Clinical Staging and Survival in Refractory Celiac Disease: A Single Center Experience

Abstract

Background & Aims Refractory celiac disease (RCD) occurs when both symptoms and intestinal damage persist or recur despite strict adherence to a gluten-free diet. In RCD, the immunophenotype of intraepithelial lymphocytes may be normal and polyclonal (RCD I) or abnormal and monoclonal (RCD II). The aim is to describe the clinical characteristics, treatment, and long-term outcome in a large single-center cohort of patients with RCD. Methods We compared the clinical characteristics and outcome in 57 patients with RCD: 42 with RCD I and 15 with RCD II. Results The overall 5-year cumulative survival is 70, 80 and 45 percent in the entire cohort, RCD I, and RCD II respectively (p = 0.07, among subtypes). The drop in survival was more evident during the first two years after the diagnosis. Fifteen (26%) patients died (n=8 with RCD I and n=7 with RCD II). The refractory state itself and enteropathy-associated T-cell lymphoma (EATL) were the most common causes of death, respectively. A new staging system is proposed based on the cumulative effect of five prognostic factors investigated at the time of the refractory state diagnosis: for patients in stages I, II, and III, the 5-year cumulative survival rate was 96, 71, and 19 percent, respectively (p<0.0001). Conclusions RCD is associated with high mortality with RCD II having an especially poor prognosis because of the development of EATL. A new staging model is proposed that may improve the precision of prognosis in patients with RCD.

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

“…Although we took into account only coeliac patients directly diagnosed in our centres and excluded those patients diagnosed elsewhere and then referred to us, our results could overestimate the risk of complications. Despite this, our results show that the complications of CD are rare but that they can occur even many years after the diagnosis of CD and confirm that these complications have a high mortality rate [2,[8][9][10][11][12][13]. Since we did not record any death from other causes among either cases or controls (this means none of them died from cardiovascular disease, infections, any type of cancer, respiratory disease etc.…”

“…Among all the remaining patients, those whose malabsorption symptoms persisted despite at least 12 months of a gluten-free diet and in whom a diagnosis of complicated CD was made were selected. More specifically, the diagnosis of RCD2 was based on a flat duodenal mucosa not responding to 12 months on a gluten-free diet 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 gluten-free diet but without the diagnostic criteria for RCD2; finally, the diagnoses of EATL, ABL, UJI, and SBC were based on morphological criteria [9][10][11][12]. For all these patients, the following information was collected: sex, date of birth, date of diagnosis of CD, date of the last examination at the centre, date of diagnosis of complications of CD, if applicable, and date of death, if applicable.…”

“…The funding source had no role.the prognosis [3][4][5][6][7]. In particular, the five-year survival rate is between 80% and 96% in patients with RCD1, between 40% and 58% in patients with RCD2 and drops to less than 20% in patients with CD complicated by EATL [8][9][10][11][12][13].…”

“…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].…”

Olmesartan-associated enteropathy: results of a national survey

“…Clinical, biological and histological manifestations All 32 patients (100%) had diarrhoea, with a median number of 8 liquid stools per day [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. Twenty-four patients (75%) had abdominal pain, which was rarely severe (n = 3).…”