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Hugh J Freeman - One of the best experts on this subject based on the ideXlab platform.
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Olmesartan-Induced Collagenous Sprue
International Journal of Celiac Disease, 2020Co-Authors: Hugh J FreemanAbstract:A 65-yr-old female with diarrhea for 3 months and weight loss was evaluated. There was no travel or family history, but she had been treated with olmesartan for an elevated blood pressure. Fecal studies for an infectious cause were negative and serological studies for celiac disease were negative. A small bowel biopsy showed changes of Collagenous Sprue. The drug was discontinued. Diarrhea ceased and she regained her weight. Repeat biopsies of the small bowel were normal. Except for cessation of the olmesartan, no other dietary (eg., gluten-free diet) or treatment medication (eg., steroids) was provided. Long-term follow-up revealed no recurrence of symptoms in patients with Sprue-like intestinal disease or Collagenous Sprue, consideration should be given to a drug-induced cause. This may limit the need for further clinical studies, restrictive diets or powerful prescribed medications, including steroids and immunosuppressive agents.
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Collagenous Sprue, a Heterogeneous Small Bowel Disorder
International Journal of Celiac Disease, 2019Co-Authors: Hugh J FreemanAbstract:Collagenous Sprue is a disorder characterized by severe diarrhea, progressive malaborption of multiple nutrients, protein depletion and weight loss accompanied by a pathognomic small bowel biopsy lesion characterized by a villus atrophy and a band-like subsepithelial mucosal deposit containing collagen. It has been closely linked to celiac disease, tends to be refractory to treatment and prognosis has been poor with only anecdotes of successful therapy. In recent years, the disorder has been detected in different settings, including early malignancies as a paraneoplastic phenomenon and due to the toxic effects of some medications including non-steroidal anti-inflammatory drugs (NSAIDs) and the angiotensin II receptor antagonist, olmesartan. Here, dramatic reversal to normal of the clinical and pathological changes have been documented so that a medication history is critical in any patient presenting with Sprue-like intestinal disease.
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Sprue-like Intestinal Disease
International Journal of Celiac Disease, 2016Co-Authors: Hugh J FreemanAbstract:Recurrent symptoms in well-established celiac disease may result in further clinical evaluation. In most, poor diet compliance is present. Other considerations include an unidentified gluten source, an erroneous initial diagnosis, another or second and superimposed cause for symptoms, or a complication, including Collagenous Sprue or an intestinal lymphoma. Failure to define an initial gluten-free diet response, however, suggests that celiac disease may not be present. Instead, a distinctive enteropathic process, refractory to diet restrictions, including gluten, is evident. This “Sprue-like” intestinal disorder or enteropathy remains unclassified, and probably, represents a heterogeneous entity. Molecular changes suggestive of early clonal expansion of an aberrant population of intra-epithelial lymphocytes may be detected in some (with or without a prior gluten-free diet response), and these changes may signify an early or “cryptic” lymphoma. Other newly recognized lymphoproliferative disorders occurring in the setting of celiac disease have been recorded, including hepatosplenic delta-gamma T-cell lymphoma, an indolent CD4+ T-cell lymphoma, particularly in younger males, and large granular lymphocytic leukemia, a possibly treatable disorder characterized by the clonal expansion of T-cells in blood and small intestinal mucosa. Studies using IL-15 blockade in a transgenic mouse model with pathologic features of a Sprue-like intestinal disease has led to human clinical trials with encouraging positive results.
