Refractory sprue, coeliac disease, and enteropathy-associated T-cell lymphoma

Collagenous sprue is not always associated with dismal outcomes: a clinicopathological study of 19 patients

“…These cases did not meet the criteria for refractory celiac disease type II. 5 A dominant clone was detected in the small bowel biopsy in only one case. The intraepithelial lymphocytes (CD3 þ CD8 þ ) lacked an aberrant phenotype by immunohistochemical staining; however, as flow cytometry was not performed in this case, surface CD3 expression could not be evaluated.…”

“…11 Still others have based the diagnosis of collagenous sprue on the presence of 410 mm thickness of the subepithelial collagen band, using criteria proposed for the diagnosis of collagenous colitis. 5 Morphometric analysis of the subepithelial collagen layer in small bowel biopsies from patients with active celiac disease, or for that matter other small intestinal diseases, has not been described. Hence, there is a lack of consensus as to what represents a 'significant' increase in subepithelial collagen, hampering the promulgation of histological criteria required for diagnosing collagenous sprue.…”

“…[3][4][5][6][7][8][9][10][11][12][13][14][15][16] The occurrence of collagenous sprue has been reported in individuals with celiac disease, tropical sprue, milk intolerance, myotonic dystrophy, malignancy-related paraneoplastic syndrome, and common variable immunodeficiency. 4,5,10,11,14,17,18 These studies have suggested that subepithelial collagen deposition might be a non-specific feature.…”

“…5,11 A few studies, however, have reported a good response to treatment and favorable outcomes. 4,[8][9][10] The discrepant findings have been attributed to inclusion of cases with mild alterations of the subepithelial collagen table in some studies, but currently there are no well-defined histological criteria for diagnosing collagenous sprue.…”

Is Refractory Celiac Disease More Severe in Old Europe?

“…Diagnosis of this condition defi ned as refractory celiac disease (RCD) is made aft er exclusion of other small bowel diseases, such as autoimmune enteropathy ( 1 ), tropical sprue (2), or common variable immunodefi ciency ( 3 ). RCD has been subdivided into two subgroups: type I RCD (RCDI), defi ned by persisting villous atrophy despite a strict GFD associated with an increased number of intraepithelial lymphocytes (IELs) bearing a normal phenotype with surface CD3 and CD8 expression, and RCD of type II (RCDII) characterized by clonal expansion of abnormal IEL lacking surface markers CD3, CD8, and T-cell receptor (CD3s − , CD8s − , and TCR − ) and preserved expression of intracellular CD3 ( 4,5 ). Besides the abnormal phenotype of IEL, RCDII has a more severe clinical presentation and is frequently associated with ulcerative jejunitis responsible for protein loss enteropathy.…”