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

Biagi, Federico; Gobbi, Paolo G.; Marchese, A.; Borsotti, Edoardo; Zingone, Fabiana; Ciacci, Carolina; Volta, Umberto; Caio, Giacomo; Carroccio, Antonio; Ambrosiano, Giuseppe; Mansueto, Pasquale

doi:10.1016/j.dld.2013.10.010

Cited by 59 publications

(49 citation statements)

References 28 publications

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“…Two of our patients had CD. The association between different neoplasms and CD is well established, although the incidence of complicated CD is low . Patients have an increased risk of developing malignancy in relation to the age at diagnosis of CD.…”

Section: Discussionmentioning

confidence: 99%

Medullary carcinoma of the small bowel

et al. 2016

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Section: Discussionmentioning

confidence: 99%

Medullary carcinoma of the small bowel

et al. 2016

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“…This suggests the importance of serial IEL phenotyping and TCR clonality analysis also in patients with adult AE to early detect those at risk to develop an overt lymphoma. This is a crucial point if considering that in face of the rarity of the disorder, with a total of 35 cases reported so far, this is the second described patient with an EATL‐like lymphoma, thus meaning a rate of 6%, that is a huge percentage in comparison with the prevalence of complications among adult celiac patients (0.49%) . Thus, the identification of precursor cells from which T‐cell lymphoma arises in this specific clinical setting and a better understanding of the role of CD5 form an important area of research in the near future.…”

Section: Resultsmentioning

confidence: 99%

Intestinal T‐cell lymphoma with enteropathy‐associated T‐cell lymphoma‐like features arising in the setting of adult autoimmune enteropathy

Ciccocioppo

Croci

Biagi

et al. 2018

Hematological Oncology

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Enteropathy-associated T-cell lymphoma is regarded as a dismal, late complication of coeliac disease, though a single case of T-cell lymphoma with such features arising in the setting of autoimmune enteropathy of the adult has been reported to date. We aim to describe the case of a 41-year-old woman complaining of severe malabsorption syndrome, who was diagnosed with autoimmune enteropathy based on the presence of flat intestinal mucosa unresponsive to any dietary restriction and positivity for enterocyte autoantibodies. Steroid therapy led to a complete recovery of both mucosal and clinical findings over 12 years, when disease relapse was accompanied by the appearance of monoclonal rearrangement of T-cell receptor-γ and peculiar T-cell phenotypic abnormalities, leading to a rapid transition to an overt T-cell lymphoma with features of the enteropathy-associated subtype. Despite intensive treatment, the patient developed cerebral metastasis and died 9 months later. Our case enhances the concept of enteropathy-associated T-cell lymphoma as a disease that may arise in the setting of enteropathies other than coeliac disease, thus representing a heterogeneous entity. Moreover, our observations support the need of a close follow-up of these patients, coupled with comprehensive characterization of mucosal biopsies.

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“…This is, however, very difficult to organise. Since CCD develops in less than 1% of patients [4-6,8], then to enrol at least 100 patients with CCD we would have to diagnose at least 10000 new adult patients with CD, follow them for several years before identifying those that will develop a complication and then study their clinical evolution and survival.…”

Section: Discussionmentioning

confidence: 99%

A multicentre case control study on complicated coeliac disease: two different patterns of natural history, two different prognoses

et al. 2014

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BackgroundCoeliac disease is a common enteropathy characterized by an increased mortality mainly due to its complications. The natural history of complicated coeliac disease is characterised by two different types of course: patients with a new diagnosis of coeliac disease that do not improve despite a strict gluten-free diet (type A cases) and previously diagnosed coeliac patients that initially improved on a gluten-free diet but then relapsed despite a strict diet (type B cases). Our aim was to study the prognosis and survival of A and B cases.MethodsClinical and laboratory data from coeliac patients who later developed complications (A and B cases) and sex- and age-matched coeliac patients who normally responded to a gluten-free diet (controls) were collected among 11 Italian centres.Results87 cases and 136 controls were enrolled. Complications tended to occur rapidly after the diagnosis of coeliac disease and cumulative survival dropped in the first months after diagnosis of complicated coeliac disease. Thirty-seven cases died (30/59 in group A, 7/28 in group B). Type B cases presented an increased survival rate compared to A cases.ConclusionsComplicated coeliac disease is an extremely serious condition with a high mortality and a short survival. Survival depends on the type of natural history.

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

Cited by 59 publications

References 28 publications

Medullary carcinoma of the small bowel

Medullary carcinoma of the small bowel

Intestinal T‐cell lymphoma with enteropathy‐associated T‐cell lymphoma‐like features arising in the setting of adult autoimmune enteropathy

A multicentre case control study on complicated coeliac disease: two different patterns of natural history, two different prognoses

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