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spontaneous free perforation of the small intestine in adults
World Journal of Gastroenterology, 2014Co-Authors: Hugh J FreemanAbstract:Spontaneous free perforation of the small intestine is uncommon, especially if there is no prior history of visceral trauma. However, free, even recurrent, perforation may complicate a defined and established clinical disorder, such as Crohn’s disease. In addition, free perforation may be the initial clinical presentation of an occult intestinal disorder, such as a lymphoma complicating celiac disease, causing diffuse peritonitis and an acute abdomen. Initial diagnosis of the precise cause may be difficult, but now has been aided by computerized tomographic imaging. The site of perforation may be helpful in defining a cause (e.g., ileal perforation in Crohn’s disease, jejunal perforation in celiac disease, complicated by lymphoma or Collagenous Sprue). Urgent surgical intervention, however, is usually required for precise diagnosis and treatment. During evaluation, an expanding list of other possible causes should be considered, even after surgery, as subsequent management may be affected. Free perforation may not only complicate an established intestinal disorder, but also a new acute process (e.g., caused by different infectious agents) or a longstanding and unrecognized disorder (e.g., congenital, metabolic and vascular causes). Moreover, new endoscopic therapeutic and medical therapies, including use of emerging novel biological agents, have been complicated by intestinal perforation. Recent studies also support the hypothesis that perforation of the small intestine may be genetically-based with different mutations causing altered connective tissue structure, synthesis and repair.
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Update on Collagenous Sprue
World journal of gastroenterology, 2010Co-Authors: Hugh J FreemanAbstract:Collagenous Sprue has traditionally been defined as a small intestinal mucosal disorder characterized by persistent diarrhea, severe malabsorption with multiple nutrient deficiencies and progressive weight loss. Pathologically, a severe to variably severe "flattened" mucosal biopsy lesion with distinctive sub-epithelial deposits in the lamina propria region is detected. Histochemical stains and ultrastructural studies have confirmed that these deposits contain collagens. Often, an initial diagnosis of celiac disease is considered but no continued response to treatment with a gluten-free diet occurs. Recent reports indicate an intimate relationship between Collagenous Sprue and celiac disease, sometimes with concomitant T-cell enteropathy. In addition, permanent disappearance of these deposits after resection of a localized colon cancer suggested that this disorder could actually represent a paraneoplastic morphologic marker of an occult malignancy. Studies showing either gastric or colonic involvement (or both) with this unusual Collagenous inflammatory mucosal process may also reflect a far more extensive and heterogeneous process than previously appreciated.
Joseph A. Murray - One of the best experts on this subject based on the ideXlab platform.
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Collagenous Sprue cross-sectional imaging: a comparative blinded study
Abdominal Radiology, 2017Co-Authors: Badr Al-bawardy, Joseph A. Murray, Shannon P. Sheedy, Michelle B. Herberts, Alberto Rubio-tapia, Elizabeth Rajan, David H. Bruining, Stephanie L. Hansel, John M. Barlow, Joel G. FletcherAbstract:Purpose Collagenous Sprue (CS) is a rare enteropathy characterized by villous atrophy and a thickened subepithelial collagen band. The aim of this study is to describe the cross-sectional imaging findings of CS. Methods A case–control, retrospective study with cases of all CS patients from January 2000 to 2015 was performed. Inclusion criteria were (1) Histopathologic diagnosis and (2) Imaging with computed tomography abdomen/pelvis (CT A/P), CT enterography (CTE), or magnetic resonance enterography within 6 months of small bowel (SB) biopsy. Control subjects were irritable bowel syndrome (IBS) patients who underwent CTE. Imaging studies were examined by two GI radiologists, blinded to patient data. Results 108 patients (54 CS; 54 IBS) were included. Mean age was 56.7 ± 16.5 years, and 68% were female (72% in CS group vs. 63% in IBS group; p = 0.3). CS patients were significantly older (67 ± 12 vs. 47 ± 15 year; p
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Collagenous Sprue cross-sectional imaging: a comparative blinded study.
