Original ArticlesCollagenous Sprue: A Clinicopathologic Study of 12 CasesMaguire, Aoife A. MB, MRCPI*; Greenson, Joel K. MD†; Lauwers, Greg Y. MD‡; Ginsburg, Richard E. DO, FACOI§; Williams, Geraint T. MD, FRCP, FRCPath∥; Brown, Ian S. MBBS, FRCPA¶; Riddell, Robert H. MD, FRCPath, FRCPC♯; O'Donoghue, Diarmuid MD, FRCP, FRCPI**; Sheahan, Kieran D. MB, BSc, FRCPI, FCAP, FRCPath*Author Information *Department of Histopathology, St Vincent's University Hospital, Dublin, Ireland †Department of Pathology, University of Michigan Medical School, Ann Arbor, MI ‡Department of Pathology, Massachussetts General Hospital, Boston, MA §Montgomery Gastroenterology Specialists, P.C., Montgomery, AL ∥Department of Pathology, School of Medicine, Cardiff University, Cardiff, Wales, UK ¶Sullivan Nicolaides Pathology, Brisbane, Queensland, Australia ♯Department of Pathology and Laboratory Medicine, Mount Sinai Hospital, Toronto, Ontario, Canada **Department of Gastroenterology, St. Vincent's University Hospital, Dublin, Ireland Disclosure of funding from the specified organizations is not applicable. Correspondence: Aoife A. Maguire, MB, MRCPI, Department of Histopathology, St Vincent's University Hospital, Elm Park, Dublin 4, Ireland (e-mail: Aoife.firstname.lastname@example.org). There is no pharmaceutical or industry support involved. The American Journal of Surgical Pathology: October 2009 - Volume 33 - Issue 10 - p 1440-1449 doi: 10.1097/PAS.0b013e3181ae2545 Buy SDC Metrics Abstract Collagenous sprue is a rare form of small bowel enteropathy characterized by chronic diarrhea and progressive malabsorption with little data available on its natural history. The pathologic lesion consists of subepithelial collagen deposition associated with variable alterations in villous architecture. The small bowel biopsies of 12 cases were reviewed. Clinical details, celiac serology, and T-cell receptor gene rearrangement study results, when available, were collated. There were 8 females and 4 males (age ranged from 41 to 84 y) who presented with chronic diarrhea and weight loss. Small intestinal biopsies showed subepithelial collagen deposition with varying degrees of villous atrophy and varying numbers of intraepithelial lymphocytes. Four patients had previous biopsies showing enteropathic changes without collagen deposition. Seven cases were associated with collagenous colitis and 1 also had features of lymphocytic colitis. Three patients also had collagen deposition in gastric biopsies. One case was associated with lymphocytic gastritis. Celiac disease (CD, gluten-sensitive enteropathy) was documented in 4 patients. Five patients made a clinical improvement with combinations of a gluten-free diet and immunosuppressive therapy. Two patients died of complications of malnutrition and 1 of another illness. Clonal T-cell populations were identified in 5 of 6 cases tested. Four of these patients improved clinically after treatment but 1 has died. Collagenous sprue evolved on a background of CD in 4 cases. There was no history of CD in others and these cases may be the result of a biologic insult other than gluten sensitivity. None has developed clinical evidence of lymphoma to date. © 2009 Lippincott Williams & Wilkins, Inc.