Is Refractory Celiac Disease More Severe in Old Europe?

Malamut, Georgia; Cellier, Christophe

doi:10.1038/ajg.2011.105

Cited by 26 publications

(23 citation statements)

References 28 publications

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“…Although it has been known for many years that the increased mortality in CD is due to its feared complications [23], the literature reports very few epidemiological data on these complications [8,[15][16][17][18][19]. This lack of data can undoubtedly be attributed both to the significant selection biases in these series of patients and to the difficulties caused by the lack of uniform diagnostic criteria for the various forms of CCD [15,16].…”

Section: Discussionmentioning

confidence: 99%

“…This lack of data can undoubtedly be attributed both to the significant selection biases in these series of patients and to the difficulties caused by the lack of uniform diagnostic criteria for the various forms of CCD [15,16].…”

Section: Discussionmentioning

confidence: 99%

“…The first papers on the molecular diagnosis of complicated CD (CCD) were published more than 10 years ago [14], and since then several papers were published on this subject [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]. Despite these achievements, the literature still provides only minor and insufficient indications on the incidence of these conditions in patients with CD [8,[15][16][17][18][19].…”

Section: Introductionmentioning

confidence: 99%

“…Despite these achievements, the literature still provides only minor and insufficient indications on the incidence of these conditions in patients with CD [8,[15][16][17][18][19]. The reason for this lack of data can probably be attributed to the fact that most patients with CCD are sent to large referral centres.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Low incidence but poor prognosis of complicated coeliac disease: A retrospective multicentre study

Biagi

Gobbi

Marchese

et al. 2014

Digestive and Liver Disease

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Low incidence but poor prognosis of complicated coeliac disease: A retrospective multicentre study

Biagi

Gobbi

Marchese

et al. 2014

Digestive and Liver Disease

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“…This was obviously because of the lack of consensus on the definition of nonresponsiveness. 4 In the last few years, some evidence has suggested that the prevalence of RCD is low, ranging from 0.6% to 1.5%. 3,5,6 Although the presence of RCD in CD is probably to be approximately 1%, the prevalence of RCD2 among patients with RCD is far from being ascertained.…”

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confidence: 99%

How I treat enteropathy-associated T-cell lymphoma

Sabatino

Biagi

Gobbi

et al. 2012

Blood

104

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Enteropathy-associated T-cell lymphoma (EATL) is a complication of celiac disease (CD). This tumor derives from the neoplastic transformation of aberrant intraepithelial T lymphocytes emerging in celiac patients unresponsive to a gluten-free diet. Poor adherence to a gluten-free diet, HLA-DQ2 homozygosity, and late diagnosis of CD are recognized as risk factors for malignant evolution of CD. Recurrence of diarrhea, unexplained weight loss, abdominal pain, fever, and night sweating should alert physicians to this complication. The suspicion of EATL should lead to an extensive diagnostic workup in which magnetic resonance enteroclysis, positron emission tomography scan, and histologic identification of lesions represent the best options. Treatment includes high-dose chemotherapy preceded by surgical resection and followed by autologous stem cell transplantation, although biologic therapies seem to be promising. Strict adherence to a gluten-free diet remains the only way to prevent EATL. (Blood. 2012;119(11): 2458-2468) IntroductionCeliac disease (CD) is a chronic gluten-sensitive enteropathy characterized by a high prevalence in the general population and an increased mortality. 1,2 It is well known that the increased mortality is mainly the result of the complications of CD itself, represented by refractory CD (RCD) and enteropathy-associated T-cell lymphoma (EATL). 2 RCD is a form of CD that does not respond histologically to at least 12 months of a strict gluten-free diet (GFD). 3 RCD can also evolve in patients who initially responded normally to a GFD and who are still maintaining a strict GFD. On the basis of the intraepithelial lymphocyte (IEL) population, RCD is further classified into type 1 and type 2. RCD type 1 (RCD1) is characterized by persisting villous atrophy despite a strict GFD associated with increased but still phenotypically normal IELs. Conversely, a clonal expansion of abnormal IELs lacking surface CD3, CD8, and T-cell receptor (TCR) markers but expressing intracellular CD3 indicates RCD type 2 (RCD2), a condition that frequently evolves into EATL, the most serious complication of CD. 4 In the past, up to 20% of celiac patients were considered to be affected by a nonresponsive form of CD. This was obviously because of the lack of consensus on the definition of nonresponsiveness. 4 In the last few years, some evidence has suggested that the prevalence of RCD is low, ranging from 0.6% to 1.5%. 3,5,6 Although the presence of RCD in CD is probably to be approximately 1%, the prevalence of RCD2 among patients with RCD is far from being ascertained. Probably because of not only the great differences in the diagnostic criteria, 4 but also referral bias, different papers report very different results, which vary from 15% to 75%. 7-9 Finally, although the incidence of EATL was reported to be rare in the general population (1 per million person-years), 10,11 it was shown that it occurs in 60% to 80% of patients with RCD2 within 5 years. [7][8][9]12 The description of EATL arising in patients with RCD...

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Increasing incidence of enteropathy‐associated T‐cell lymphoma in the United States, 1973‐2008

Sharaiha

Lebwohl

Reimers³

et al. 2011

Cancer

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Introduction Enteropathy-associated T cell lymphoma (EATL) is a rare lymphoma subtype that is strongly associated with celiac disease (CD), an autoimmune disease triggered by the ingestion of gluten. Since CD rates are increasing in the U.S., we sought to determine whether the incidence rates of EATL are increasing as well. Method We identified primary, pathologically confirmed lymphoma cases from the SEER database registries from 1973 to 2008. To ensure capture of all cases of EATL, we included the following lymphoma subtypes, limited to the small bowel: non-Hodgkin lymphoma (NOS) T-cell, Peripheral T-cell lymphoma (NOS), and enteropathy type T-cell lymphoma, and calculated their age and sex-adjusted incidence rates over time. Survival was estimated using Kaplan-Meier curves. Results We identified 161 small bowel lymphomas diagnosed between 1973 and 2008. The overall age and sex-adjusted annual incidence for all bowel lymphomas was 0.016 per 100,000, which increased over the study period from 0.006 to 0.024 per 100,000. These tumors were most common in males (age-adjusted incidence rate= 0.021 per 100,000) with the highest incidence rate in Hispanics (age-adjusted incidence rate =0.033 per 100,000). The median overall survival was 7 months. There was no difference in survival by race/ethnicity (p=0.09), or gender (P= 0.06). Conclusion Our results indicate a significant increase in the incidence of EATL in the U.S., which could reflect the increasing seroprevalence of CD and better recognition of rare types of T-cell lymphomas. The incidence may continue to rise given the large ratio of undiagnosed-to-diagnosed individuals with CD in the U.S.

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Is Refractory Celiac Disease More Severe in Old Europe?

Cited by 26 publications

References 28 publications

Low incidence but poor prognosis of complicated coeliac disease: A retrospective multicentre study

Low incidence but poor prognosis of complicated coeliac disease: A retrospective multicentre study

How I treat enteropathy-associated T-cell lymphoma

Increasing incidence of enteropathy‐associated T‐cell lymphoma in the United States, 1973‐2008

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