Abdominal radiology (New York), 2017Co-Authors: Badr Al-bawardy, Joseph A. Murray, Shannon P. Sheedy, Michelle B. Herberts, Alberto Rubio-tapia, Elizabeth Rajan, David H. Bruining, Stephanie L. Hansel, John M. Barlow, Joel G. FletcherAbstract:Collagenous Sprue (CS) is a rare enteropathy characterized by villous atrophy and a thickened subepithelial collagen band. The aim of this study is to describe the cross-sectional imaging findings of CS. A case–control, retrospective study with cases of all CS patients from January 2000 to 2015 was performed. Inclusion criteria were (1) Histopathologic diagnosis and (2) Imaging with computed tomography abdomen/pelvis (CT A/P), CT enterography (CTE), or magnetic resonance enterography within 6 months of small bowel (SB) biopsy. Control subjects were irritable bowel syndrome (IBS) patients who underwent CTE. Imaging studies were examined by two GI radiologists, blinded to patient data. 108 patients (54 CS; 54 IBS) were included. Mean age was 56.7 ± 16.5 years, and 68% were female (72% in CS group vs. 63% in IBS group; p = 0.3). CS patients were significantly older (67 ± 12 vs. 47 ± 15 year; p
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Collagenous Sprue cross sectional imaging a comparative blinded study
Abdominal Radiology, 2017Co-Authors: Badr Albawardy, Joseph A. Murray, Shannon P. Sheedy, Michelle B. Herberts, Elizabeth Rajan, David H. Bruining, Stephanie L. Hansel, John M. Barlow, Alberto Rubiotapia, Joel G. FletcherAbstract:Collagenous Sprue (CS) is a rare enteropathy characterized by villous atrophy and a thickened subepithelial collagen band. The aim of this study is to describe the cross-sectional imaging findings of CS. A case–control, retrospective study with cases of all CS patients from January 2000 to 2015 was performed. Inclusion criteria were (1) Histopathologic diagnosis and (2) Imaging with computed tomography abdomen/pelvis (CT A/P), CT enterography (CTE), or magnetic resonance enterography within 6 months of small bowel (SB) biopsy. Control subjects were irritable bowel syndrome (IBS) patients who underwent CTE. Imaging studies were examined by two GI radiologists, blinded to patient data. 108 patients (54 CS; 54 IBS) were included. Mean age was 56.7 ± 16.5 years, and 68% were female (72% in CS group vs. 63% in IBS group; p = 0.3). CS patients were significantly older (67 ± 12 vs. 47 ± 15 year; p < 0.001) and more likely to be on angiotensin receptor blockers (41% vs. 6%; p < 0.001) as compared to the IBS group. Compared to IBS, CS patients were more likely to have mesenteric lymph node (LN) prominence (56% vs. 15%; p < 0.001), jejunoileal fold pattern reversal (46% vs. 6%; p < 0.001), SB dilation (28% vs. 0%; p < 0.001), SB conformational change (28% vs. 6%; p = 0.002), SB wall thickening (13% vs. 2%; p = 0.03), and ulcerative jejunoileitis (4% vs. 0%; p = 0.01). Radiologists suspected malabsorption in 72% in the CS group and 2% in the IBS group (p < 0.001). Imaging findings suggestive of mucosal malabsorption are commonly demonstrated in CS.
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Increased immunoglobulin G4-positive plasma cells in Collagenous Sprue.
Human pathology, 2013Co-Authors: J. Kenneth Schoolmeester, Sarah M. Jenkins, Joseph A. Murray, Vishal S. ChandanAbstract:Collagenous Sprue is a rare enteropathy whose etiology is unknown, but immune-mediated mechanisms are one of several possibilities. However, the role of immunoglobulin G4 (IgG4)-positive plasma cells has not been studied in Collagenous Sprue. Endoscopic biopsies from the duodenum with a histologic diagnosis of Collagenous Sprue (n = 40 from 35 patients), celiac disease (n = 25), peptic duodenitis (n = 15) and normal duodenum (n = 25) were immunohistochemically stained with IgG4 and CD138 antibodies. For each case, the quantities of IgG4- and CD138-positive plasma cells in the lamina propria were estimated by averaging the number in 3 high-power fields (hpf) that showed the highest concentration. Nine of forty Collagenous Sprue samples showed a mean of 10 or more IgG4 plasma cells per hpf, whereas none of the duodenal control biopsies showed 10 or more IgG4 plasma cells per hpf: celiac disease (P = .01), peptic duodenitis (P = .05), and normal duodenum (P = .01). Our study demonstrates that increased IgG4-positive plasma cells are present in a subset (23%) of Collagenous Sprue and may play a role in its pathogenesis.
