker for small intestinal lesions.Methods: Sera obtained from patients visiting our outpatient clinic for routine serological tests for diagnosis and/or follow-up of inflammatory bowel disease (n=78), active CD (n=45), GFD (n=34) and RCD (n=15) were analysed for GP2A titres.Results: Increased GP2A-IgA levels in CrD and active CD as compared to controls (p<0.001) and lack thereof in UC was confirmed. However, we could not confirm the association with small bowel localization within the CrD patient group. Within CD patients, we demonstrated a significant decrease of GP2A-IgA titres upon a GFD and increased levels in RCD patients as compared to patients on a GFD. Although GP2A-IgA was not associated with the degree of villous atrophy, GP2A-IgA levels were able to distinguish RCD patients from GFD patients (ROC AUC=0.79, p=0.002).Conclusion: Follow-up of GP2A-IgA titres in CD patients on a GFD may help to identify patients at risk for developing RCD.Abbreviations: ABSA: anti-bovine serum albumine; ASCA: anti- Saccharomyces cerevisiae antigen; AU:artificial units; AUC: area under the curve; CD: celiac disease; CrD: Crohn's disease; EATL: enteropathyassociated T-cell lymphoma; EmA: anti-endomysium antibodies; GFD: gluten free diet; GP2A: glycoprotein 2 antibodies; IEL: intraepithelial lymphocytes; RCD: refractory celiac disease; ROC: receiver operator curve; TG2A: transglutaminase-2 antibodies; UC: ulcerative colitis.

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Chibbar, R., J. Nostedt, D. Mihalicz, R. Mclean, J. deschenes, and L. A. Dieleman. "A242 SIGMOID PERFORATION: A RARE CASE OF REFRACTORY CELIAC DISEASE TYPE II." Journal of the Canadian Association of Gastroenterology 1, suppl_2 (February 2018): 354. http://dx.doi.org/10.1093/jcag/gwy009.242.

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Mandhwani, Rajesh M., Rajesh K. Wadhwa, Syed Mudassir Laeeq, Nasir Hasan Luck, Mohammad Mubarak, and Zain Majid. "Refractory coeliac disease a reality: views from Pakistan." Tropical Doctor 47, no. 1 (July 10, 2016): 51–53. http://dx.doi.org/10.1177/0049475516631710.

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Refractory coeliac disease (RCD) is described as persistence or recurrence of signs and symptoms of malabsorption with small-intestinal villous atrophy despite being on a strict gluten-free diet (GFD) for more than 12 months. RCD is a diagnosis of exclusion. There are two types of RCD, based upon the immunohistochemical features (presence of intraepithelial lymphocytes), response to treatment and prognosis. The treatment of RCD includes GFD and immunosuppressive agents. We hereby present a case of refractory celiac disease type II in a young man who later went on to develop Addisonian crisis and did not survive.

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Van Weyenberg, Stijn J. B., Chris J. J. Mulder, and Jan Hein T. M. Van Waesberghe. "Small Bowel Imaging in Celiac Disease." Digestive Diseases 33, no. 2 (2015): 252–59. http://dx.doi.org/10.1159/000369516.

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Background: Modern small bowel imaging techniques allow detailed depiction of small-intestinal abnormalities. The role of these techniques in the investigation of celiac disease is increasing, especially in patients with suspected complicated celiac disease. Key Messages: In general, there is no need for radiological small bowel imaging in uncomplicated celiac disease. It is however important that clinicians and radiologists are aware of certain specific radiological findings that may suggest celiac disease, especially since celiac disease is often not considered in adult patients, and small bowel radiology may be performed before specific tests for celiac disease. Radiological abnormalities can be observed with both conventional small bowel radiology studies, like small bowel follow-through or double-contrast small bowel enteroclysis, and newer modalities, like computed tomography or magnetic resonance enterography or enteroclysis. These signs include a decreased number of jejunal folds, an increased number of ileal folds, small bowel dilatation, wall thickening and intussusception. Extraintestinal abnormalities include mesenteric lymphadenopathy, vascular changes and splenic atrophy. Abnormalities congruent with refractory celiac disease type II include a severe decrease in jejunal folds, infiltration of the mesenteric fat and thickening of the small bowel wall. Additionally, a severely decreased splenic volume may indicate complicated celiac disease. Malignant complications of celiac disease, such as enteropathy-associated T-cell lymphoma and small-intestinal adenocarcinoma, can be reliably investigated with cross-sectional enteroclysis techniques. Conclusions: Small bowel imaging and especially cross-sectional enteroclysis techniques are important extensions to the diagnostic workup of clinicians involved in the care of patients with celiac disease, especially those with suspected complicated disease.

