High rates of complications and substantial mortality in both types of refractory sprue

Abstract

RS comprises a very heterogenous group of patients with long-term survival seen even in single patients with RS type II. Overall, survival is shorter in RS type II in comparison with RS type I. Patients with RS type I, however, show similar rates of disease-related complications as well as substantial mortality.

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

“…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].…”

Is Refractory Celiac Disease More Severe in Old Europe?

“…Th ree recent European-published series of RCD patients reported high percentages of RCDII patients (28 -75 % ) among RCD patients and highlighted their poor outcome, with less than half of the patients still alive 5 years aft er the diagnosis ( 6,12,13 ). In the present issue, Roshan et al ( 14 ) ( Am J Gastroenterol 2011) report 34 RCD patients in a cohort of 844 celiac patients.…”

“…In contrast, diagnosis of RCDII in North American studies was more oft en based on immunohistochemistry alone (Roshan and colleagues ( 17 )) potentially combined to PCR or Southern analysis, but FACs analysis of small bowel IEL was lacking. Similarly, the diff erences in frequency of RCDII observed in Europe between Germany (28 % ) or Netherlands (54 % ) and France (75 % ) may result from the recruitment types and also from the delayed acquisition of FACs analysis of isolated IEL by German and Dutch groups, respectively (6,12,13).…”