Collagenous Sprue
Definition
- Enteropathy demonstrating subepithelial collagen deposition combined with villous changes typical of celiac disease
Alternate/Historical Names
- Refractory celiac disease
- Refractory sprue
Diagnostic Criteria
- Irregular, thickened collagen layer below the luminal basement membrane
- Envelops capillaries and inflammatory cells
- Ragged interface with lamina propria
- Normal basement membrane is 1-2 microns, with different inclusion criteria
- Range 48-260 microns maximum thickness (Maguire)
- Over 5 microns thickness (Vakiani)
- Trichrome stain is useful for demonstration of the layer
- Villous atrophy ranging from partial to total
- Increased intraepithelial lymphocytes in small intestine
- 6-92 / 100 enterocytes
- T cell phenotype
- CD3+, CD8 variable
- CD3 stain is useful for identification and counting
- T cell clonality may be present
- Seen in both intestinal tissues and peripherally
- Not clearly related to disease outcome
- Chronic inflammatory infiltrate in lamina propria
- Lymphocytes, plasma cells and eosinophils most common
- Surface mucosa may detach
- Anti-endomysial antibody may be present (4 of 12 cases)
- There is some debate regarding whether or not collagenous sprue is a distinct entity
- It has been suggested that it may simply represent a pattern of refractory celiac disease
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting/updates: 11/11/09, 12/3/09, 10/25/10