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Severe Spruelike Enteropathy Associated With Olmesartan
Mayo Clinic proceedings, 2012Co-Authors: Alberto Rubio-tapia, Margot L. Herman, Jonas F. Ludvigsson, Darlene G. Kelly, Thomas F. Mangan, Joseph A. MurrayAbstract:Objective: To report the response to discontinuation of olmesartan, an angiotensin II receptor antagonist commonly prescribed for treatment of hypertension, in patients with unexplained severe Spruelike enteropathy. Patients and Methods: All 22 patients included in this report were seen at Mayo Clinic in Rochester, Minnesota, between August 1, 2008, and August 1, 2011, for evaluation of unexplained chronic diarrhea and enteropathy while taking olmesartan. Celiac disease was ruled out in all cases. To be included in the study, the patients also had to have clinical improvement after suspension of olmesartan. Results: The 22 patients (13 women) had a median age of 69.5 years (range, 47-81 years). Most patients were taking 40 mg/d of olmesartan (range, 10-40 mg/d). The clinical presentation was of chronic diarrhea and weight loss (median, 18 kg; range, 2.5-57 kg), which required hospitalization in 14 patients (64%). Intestinal biopsies showed both villous atrophy and variable degrees of mucosal inflammation in 15 patients, and marked subepithelial collagen deposition (Collagenous Sprue) in 7. Tissue transglutaminase antibodies were not detected. A gluten-free diet was not helpful. Collagenous or lymphocytic gastritis was documented in 7 patients, and microscopic colitis was documented in 5 patients. Clinical response, with a mean weight gain of 12.2 kg, was demonstrated in all cases. Histologic recovery or improvement of the duodenum after discontinuation of olmesartan was confirmed in all 18 patients who underwent follow-up biopsies. Conclusion: Olmesartan may be associated with a severe form of Spruelike enteropathy. Clinical response and histologic recovery are expected after suspension of the drug.
Alberto Rubio-tapia - One of the best experts on this subject based on the ideXlab platform.
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Collagenous Sprue cross-sectional imaging: a comparative blinded study
Abdominal Radiology, 2017Co-Authors: Badr Al-bawardy, Joseph A. Murray, Shannon P. Sheedy, Michelle B. Herberts, Alberto Rubio-tapia, Elizabeth Rajan, David H. Bruining, Stephanie L. Hansel, John M. Barlow, Joel G. FletcherAbstract:Purpose Collagenous Sprue (CS) is a rare enteropathy characterized by villous atrophy and a thickened subepithelial collagen band. The aim of this study is to describe the cross-sectional imaging findings of CS. Methods A case–control, retrospective study with cases of all CS patients from January 2000 to 2015 was performed. Inclusion criteria were (1) Histopathologic diagnosis and (2) Imaging with computed tomography abdomen/pelvis (CT A/P), CT enterography (CTE), or magnetic resonance enterography within 6 months of small bowel (SB) biopsy. Control subjects were irritable bowel syndrome (IBS) patients who underwent CTE. Imaging studies were examined by two GI radiologists, blinded to patient data. Results 108 patients (54 CS; 54 IBS) were included. Mean age was 56.7 ± 16.5 years, and 68% were female (72% in CS group vs. 63% in IBS group; p = 0.3). CS patients were significantly older (67 ± 12 vs. 47 ± 15 year; p
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Collagenous Sprue cross-sectional imaging: a comparative blinded study.
