BACKGROUND Autoimmune enteropathy(AIE)is a rare disease whose diagnosis and long-term prognosis remain challenging,especially for adult AIE patients.AIM To improve overall understanding of this disease’s diagnosis an...BACKGROUND Autoimmune enteropathy(AIE)is a rare disease whose diagnosis and long-term prognosis remain challenging,especially for adult AIE patients.AIM To improve overall understanding of this disease’s diagnosis and prognosis.METHODS We retrospectively analyzed the clinical,endoscopic and histopathological characteristics and prognoses of 16 adult AIE patients in our tertiary medical center between 2011 and 2023,whose diagnosis was based on the 2007 diagnostic criteria.RESULTS Diarrhea in AIE patients was characterized by secretory diarrhea.The common endoscopic manifestations were edema,villous blunting and mucosal hyperemia in the duodenum and ileum.Villous blunting(100%),deep crypt lymphocytic infiltration(67%),apoptotic bodies(50%),and mild intraepithelial lymphocytosis(69%)were observed in the duodenal biopsies.Moreover,there were other remarkable abnormalities,including reduced or absent goblet cells(duodenum 94%,ileum 62%),reduced or absent Paneth cells(duodenum 94%,ileum 69%)and neutrophil infiltration(duodenum 100%,ileum 69%).Our patients also fulfilled the 2018 diagnostic criteria but did not match the 2022 diagnostic criteria due to undetectable anti-enterocyte antibodies.All patients received glucocorticoid therapy as the initial medication,of which 14/16 patients achieved a clinical response in 5(IQR:3-20)days.Immunosuppressants were administered to 9 patients with indications of steroid dependence(6/9),steroid refractory status(2/9),or intensified maintenance medication(1/9).During the median of 20.5 months of followup,2 patients died from multiple organ failure,and 1 was diagnosed with non-Hodgkin’s lymphoma.The cumulative relapse-free survival rates were 62.5%,55.6%and 37.0%at 6 months,12 months and 48 months,respectively.CONCLUSION Certain histopathological findings,including a decrease or disappearance of goblet and Paneth cells in intestinal biopsies,might be potential diagnostic criteria for adult AIE.The long-term prognosis is still unsatisfactory despite corticosteroid and immunosuppressant medications,which highlights the need for early diagnosis and novel medications.展开更多
BACKGROUND Percutaneous drainage(PCD)and endoscopic approaches have largely replaced surgical drainage as the initial approach for(peri)pancreatic fluid collections(PFC)s,while complications associated with endoscopic...BACKGROUND Percutaneous drainage(PCD)and endoscopic approaches have largely replaced surgical drainage as the initial approach for(peri)pancreatic fluid collections(PFC)s,while complications associated with endoscopic stent implantation are common.AIM To introduce a novel endoscopic therapy named endoscopic transgastric fenestration(ETGF),which involves resection of tissue by endoscopic accessory between gastric and PFCs without stent implantation,and to evaluate its efficacy and safety compared with PCD for the management of PFCs adjacent to the gastric wall.METHODS Patients diagnosed with PFCs adjacent to the gastric wall and who subsequently received ETGF or PCD were restrospectively enrolled.Indications for intervention were consistent with related guidelines.We analyzed patients baseline characteristics,technical and clinical success rate,recurrence and reintervention rate,procedure-related complications and adverse events.RESULTS Seventy-two eligible patients were retrospectively identified(ETGF=34,PCD=38)from October 2017 to May 2021.Patients in the ETGF group had a significantly higher clinical success rate than those in the PCD group(97.1 vs 76.3%,P=0.01).There were no statistically significant differences regarding recurrence,reintervention and incidence of complication between the two groups.While long-term catheter drainage was very common in the PCD group.CONCLUSION Compared with PCD,ETGF has a higher clinical success rate in the management of PFCs adjacent to the gastric wall.ETGF is an alternative effective strategy for the treatment of PFCs adjacent to the gastric wall.展开更多
