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

Abstract

Collagenous sprue is associated with high morbidity; however, the etiology of this disorder is unclear. Data regarding the pathological and clinical manifestations of patients with collagenous sprue are also limited. We, thus, undertook this study to gain insight into the etiology, disease manifestations and outcomes of collagenous sprue. We searched our departmental database (1999-2008) to identify cases of collagenous sprue and to obtain clinical and laboratory data. Small bowel histology, including thickness of subepithelial collagen, intra-epithelial lymphocyte phenotype and results of T-cell clonality assays were evaluated. Nineteen patients (15 women, 4 men, age 22-80 years, mean 57 years) were identified. Seventeen (89%) had celiac disease and two had unclassified sprue; 9 of 17 (53%) celiac disease patients had refractory disease; 5 of 15 (33%) lacked diarrhea (atypical presentation), including 2 of 6 (33%) with active (untreated) celiac disease and 3 of 9 (33%) with refractory celiac disease. Autoimmune disorders were seen in 12 of 19 (63%) patients and microscopic colitis (n=7), lymphocytic gastritis (n=2) or collagenous gastritis (n=2) were seen in nine patients. Subepithelial collagen thickness was mildly (n=6), moderately (n=10), or markedly (n=3) increased and villous atrophy was total (n=13) or subtotal (n=6). Phenotypically aberrant intraepithelial lymphocytes were not detected in any case. Polymerase chain reaction analysis showed a dominant T-cell clone in the only patient with refractory celiac disease type II. Histological improvement occurred in 7 of 11 (64%) patients. Overall, 8 of 19 (42%) responded to gluten-free diet, including 2 of 9 (22%) with refractory celiac disease and 10 responded to immunomodulatory therapy, including 6 of 9 (67%) with refractory celiac disease. Only one patient died from complications of refractory celiac disease. No patient developed lymphoma. The vast majority of our patients with collagenous sprue had celiac disease. Although, many patients required immunomodulatory therapy for symptom control, a subset responded to gluten-free diet alone. In our experience, collagenous sprue patients had relatively good clinical outcomes.

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  • Authors+Show Affiliations

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    Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA.

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    MeSH

    Adult
    Aged
    Aged, 80 and over
    Celiac Disease
    Cell Separation
    Female
    Flow Cytometry
    Humans
    Immunohistochemistry
    Male
    Middle Aged
    T-Lymphocytes

    Pub Type(s)

    Journal Article

    Language

    eng

    PubMed ID

    19855376

    Citation

    Vakiani, Efsevia, et al. "Collagenous Sprue Is Not Always Associated With Dismal Outcomes: a Clinicopathological Study of 19 Patients." Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc, vol. 23, no. 1, 2010, pp. 12-26.
    Vakiani E, Arguelles-Grande C, Mansukhani MM, et al. Collagenous sprue is not always associated with dismal outcomes: a clinicopathological study of 19 patients. Mod Pathol. 2010;23(1):12-26.
    Vakiani, E., Arguelles-Grande, C., Mansukhani, M. M., Lewis, S. K., Rotterdam, H., Green, P. H., & Bhagat, G. (2010). Collagenous sprue is not always associated with dismal outcomes: a clinicopathological study of 19 patients. Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc, 23(1), pp. 12-26. doi:10.1038/modpathol.2009.151.
    Vakiani E, et al. Collagenous Sprue Is Not Always Associated With Dismal Outcomes: a Clinicopathological Study of 19 Patients. Mod Pathol. 2010;23(1):12-26. PubMed PMID: 19855376.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Collagenous sprue is not always associated with dismal outcomes: a clinicopathological study of 19 patients. AU - Vakiani,Efsevia, AU - Arguelles-Grande,Carolina, AU - Mansukhani,Mahesh M, AU - Lewis,Suzanne K, AU - Rotterdam,Heidrun, AU - Green,Peter H, AU - Bhagat,Govind, Y1 - 2009/10/23/ PY - 2009/10/27/entrez PY - 2009/10/27/pubmed PY - 2010/3/4/medline SP - 12 EP - 26 JF - Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc JO - Mod. Pathol. VL - 23 IS - 1 N2 - Collagenous sprue is associated with high morbidity; however, the etiology of this disorder is unclear. Data regarding the pathological and clinical manifestations of patients with collagenous sprue are also limited. We, thus, undertook this study to gain insight into the etiology, disease manifestations and outcomes of collagenous sprue. We searched our departmental database (1999-2008) to identify cases of collagenous sprue and to obtain clinical and laboratory data. Small bowel histology, including thickness of subepithelial collagen, intra-epithelial lymphocyte phenotype and results of T-cell clonality assays were evaluated. Nineteen patients (15 women, 4 men, age 22-80 years, mean 57 years) were identified. Seventeen (89%) had celiac disease and two had unclassified sprue; 9 of 17 (53%) celiac disease patients had refractory disease; 5 of 15 (33%) lacked diarrhea (atypical presentation), including 2 of 6 (33%) with active (untreated) celiac disease and 3 of 9 (33%) with refractory celiac disease. Autoimmune disorders were seen in 12 of 19 (63%) patients and microscopic colitis (n=7), lymphocytic gastritis (n=2) or collagenous gastritis (n=2) were seen in nine patients. Subepithelial collagen thickness was mildly (n=6), moderately (n=10), or markedly (n=3) increased and villous atrophy was total (n=13) or subtotal (n=6). Phenotypically aberrant intraepithelial lymphocytes were not detected in any case. Polymerase chain reaction analysis showed a dominant T-cell clone in the only patient with refractory celiac disease type II. Histological improvement occurred in 7 of 11 (64%) patients. Overall, 8 of 19 (42%) responded to gluten-free diet, including 2 of 9 (22%) with refractory celiac disease and 10 responded to immunomodulatory therapy, including 6 of 9 (67%) with refractory celiac disease. Only one patient died from complications of refractory celiac disease. No patient developed lymphoma. The vast majority of our patients with collagenous sprue had celiac disease. Although, many patients required immunomodulatory therapy for symptom control, a subset responded to gluten-free diet alone. In our experience, collagenous sprue patients had relatively good clinical outcomes. SN - 1530-0285 UR - https://www.unboundmedicine.com/medline/citation/19855376/Collagenous_sprue_is_not_always_associated_with_dismal_outcomes:_a_clinicopathological_study_of_19_patients_ L2 - http://dx.doi.org/10.1038/modpathol.2009.151 DB - PRIME DP - Unbound Medicine ER -