Abdominal radiology (New York), 2017Co-Authors: Badr Al-bawardy, Joseph A. Murray, Shannon P. Sheedy, Michelle B. Herberts, Alberto Rubio-tapia, Elizabeth Rajan, David H. Bruining, Stephanie L. Hansel, John M. Barlow, Joel G. FletcherAbstract:Collagenous Sprue (CS) is a rare enteropathy characterized by villous atrophy and a thickened subepithelial collagen band. The aim of this study is to describe the cross-sectional imaging findings of CS. A case–control, retrospective study with cases of all CS patients from January 2000 to 2015 was performed. Inclusion criteria were (1) Histopathologic diagnosis and (2) Imaging with computed tomography abdomen/pelvis (CT A/P), CT enterography (CTE), or magnetic resonance enterography within 6 months of small bowel (SB) biopsy. Control subjects were irritable bowel syndrome (IBS) patients who underwent CTE. Imaging studies were examined by two GI radiologists, blinded to patient data. 108 patients (54 CS; 54 IBS) were included. Mean age was 56.7 ± 16.5 years, and 68% were female (72% in CS group vs. 63% in IBS group; p = 0.3). CS patients were significantly older (67 ± 12 vs. 47 ± 15 year; p
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Sprue-Like Enteropathy Associated With Olmesartan: A New Kid on the Enteropathy Block Enteropatia Tipo Sprue Induzida Por Olmesartan: Uma Nova Entidade no Campo Das Enteropatias
2016Co-Authors: Isabel A. Hujoel, Alberto Rubio-tapiaAbstract:Sprue-like enteropathy associated with olmesartan, first identified by our group in 2012, is characterized by chronic diarrhea (often severe) and weight loss that is unresponsive to a gluten-free diet. Laboratory work-up commonly reveals non-specific anemia, hypoalbuminemia, electrolyte imbalance, and vitamin deficiencies, consistent with a severe malabsorption process. Histopathological findings include a combination of duodenal villous atrophy, increased intraepithelial lymphocytes, and a thickened subepithelial collagen layer (Collagenous Sprue). Histologic changes can be limited to the small bowel, or may include the entire gastrointestinal tract, with findings such as lymphocytic/Collagenous gastritis and colitis. Individuals with Sprue-like enteropathy associated with olmesartan have negative celiac serology. The majority may have either HLA-DQ2 or DQ8 haplotypes (61--81%). Diagnosis of olmesartan associated enteropathy should therefore be considered in cases of villous atrophy with negative celiac serology (so-called seronegative villous atrophy). Confirmation of diagnosis requires clinical resolution of symptoms after olmesartan withdrawal. Mucosal recovery is also expected within 3--6 months of olmesartan withdrawal and a follow-up duodenal biopsy is reasonable.
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Severe Spruelike Enteropathy Associated With Olmesartan
Mayo Clinic proceedings, 2012Co-Authors: Alberto Rubio-tapia, Margot L. Herman, Jonas F. Ludvigsson, Darlene G. Kelly, Thomas F. Mangan, Joseph A. MurrayAbstract:Objective: To report the response to discontinuation of olmesartan, an angiotensin II receptor antagonist commonly prescribed for treatment of hypertension, in patients with unexplained severe Spruelike enteropathy. Patients and Methods: All 22 patients included in this report were seen at Mayo Clinic in Rochester, Minnesota, between August 1, 2008, and August 1, 2011, for evaluation of unexplained chronic diarrhea and enteropathy while taking olmesartan. Celiac disease was ruled out in all cases. To be included in the study, the patients also had to have clinical improvement after suspension of olmesartan. Results: The 22 patients (13 women) had a median age of 69.5 years (range, 47-81 years). Most patients were taking 40 mg/d of olmesartan (range, 10-40 mg/d). The clinical presentation was of chronic diarrhea and weight loss (median, 18 kg; range, 2.5-57 kg), which required hospitalization in 14 patients (64%). Intestinal biopsies showed both villous atrophy and variable degrees of mucosal inflammation in 15 patients, and marked subepithelial collagen deposition (Collagenous Sprue) in 7. Tissue transglutaminase antibodies were not detected. A gluten-free diet was not helpful. Collagenous or lymphocytic gastritis was documented in 7 patients, and microscopic colitis was documented in 5 patients. Clinical response, with a mean weight gain of 12.2 kg, was demonstrated in all cases. Histologic recovery or improvement of the duodenum after discontinuation of olmesartan was confirmed in all 18 patients who underwent follow-up biopsies. Conclusion: Olmesartan may be associated with a severe form of Spruelike enteropathy. Clinical response and histologic recovery are expected after suspension of the drug.
