Celiac disease is a gluten-dependent small intestinal mucosal disorder that causes malabsorption, often with diarrhea and weight loss. Diagnosis is based on detection of typical biopsy changes in the proximal small bo...Celiac disease is a gluten-dependent small intestinal mucosal disorder that causes malabsorption, often with diarrhea and weight loss. Diagnosis is based on detection of typical biopsy changes in the proximal small bowel, followed by evidence for an unequivocal response to a gluten-free diet. Refractoriness in celiac disease may be due to poor diet compliance, sometimes intentional, or consumption of ubiquitious sources of gluten. Alternatively, the original diagnosis may not be correct (eg., duodenal Crohn's disease), or a second cause for symptoms may be present (eg., collagenous colitis, functional bowel disorder). In some with recurrent symptoms, a complication may be present (eg., collagenous sprue, small bowel carcinoma, lymphoma). In some, a response to a gluten-free diet can not be unequivocally defined, and more precise historical terms have been used including "sprue-like intestinal disease" or "unclassified sprue". Although a "wastebasket diagnosis", these likely represent a heterogeneous group, and some, but not all, may develop lymphoma. Precise definition will be critical in the future as an array of new treatments, including biological agents, may emerge.展开更多
A 67-year-old man with celiac disease developed recurrent diarrhea,profound weakness and weight loss, with evidence of marked protein depletion.His clinical course was refractory to a strict gluten-free diet and stero...A 67-year-old man with celiac disease developed recurrent diarrhea,profound weakness and weight loss, with evidence of marked protein depletion.His clinical course was refractory to a strict gluten-free diet and steroid therapy.Postmortem studies led to definition of unrecognized collagenous sprue that caused ulceration and small intestinal perforation.Although PCR showed identical monoclonal T-cell populations in antemortem duodenal biopsies and postmortem jejunum,careful pathological evaluation demonstrated no frank lymphoma.Rarely,overt or even cryptic T-cell lymphoma may complicate collagenous sprue,however, small intestinal ulcers and perforation may also develop independently.The dramatic findings here may reflect an underlying or early molecular event in the eventual clinical appearance of overt T-cell lymphoma.展开更多
Collagenous sprue(CS) is a pattern of small-bowel injury characterized histologically by marked villous blunting,intraepithelial lymphocytes,and thickened sub-epithelial collagen table. Clinically,patients present wit...Collagenous sprue(CS) is a pattern of small-bowel injury characterized histologically by marked villous blunting,intraepithelial lymphocytes,and thickened sub-epithelial collagen table. Clinically,patients present with diarrhea,abdominal pain,malabsorption,and weight loss. Gluten intolerance is the most common cause of villous blunting in the duodenum; however,in a recent case series by the Mayo Clinic,it has been reported that olmesartan can have a similar effect. In this case report,a 62-year-old female with a history of hypothyroidism and hypertension managed for several years with olmesartan presented with abdominal pain,weight loss,and nausea. Despite compliance to a gluten-free diet,the patient's symptoms worsened,losing 20 pounds in 3 wk. Endoscopy showed thickening,scalloping,and mosaiform changes of the duodenal mucosa. The biopsy showed CS characterized by complete villous atrophy,lymphocytosis,and thickened sub-epithelial collagen table. After 2 mo cessation of olmesartan,the patient's symptoms improved,and follow-up endoscopy was normal with complete villous regeneration. These findings suggest that olmesartan was a contributing factor in the etiology of this patient's CS.Clinicians should be aware of the possibility of druginduced CS and potential reversibility after discontinuation of medication.展开更多
Collagenous sprue has traditionally been defined as a small intestinal mucosal disorder characterized by persistent diarrhea, severe malabsorption with multiple nutrient def iciencies and progressive weight loss. Path...Collagenous sprue has traditionally been defined as a small intestinal mucosal disorder characterized by persistent diarrhea, severe malabsorption with multiple nutrient def iciencies 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 conf irmed 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.展开更多
BACKGROUND Collagenous sprue is a serious intestinal malabsorption disorder characterized by dull and flattened intestinal villi with subepithelial collagen deposition.At present,fewer than 100 cases have been reporte...BACKGROUND Collagenous sprue is a serious intestinal malabsorption disorder characterized by dull and flattened intestinal villi with subepithelial collagen deposition.At present,fewer than 100 cases have been reported in the literature.Owing to the rarity of this disease,clinicians and pathologists have insufficient understanding of it.CASE SUMMARY The authors present a case of a 34-year-old female patient who presented with severe watery diarrhea with significant weight loss.She also experienced blurred binocular vision.The intestinal lesions were located mainly in the small intestine,which presented flat and dull intestinal villi with subepithelial collagen deposition.Masson staining was positive.Glucocorticoids were effective for this patient,but there seemed to be glucocorticoid dependence.CONCLUSION Collagenous stomatitis diarrhea is a rare intestinal malabsorption disease that needs to be diagnosed in combination with special clinical manifestations and unique histological features.展开更多
