In the latest research on collagenous sprue outcomes, (8) where 19 cases were studied on multiple clinical, histopathologic, and molecular aspects, 8 patients (42%) responded to the gluten-free diet 10 patients (53%) responded to immunomodulatory therapy, only 1 patient (5%) died from complications of refractory sprue, and no patient developed lymphoma.
COLLAGENOUS SPRUE VERSUS CELIAC DISEASE: SEROLOGIC TESTS
Patients with collagenous sprue can have negative HLA-DQw2 test results.
COLLAGENOUS SPRUE VERSUS CELIAC DISEASE VERSUS REFRACTORY SPRUE: HISTOPATHOLOGY AND IMMUNOPHENOTYPING
7) In collagenous sprue (Figure 2, B through F), a variable degree of villous blunting is a consistent observation.
The entrapment of cellular elements within the collagen layer is a mandatory diagnostic criterion for collagenous sprue.
Whereas the histologic features of villous atrophy of the small intestine mimic those of classic celiac disease (more specifically, Marsh-Oberhuber destructive type 3), collagenous sprue is distinctive, with crypt atrophy as well as villous atrophy, (6) resulting in a mucosal thickness markedly less than the reference range (Figure 2, B).
Thin mucosa in collagenous sprue can be accompanied by hyperchromasia of the crypt epithelium (increased nuclear to cytoplasmic ratio) and may represent an inability to maintain healthy cell proliferation because of the severe malabsorption.
In contrast to classic celiac disease, spruelike enteropathies refractory to a gluten-free diet, including collagenous sprue, usually lack an intraepithelial lymphocytosis (Figure 2, C).
The expression patterns of genes involved in the fibrogenesis and fibrolysis and the composition of the subepithelial collagen band in collagenous sprue have been studied recently.
Furthermore, the increased eosinophils in collagenous sprue biopsies that display more severe fibrosis may suggest a role in the pathogenesis of collagenous sprue.