基金Supported by National High Level Hospital Clinical Research Funding,No.2022-PUMCH-B-022 and No.2022-PUMCH-D-002CAMS Innovation Fund for Medical Sciences,No.2021-1-I2M-003+1 种基金Undergraduate Innovation Program,No.2023-zglc-06034National Key Clinical Specialty Construction Project,No.ZK108000。
文摘BACKGROUND Autoimmune enteropathy(AIE)is a rare disease whose diagnosis and long-term prognosis remain challenging,especially for adult AIE patients.AIM To improve overall understanding of this disease’s diagnosis and prognosis.METHODS We retrospectively analyzed the clinical,endoscopic and histopathological characteristics and prognoses of 16 adult AIE patients in our tertiary medical center between 2011 and 2023,whose diagnosis was based on the 2007 diagnostic criteria.RESULTS Diarrhea in AIE patients was characterized by secretory diarrhea.The common endoscopic manifestations were edema,villous blunting and mucosal hyperemia in the duodenum and ileum.Villous blunting(100%),deep crypt lymphocytic infiltration(67%),apoptotic bodies(50%),and mild intraepithelial lymphocytosis(69%)were observed in the duodenal biopsies.Moreover,there were other remarkable abnormalities,including reduced or absent goblet cells(duodenum 94%,ileum 62%),reduced or absent Paneth cells(duodenum 94%,ileum 69%)and neutrophil infiltration(duodenum 100%,ileum 69%).Our patients also fulfilled the 2018 diagnostic criteria but did not match the 2022 diagnostic criteria due to undetectable anti-enterocyte antibodies.All patients received glucocorticoid therapy as the initial medication,of which 14/16 patients achieved a clinical response in 5(IQR:3-20)days.Immunosuppressants were administered to 9 patients with indications of steroid dependence(6/9),steroid refractory status(2/9),or intensified maintenance medication(1/9).During the median of 20.5 months of followup,2 patients died from multiple organ failure,and 1 was diagnosed with non-Hodgkin’s lymphoma.The cumulative relapse-free survival rates were 62.5%,55.6%and 37.0%at 6 months,12 months and 48 months,respectively.CONCLUSION Certain histopathological findings,including a decrease or disappearance of goblet and Paneth cells in intestinal biopsies,might be potential diagnostic criteria for adult AIE.The long-term prognosis is still unsatisfactory despite corticosteroid and immunosuppressant medications,which highlights the need for early diagnosis and novel medications.
基金Zhongyuan Talent Program,No.ZYYCYU202012113The Key R and D Program of Henan Province,No.222102310038.
文摘BACKGROUND Percutaneous drainage(PCD)and endoscopic approaches have largely replaced surgical drainage as the initial approach for(peri)pancreatic fluid collections(PFC)s,while complications associated with endoscopic stent implantation are common.AIM To introduce a novel endoscopic therapy named endoscopic transgastric fenestration(ETGF),which involves resection of tissue by endoscopic accessory between gastric and PFCs without stent implantation,and to evaluate its efficacy and safety compared with PCD for the management of PFCs adjacent to the gastric wall.METHODS Patients diagnosed with PFCs adjacent to the gastric wall and who subsequently received ETGF or PCD were restrospectively enrolled.Indications for intervention were consistent with related guidelines.We analyzed patients baseline characteristics,technical and clinical success rate,recurrence and reintervention rate,procedure-related complications and adverse events.RESULTS Seventy-two eligible patients were retrospectively identified(ETGF=34,PCD=38)from October 2017 to May 2021.Patients in the ETGF group had a significantly higher clinical success rate than those in the PCD group(97.1 vs 76.3%,P=0.01).There were no statistically significant differences regarding recurrence,reintervention and incidence of complication between the two groups.While long-term catheter drainage was very common in the PCD group.CONCLUSION Compared with PCD,ETGF has a higher clinical success rate in the management of PFCs adjacent to the gastric wall.ETGF is an alternative effective strategy for the treatment of PFCs adjacent to the gastric wall.