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Olteanu, Dan, Alexandru Diaconescu, Radu Voiosu, Andrei Voiosu, and Cristina Olariu. "PRESENT DATA IN THE DIAGNOSIS AND TREATMENT OF COELIAC DISEASE (2)." Romanian Medical Journal 64, no. 1 (March 31, 2017): 25–33. http://dx.doi.org/10.37897/rmj.2017.1.5.

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Coeliac disease incidence rised during the last 50 years and represents a concern by diagnostic problems and costs. The recent data regarding etiology, pathogeny, comparative diagnostic value of serology and small intestinal biopsy are summarised. The new data about refractory celiac disease to gluten free diet and therapeutic perspectives are also presented (glutenases, larazotide acetate, genetic alteration of cereals, tissulary transglutaminase inhibitors etc).

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Jamma, Shailaja, Daniel A. Leffler, Melinda Dennis, Robert M. Najarian, Detlef B. Schuppan, Sunil Sheth, and Ciaran P. Kelly. "Small Intestinal Release Mesalamine for the Treatment of Refractory Celiac Disease Type I." Journal of Clinical Gastroenterology 45, no. 1 (January 2011): 30–33. http://dx.doi.org/10.1097/mcg.0b013e3181f42401.

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Tack, G. J., M. J. Wondergem, A. Al-Toma, W. H. M. Verbeek, A. Schmittel, M. V. Machado, F. Perri, et al. "Auto-SCT in refractory celiac disease type II patients unresponsive to cladribine therapy." Bone Marrow Transplantation 46, no. 6 (September 6, 2010): 840–46. http://dx.doi.org/10.1038/bmt.2010.199.

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Bănică, Diana, Ramona Frăţilă, Alexandra Sima, Adrian Vlad, and Romulus Timar. "Autoimmune polyglandular syndrome type 2 - a case report." Romanian Journal of Diabetes Nutrition and Metabolic Diseases 21, no. 1 (March 1, 2014): 25–28. http://dx.doi.org/10.2478/rjdnmd-2014-0004.

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Abstract Autoimmune polyglandular syndromes are characterized by the association of two or more autoimmune diseases. They are classified into two major subtypes, each having its own characteristics. The autoimmune polyglandular syndrome type 2 is defined by the presence of at least two of the following diseases: Addison’s disease, type 1 diabetes mellitus and thyroid autoimmune disease. Other autoimmune diseases belonging to the autoimmune polyglandular syndrome type 2 are: primary hypogonadism, myasthenia gravis, celiac disease, pernicious anemia, alopecia, vitiligo. We are going to present the case of a patient, aged 40, with diabetes mellitus (probably latent autoimmune diabetes of the adult), chronic autoimmune thyroiditis and celiac disease.

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Al-toma, Abdulbaqi, Otto J. Visser, Hyacintha M. van Roessel, B. Mary E. von Blomberg, Wieke H. M. Verbeek, Petra E. T. Scholten, Gert J. Ossenkoppele, Peter C. Huijgens, and Chris J. J. Mulder. "Autologous hematopoietic stem cell transplantation in refractory celiac disease with aberrant T cells." Blood 109, no. 5 (October 26, 2006): 2243–49. http://dx.doi.org/10.1182/blood-2006-08-042820.