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M1189 Collagenous Sprue: Clinical Characteristics, Response to Steroids, and Prognosis
Gastroenterology, 2009Co-Authors: Alberto Rubio-tapia, Nicholas J Talley, Suryakanth R Gurudu, Joseph A. MurrayAbstract:Background: Collagenous Sprue (CS) is a very rare cause of severe malabsorption with an enteropathy that is characterized by villous atrophy and a distinctive band of subepithelial collagen deposition. A poor outcome has been reported historically. Aim: To describe the clinical characteristics, treatment, and prognosis in a contemporary cohort of patients with CS. Methods: We included cases with biopsy-proven CS evaluated at the Mayo Clinic Results: 15 patients (80% females) with a median age of 67 years (range, 53-83) were included. The clinical manifestations were diarrhea (100%), loss of weight (93%), and abdominal pain (14%). Hospitalizationwas required because severe diarrhea in 9 (64%) patients. Hypoalbuminemia and anemia were evident in 9 (64%) and 7 (50%) patients, respectively. Humanleukocyte antigen genes DQ2 or DQ8 were present in 12 (80%) patients (DQ2+ single dose [n=8], DQ2+ double dose [n=3], and DQ2+/DQ8+ [n=1]). Celiac antibodies were present in only 1 patient. A prior diagnosis of celiac disease was evident in 7 (47%) patients. Histologically, all cases had both the abnormal collagen deposition and some degree of villous atrophy. Aberrant (clonal) intraepithelial lymphocytes were detected in 1 (of 12) patients tested by both immunostaining and T-cell clonality analysis. Associated disorders were Collagenous gastritis (n=4), Collagenous colitis (n=4), small-intestine bacterial overgrowth (n=4), lymphocytic colitis (n=2), lymphocytic gastritis (n=1), autoimmune hepatitis (n=1), and hyposplenism (n=1). All patients received treatment with a combination of a strict gluten-free diet and steroids such as budesonide 9mg/day (n=12), prednisone 30-40 mg/day (n=2) and dexamethasone 8 mg/day (n=1). Parenteral nutrition was necessary in 11 (79%) patients. One patient developed enteropathy-type T-cell lymphoma during followup and received a combination of nitrogen mustard and methylprednisolone. Clinical followup after treatment was available in 12 (92%) patients (median time = 16 months; range, 172), disappearance of diarrhea was observed in 11 with complete histologic remission documented in one case. Three patients died during follow-up because of emaciation, enteropathy-type T-cell lymphoma, and aspiration pneumonia, respectively. Conclusions: We report the largest case series of CS. Treatment with a combination of gluten-free diet and steroids (especially budesonide) was useful to control symptoms in most patients; however, length of follow-up was limited so far to a few months in the majority of our patients. CS was associated with collagen deposition in other organs, autoimmune conditions, and lymphoma.
Joel G. Fletcher - One of the best experts on this subject based on the ideXlab platform.