文摘Celiac disease is a gluten-dependent small intestinal mucosal disorder that causes malabsorption, often with diarrhea and weight loss. Diagnosis is based on detection of typical biopsy changes in the proximal small bowel, followed by evidence for an unequivocal response to a gluten-free diet. Refractoriness in celiac disease may be due to poor diet compliance, sometimes intentional, or consumption of ubiquitious sources of gluten. Alternatively, the original diagnosis may not be correct (eg., duodenal Crohn's disease), or a second cause for symptoms may be present (eg., collagenous colitis, functional bowel disorder). In some with recurrent symptoms, a complication may be present (eg., collagenous sprue, small bowel carcinoma, lymphoma). In some, a response to a gluten-free diet can not be unequivocally defined, and more precise historical terms have been used including "sprue-like intestinal disease" or "unclassified sprue". Although a "wastebasket diagnosis", these likely represent a heterogeneous group, and some, but not all, may develop lymphoma. Precise definition will be critical in the future as an array of new treatments, including biological agents, may emerge.
文摘A 67-year-old man with celiac disease developed recurrent diarrhea,profound weakness and weight loss, with evidence of marked protein depletion.His clinical course was refractory to a strict gluten-free diet and steroid therapy.Postmortem studies led to definition of unrecognized collagenous sprue that caused ulceration and small intestinal perforation.Although PCR showed identical monoclonal T-cell populations in antemortem duodenal biopsies and postmortem jejunum,careful pathological evaluation demonstrated no frank lymphoma.Rarely,overt or even cryptic T-cell lymphoma may complicate collagenous sprue,however, small intestinal ulcers and perforation may also develop independently.The dramatic findings here may reflect an underlying or early molecular event in the eventual clinical appearance of overt T-cell lymphoma.
文摘Collagenous sprue(CS) is a pattern of small-bowel injury characterized histologically by marked villous blunting,intraepithelial lymphocytes,and thickened sub-epithelial collagen table. Clinically,patients present with diarrhea,abdominal pain,malabsorption,and weight loss. Gluten intolerance is the most common cause of villous blunting in the duodenum; however,in a recent case series by the Mayo Clinic,it has been reported that olmesartan can have a similar effect. In this case report,a 62-year-old female with a history of hypothyroidism and hypertension managed for several years with olmesartan presented with abdominal pain,weight loss,and nausea. Despite compliance to a gluten-free diet,the patient's symptoms worsened,losing 20 pounds in 3 wk. Endoscopy showed thickening,scalloping,and mosaiform changes of the duodenal mucosa. The biopsy showed CS characterized by complete villous atrophy,lymphocytosis,and thickened sub-epithelial collagen table. After 2 mo cessation of olmesartan,the patient's symptoms improved,and follow-up endoscopy was normal with complete villous regeneration. These findings suggest that olmesartan was a contributing factor in the etiology of this patient's CS.Clinicians should be aware of the possibility of druginduced CS and potential reversibility after discontinuation of medication.
文摘Collagenous sprue has traditionally been defined as a small intestinal mucosal disorder characterized by persistent diarrhea, severe malabsorption with multiple nutrient def iciencies 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 conf irmed 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.
文摘BACKGROUND Collagenous sprue is a serious intestinal malabsorption disorder characterized by dull and flattened intestinal villi with subepithelial collagen deposition.At present,fewer than 100 cases have been reported in the literature.Owing to the rarity of this disease,clinicians and pathologists have insufficient understanding of it.CASE SUMMARY The authors present a case of a 34-year-old female patient who presented with severe watery diarrhea with significant weight loss.She also experienced blurred binocular vision.The intestinal lesions were located mainly in the small intestine,which presented flat and dull intestinal villi with subepithelial collagen deposition.Masson staining was positive.Glucocorticoids were effective for this patient,but there seemed to be glucocorticoid dependence.CONCLUSION Collagenous stomatitis diarrhea is a rare intestinal malabsorption disease that needs to be diagnosed in combination with special clinical manifestations and unique histological features.