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Abstract Autologous hematopoietic stem cell transplantation (ASCT) is an increasingly accepted treatment for refractory autoimmune diseases. Refractory celiac disease with aberrant T cells (RCD type II) is unresponsive to available therapies and carries a high risk of transition into enteropathy associated T-cell lymphoma (EATL). This study reports on the feasibility, safety, and efficacy of ASCT in patients with RCD type II. Thirteen patients with RCD type II were evaluated. Seven patients (4 men, 3 women, mean age 61.5 years [range, 51-69 years]) underwent transplantation. After conditioning with fludarabine and melphalan, ASCT was performed. Patients were monitored for response, adverse effects, and hematopoietic reconstitution. All 7 patients completed the mobilization and leukapheresis procedures successfully and subsequently underwent conditioning and transplantation. Engraftment occurred in all patients. No major nonhematologic toxicity or transplantation-related mortality was observed. There was a significant reduction in the aberrant T cells in duodenal biopsies associated with improvement in clinical well-being and normalization of hematologic and biochemical markers (mean follow-up, 15.5 months; range, 7-30 months). One patient died 8 months after transplantation from progressive neuroceliac disease. These preliminary results showed that high-dose chemotherapy followed by ASCT seems feasible and safe and might result in long-term improvement of patients with RCD type II whose condition did not respond promptly to available drugs.

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Nijeboer, Petula, Roy L. J. van Wanrooij, Greetje J. Tack, Chris J. J. Mulder, and Gerd Bouma. "Update on the Diagnosis and Management of Refractory Coeliac Disease." Gastroenterology Research and Practice 2013 (2013): 1–9. http://dx.doi.org/10.1155/2013/518483.

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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.

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Hastier, Audrey, Stéphanie Patouraux, Clémence M. Canivet, Cynthia Lebeaupin, Albert Tran, and Rodolphe Anty. "Nonalcoholic steatohepatitis cirrhosis and type 1 refractory celiac disease: More than a fortuitous association?" Clinics and Research in Hepatology and Gastroenterology 40, no. 1 (February 2016): 4–5. http://dx.doi.org/10.1016/j.clinre.2015.09.006.

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Mukewar, Saurabh S., Ayush Sharma, Alberto Rubio-Tapia, and Joseph A. Murray. "Mo1275 Open Capsule Budesonide for Refractory Celiac Disease: A Single Center Study." Gastroenterology 150, no. 4 (April 2016): S685—S686. http://dx.doi.org/10.1016/s0016-5085(16)32333-2.

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Ghazizadeh Esslami, Golnaz, Bahar Allahverdi, Reza Shervin Badv, Morteza Heidari, Nahid Khosroshahi, Hosein Shabani-Mirzaee, and Kambiz Eftekhari. "Clinical and Paraclinical Screening for Celiac Disease in Children with Intractable Epilepsy." Neurology Research International 2021 (April 22, 2021): 1–4. http://dx.doi.org/10.1155/2021/1639745.

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Background. Celiac disease is the inflammatory entropy caused by hypersensitivity to gluten, which occurs in susceptible individuals. Some studies have suggested a link between celiac disease and epilepsy in children. Our aim was to screen for clinical and paraclinical features of celiac disease in children with intractable epilepsy. Methods. This was a cross-sectional study. Children aged 2 to 18 years with refractory epilepsy that referred to the pediatric neurology clinic within one year (2018–2019) were enrolled. Demographic and clinical characteristics of patients, especially clinical manifestations of celiac disease, were recorded in a questionnaire. A venous blood sample was sent to determine the total IgA, anti-tTG (IgA), and anti-endomysial antibody (IgA). Endoscopy was performed in cases where the celiac serological test was positive. Results. Seventy children with idiopathic drug-resistant epilepsy (44 boys) were evaluated. The height-for-age index was 49.2% and the weight-for-age index was 38.2% less than normal. Constipation (48.6%), anorexia (25.7%), and abdominal pain (21.4%) were the most common gastrointestinal symptoms. Celiac serological tests were negative in all children. Therefore, endoscopy and bowel biopsy were not performed in any case. Conclusion. Celiac disease was not found in any patient with intractable epilepsy. Gastrointestinal symptoms and growth disorders in this group may be related to the underlying disease or medications and not to celiac disease.

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Malamut, Georgia, Pauline Afchain, Virginie Verkarre, Thierry Lecomte, Aurélien Amiot, Diane Damotte, Yoram Bouhnik, et al. "Presentation and Long-Term Follow-up of Refractory Celiac Disease: Comparison of Type I With Type II." Gastroenterology 136, no. 1 (January 2009): 81–90. http://dx.doi.org/10.1053/j.gastro.2008.09.069.