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Collagenous Sprue cross-sectional imaging: a comparative blinded study
Abdominal Radiology, 2017Co-Authors: Badr Al-bawardy, Joseph A. Murray, Shannon P. Sheedy, Michelle B. Herberts, Alberto Rubio-tapia, Elizabeth Rajan, David H. Bruining, Stephanie L. Hansel, John M. Barlow, Joel G. FletcherAbstract:Purpose Collagenous Sprue (CS) is a rare enteropathy characterized by villous atrophy and a thickened subepithelial collagen band. The aim of this study is to describe the cross-sectional imaging findings of CS. Methods A case–control, retrospective study with cases of all CS patients from January 2000 to 2015 was performed. Inclusion criteria were (1) Histopathologic diagnosis and (2) Imaging with computed tomography abdomen/pelvis (CT A/P), CT enterography (CTE), or magnetic resonance enterography within 6 months of small bowel (SB) biopsy. Control subjects were irritable bowel syndrome (IBS) patients who underwent CTE. Imaging studies were examined by two GI radiologists, blinded to patient data. Results 108 patients (54 CS; 54 IBS) were included. Mean age was 56.7 ± 16.5 years, and 68% were female (72% in CS group vs. 63% in IBS group; p = 0.3). CS patients were significantly older (67 ± 12 vs. 47 ± 15 year; p
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Collagenous Sprue cross-sectional imaging: a comparative blinded study.
Abdominal radiology (New York), 2017Co-Authors: Badr Al-bawardy, Joseph A. Murray, Shannon P. Sheedy, Michelle B. Herberts, Alberto Rubio-tapia, Elizabeth Rajan, David H. Bruining, Stephanie L. Hansel, John M. Barlow, Joel G. FletcherAbstract:Collagenous Sprue (CS) is a rare enteropathy characterized by villous atrophy and a thickened subepithelial collagen band. The aim of this study is to describe the cross-sectional imaging findings of CS. A case–control, retrospective study with cases of all CS patients from January 2000 to 2015 was performed. Inclusion criteria were (1) Histopathologic diagnosis and (2) Imaging with computed tomography abdomen/pelvis (CT A/P), CT enterography (CTE), or magnetic resonance enterography within 6 months of small bowel (SB) biopsy. Control subjects were irritable bowel syndrome (IBS) patients who underwent CTE. Imaging studies were examined by two GI radiologists, blinded to patient data. 108 patients (54 CS; 54 IBS) were included. Mean age was 56.7 ± 16.5 years, and 68% were female (72% in CS group vs. 63% in IBS group; p = 0.3). CS patients were significantly older (67 ± 12 vs. 47 ± 15 year; p
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Collagenous Sprue cross sectional imaging a comparative blinded study
Abdominal Radiology, 2017Co-Authors: Badr Albawardy, Joseph A. Murray, Shannon P. Sheedy, Michelle B. Herberts, Elizabeth Rajan, David H. Bruining, Stephanie L. Hansel, John M. Barlow, Alberto Rubiotapia, Joel G. FletcherAbstract:Collagenous Sprue (CS) is a rare enteropathy characterized by villous atrophy and a thickened subepithelial collagen band. The aim of this study is to describe the cross-sectional imaging findings of CS. A case–control, retrospective study with cases of all CS patients from January 2000 to 2015 was performed. Inclusion criteria were (1) Histopathologic diagnosis and (2) Imaging with computed tomography abdomen/pelvis (CT A/P), CT enterography (CTE), or magnetic resonance enterography within 6 months of small bowel (SB) biopsy. Control subjects were irritable bowel syndrome (IBS) patients who underwent CTE. Imaging studies were examined by two GI radiologists, blinded to patient data. 108 patients (54 CS; 54 IBS) were included. Mean age was 56.7 ± 16.5 years, and 68% were female (72% in CS group vs. 63% in IBS group; p = 0.3). CS patients were significantly older (67 ± 12 vs. 47 ± 15 year; p < 0.001) and more likely to be on angiotensin receptor blockers (41% vs. 6%; p < 0.001) as compared to the IBS group. Compared to IBS, CS patients were more likely to have mesenteric lymph node (LN) prominence (56% vs. 15%; p < 0.001), jejunoileal fold pattern reversal (46% vs. 6%; p < 0.001), SB dilation (28% vs. 0%; p < 0.001), SB conformational change (28% vs. 6%; p = 0.002), SB wall thickening (13% vs. 2%; p = 0.03), and ulcerative jejunoileitis (4% vs. 0%; p = 0.01). Radiologists suspected malabsorption in 72% in the CS group and 2% in the IBS group (p < 0.001). Imaging findings suggestive of mucosal malabsorption are commonly demonstrated in CS.
