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

Federico Biagi,Gino Roberto Corazza,Giacomo Caio,Rachele Ciccocioppo,Pasquale Mansueto,Umberto Volta,Fabiana Zingone,Alida Andrealli,Giuseppe Ambrosiano,Marco Astegiano,Carolina Ciacci,Antonio Carroccio,Matteo Neri,Antonio Gasbarrini,Francesca Ferretti,Anna D’Odorico,Sergio Segato,Annalisa Schiepatti,Alberto Meggio,A Marchese ,I De Vitis ,G De Pretis ,Paolo G Gobbi ,A.c Piscaglia

doi:10.1186/1471-230x-14-139

Abstract

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.

Highlights

Coeliac disease is a common enteropathy characterized by an increased mortality mainly due to its complications
Its prognosis is excellent in the great majority of cases, some of these patients may develop serious complications, such as refractory Coeliac disease (CD) type 1 (RCD1), refractory CD type 2 (RCD2), ulcerative jejuno-ileitis (UJI), enteropathy associated T cell lymphoma (EATL), abdominal B cell lymphoma (ABL), small bowel carcinoma (SBC) and collagenous sprue (CS) [2,3,4,5,6]
Diagnoses of EATL, ABL, SBC, and CS were based on morphological criteria; diagnosis of RCD2 was based on a flat duodenal mucosa not responding to 12 months on a gluten-free diet (GFD) and evidence of an aberrant intraepithelial lymphocyte population and/or gamma-chain T-cell monoclonal rearrangement; diagnosis of RCD1 was based on a flat duodenal mucosa not responding to 12 months on a GFD but without the diagnostic criteria for RCD2; diagnosis of UJI was based on the demonstration of small bowel ulcers but without the diagnostic criteria for EATL and RCD2 [3,9,10,11,12]

Summary

Introduction

Coeliac disease is a common enteropathy characterized by an increased mortality mainly due to its complications. Since treatment is hardly effective [7], There are various factors correlated with the risk of developing CD complications It has been known for some time that the main risk factor is poor compliance with a gluten-free diet (GFD) and, in the last few years, it has emerged that the clinical form of CD at diagnosis (classic/major, non-classic/minor, asymptomatic/silent [16]), the diagnostic delay of CD, age at diagnosis of CD, and homozygosity for HLA-DQ2 correlate with the risk of developing complications and with mortality [17,18,19,20,21,22,23]. The prevalence and, above all, the prognosis of these two different forms of onset of CCD have not yet been ascertained

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