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Raithel, Martin, Michael Weidenhiller, Dieter Schwab, Sandra Winterkamp, and Eckhart Georg Hahn. "Identification of type I allergic reactions to nutritive antigens in patients with refractory celiac disease." Gastroenterology 118, no. 4 (April 2000): A1132. http://dx.doi.org/10.1016/s0016-5085(00)80339-x.

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Fernandes, Alexandra, Ana Margarida Ferreira, Rosa Ferreira, Sofia Mendes, Cláudia Agostinho, Nuno Almeida, Pedro Figueiredo, et al. "Refractory Celiac Disease Type II: A Case Report that Demonstrates the Diagnostic and Therapeutic Challenges." GE Portuguese Journal of Gastroenterology 23, no. 2 (March 2016): 106–12. http://dx.doi.org/10.1016/j.jpge.2015.08.001.

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Soderquist, Craig R., Susan Hsiao, Mahesh M. Mansukhani, Bachir Alobeid, Peter H. Green, and Govind Bhagat. "Refractory celiac disease type II: An atypical case highlighting limitations of the current classification system." Hematological Oncology 38, no. 3 (February 19, 2020): 399–405. http://dx.doi.org/10.1002/hon.2720.

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Battista, Natalia, Antonio Di Sabatino, Monia Di Tommaso, Paolo Biancheri, Cinzia Rapino, Paolo Giuffrida, Cinzia Papadia, et al. "Altered Expression of Type-1 and Type-2 Cannabinoid Receptors in Celiac Disease." PLoS ONE 8, no. 4 (April 19, 2013): e62078. http://dx.doi.org/10.1371/journal.pone.0062078.

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Damen, M. J., A. van der Meer, N. C. Voermans, and A. A. Tieleman. "Graves’ disease and celiac disease in a patient with myotonic dystrophy type 2." Neuromuscular Disorders 28, no. 10 (October 2018): 878–80. http://dx.doi.org/10.1016/j.nmd.2018.07.003.

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Balasa, Adriana Luminita, Cristina Maria Mihai, Tatiana Chisnoiu, and Corina Elena Frecus. "Atypical presentations of celiac disease." ARS Medica Tomitana 22, no. 3 (August 1, 2016): 181–85. http://dx.doi.org/10.1515/arsm-2016-0030.

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Abstract In this study we evaluated the association of celiac disease in 81 children with autoimmune disease and genetic syndromes over a two years periods (January 2014 to July 2016) in Pediatric Clinic in Constanta. Because the extraintestinal symptoms are an atypical presentation of celiac disease we determined in these children the presence of celiac disease antibodies: Anti-tissue Transglutaminase Antibody IgA and IgA total serum level as a screening method followeds in selective cases by Anti-tissue Transglutaminase Antibody IgG, anti-endomysial antibodies, deamidated gliadin antibodies IgA and IgG and intestinal biopsia. In our study 8 patients had been diagnosed with celiac disease with extraintestinal symptoms, of which 4 with type 1 diabetes, 1 patient with ataxia, 2 patients with dermatitis herpetiformis and 1 patient with Down syndrome that associate also autoimmune thyroiditis, alopecia areata, enamel hypoplasia.

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Omar, Mahmud, Mohammad Omar, Salih Nassar, Adi Lahat, and Kassem Sharif. "Limited Clinical Impact of Genetic Associations between Celiac Disease and Type 2 Inflammatory Diseases: Insights from Mendelian Randomization." Biomedicines 12, no. 7 (June 27, 2024): 1429. http://dx.doi.org/10.3390/biomedicines12071429.