Bo Shen - One of the best experts on this subject based on the ideXlab platform.
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Comparison of clinical features, treatment, and outcomes of Collagenous Sprue, celiac disease, and Collagenous colitis.
Journal of gastroenterology and hepatology, 2017Co-Authors: Nan Lan, Bo Shen, Lisi Yuan, Xiuli LiuAbstract:BACKGROUND Collagenous Sprue (CS) is a rare form of enteropathy that had been reported to be associated with celiac disease (CD) and Collagenous colitis (CC). The aim of our study was to compare the clinical features, treatments, and outcomes of CS, CD, and CC. METHODS All patients with histologic diagnosis of CS, CD, or CC with complete clinical data were extracted from our pathology database between 1990 and 2015. Demographic and clinical features were recorded along with treatments and outcomes. RESULTS A total of 21 patients with CS were included. Overall CS patients were more symptomatic with 17 (81.0%) patients with diarrhea and 15 (71.4%) with unintentional weight loss. Positive celiac serology was noted in 5 (23.8%) CS patients. CS patients had higher rates for disease-related temporary total parenteral nutrition (TPN) use (38.1% vs. 1.1% vs. 1.0%, P
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comparison of clinical features treatment and outcomes of Collagenous Sprue celiac disease and Collagenous colitis
Journal of Gastroenterology and Hepatology, 2017Co-Authors: Bo Shen, Lisi YuanAbstract:BACKGROUND: Collagenous Sprue (CS) is a rare form of enteropathy that had been reported to be associated with celiac disease (CD) and Collagenous colitis (CC). The aim of our study was to compare the clinical features, treatments, and outcomes of CS, CD, and CC. METHODS: All patients with histologic diagnosis of CS, CD, or CC with complete clinical data were extracted from our pathology database between 1990 and 2015. Demographic and clinical features were recorded along with treatments and outcomes. RESULTS: A total of 21 patients with CS were included. Overall CS patients were more symptomatic with 17 (81.0%) patients with diarrhea and 15 (71.4%) with unintentional weight loss. Positive celiac serology was noted in 5 (23.8%) CS patients. CS patients had higher rates for disease-related temporary total parenteral nutrition (TPN) use (38.1% vs. 1.1% vs. 1.0%, P < 0.0001) and disease-related hospitalization (52.4% vs. 3.3% vs. 8.2%, P < 0.0001) than that in CD and CC patients. Twenty CS patients received treatments, including the combination of gluten-free diet (GFD) and corticosteroids (n = 12), GFD only (n = 2), and corticosteroids only (n = 6). All CS patients showed symptomatic reliefs with treatment. Although CS patients had a higher rate for hospitalization and TPN use, disease-related death was not observed in all three groups. CONCLUSIONS: Collagenous Sprue patients had more severe clinical presentation than patients with CD and CC and therefore had higher demand for temporary TPN and hospitalization. Nevertheless, a prompt use of steroids and/or GFD upon histologic diagnosis of CS may have contributed to an overall excellent prognosis.
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Concomitant presentation of Collagenous Sprue and HFE hemochromatosis
Journal of Crohn's & colitis, 2011Co-Authors: Keely R. Parisian, Thomas Plesec, Kyrsten D. Fairbanks, Anthony S. Tavill, Bo ShenAbstract:Abstract Collagenous Sprue (CS) is a progressive malabsorptive disorder characterized by collagen deposition beneath the basement membrane of small bowel epithelium in refractory celiac Sprue. 1 CS is a pathologically distinct entity from celiac disease, despite a similar clinical presentation. The etiology of CS is unclear, although there are speculations that CS and celiac disease may share similar pathogenetic pathways. 2 On the other hand, HFE hemochromatosis (HH) is a distinct disease entity. Celiac disease and HH are common HLA-associated genetic disorders in Northern European populations. There are a few case reports linking celiac disease and HH. We present a patient diagnosed with concurrent CS and HH.