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Background: Celiac disease, a gluten-triggered autoimmune disorder, is known for its systemic inflammatory effects. Its genetic associations with type 2 inflammatory diseases like asthma, allergic rhinitis, and atopic dermatitis remain unclear, prompting this study to explore their potential genetic interplay. Methods: Utilizing two-sample Mendelian randomization (TSMR), we examined the genetic associations using 15 genetic instruments from GWAS datasets. Our analysis focused on celiac disease and its relation to asthma, allergic rhinitis, atopic dermatitis, and IgE-mediated food allergies. A power analysis was conducted to determine the study’s detection capabilities, and odds ratios (ORs) with 95% confidence intervals (CIs) were calculated using various MR methods. Results: Our Mendelian randomization analysis identified statistically significant genetic associations between celiac disease and several type 2 inflammatory diseases, although these were practically insignificant. Specifically, celiac disease was associated with a slight increase in the risk of atopic dermatitis (OR = 1.037) and a minor protective effect against asthma (OR = 0.97). The link with allergic rhinitis was statistically detectable (OR = 1.002) but practically negligible. Despite robust statistical confirmation through various sensitivity analyses, all observed effects remained within the range of practical equivalence (ROPE). Conclusions: Our study identifies potential genetic associations between celiac disease and certain type 2 inflammatory diseases. However, these associations, predominantly within the ROPE range, suggest only limited clinical implications. These findings highlight the need for cautious interpretation and indicate that further exploration for clinical applications may not be warranted at this stage.

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Yousaf, Muhammad Iftikhar, Humaira Mubeen Afzal, Usama Faizan, Muhammad Haroon Yousaf, and Muhammad Asif. "Frequency of Celiac Disease Diagnosed on Endoscopic Duodenal Biopsy in Patients of Functional Dyspepsia." Pakistan Journal of Medical and Health Sciences 16, no. 4 (April 30, 2022): 964–66. http://dx.doi.org/10.53350/pjmhs22164964.

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Objective: To determine the frequency of celiac disease diagnosed on endoscopic duodenal biopsy in patients presenting with functional dyspepsia. Methods: This was a cross-sectional study was conducted at the Department of Gastroenterology and Hepatology, Shalamar Medical and Dental College, Lahore. A total 160 patients of either gender, aged 20-70 years and diagnosed with functional dyspepsia were included. The study was conducted during a period of six months from September 2021 to February 2022. The informed consent of patients was obtained. All the basic demographic information of each patient was noted and all the patients underwent endoscopic duodenal biopsies and specimens were sent to the department of histopathology for the histopathological assessment of celiac disease. The collected data were analyzed statistically by using SPSS version 12. Results: Average age of male and female patients was 45.61±14.41 and 39.83±14.91 years. Among 160 patients 60(37%) were male and 100(63%) were females. There were 90(56.3%) patients who had dysmotility like dyspepsia, 54(33.8%) patients diagnosed with an ulcer like dyspepsia and 16(10%) patients were having unspecified dyspepsia. According to the Marsh classification, 140 patients were diagnosed with type-0, 7 patients with Type-1, 2 patients with type-2, 9 patients with type-3a and 2 patients with type-3b. Celiac disease was diagnosed with the help of Modified Marsh classification. Among 160 patients 13(8%) were diagnosed with celiac disease. Conclusion: The results of the current study show a high frequency of celiac disease in patients with functional dyspepsia. Keeping this fact in mind, silent/subclinical CD should be kept in mind as a cause of functional dyspepsia during clinical activities Keywords: Celiac disease, Endoscopic duodenal biopsy, Functional dyspepsia

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Pastré, Jean, Karine Juvin, Georgia Malamut, Coralie Derrieux, Christophe Cellier, and Dominique Israël-Biet. "Phenotypically aberrant clonal T cells in the lungs of patients with type II refractory celiac disease." Blood 123, no. 23 (June 5, 2014): 3674–75. http://dx.doi.org/10.1182/blood-2014-04-566513.

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OLAUSSEN, R., A. LOVIK, S. TOLLEFSEN, P. ANDRESEN, M. VATN, T. DELANGE, J. BRATLIE, P. BRANDTZAEG, I. FARSTAD, and K. LUNDIN. "Effect of Elemental Diet on Mucosal Immunopathology and Clinical Symptoms in Type 1 Refractory Celiac Disease." Clinical Gastroenterology and Hepatology 3, no. 9 (September 2005): 875–85. http://dx.doi.org/10.1016/s1542-3565(05)00295-8.

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Journal articles on the topic 'Refractory celiac disease type 2'

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