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Revista Espanola de Enfermedades... May 2021Chronic diarrhea is a common symptom seen in the Gastroenterology clinic. Occasionally, the diagnosis is a real challenge as there are multiple entities with unremitting...
Chronic diarrhea is a common symptom seen in the Gastroenterology clinic. Occasionally, the diagnosis is a real challenge as there are multiple entities with unremitting diarrhea as a symptom. Herein, we present a patient affected with intractable diarrhea who was transferred to our department. After many laboratory, endoscopy and radiological tests, she was diagnosed with autoimmune enteropathy (AE) and achieved clinical remission with corticosteroids and azathioprine.
Topics: Azathioprine; Diarrhea; Female; Humans; Polyendocrinopathies, Autoimmune
PubMed: 33256418
DOI: 10.17235/reed.2020.7218/2020 -
Journal of Clinical Medicine Dec 2021It is well known that some pathological conditions, especially of autoimmune etiology, are associated with the HLA (human leukocyte antigen) phenotype. Among these... (Review)
Review
It is well known that some pathological conditions, especially of autoimmune etiology, are associated with the HLA (human leukocyte antigen) phenotype. Among these diseases, we include celiac disease, inflammatory bowel disease, autoimmune enteropathy, autoimmune hepatitis, primary sclerosing cholangitis and primary biliary cholangitis. Immunoglobulin G4-related diseases (IgG4-related diseases) constitute a second group of autoimmune gastrointestinal, hepatobiliary and pancreatic illnesses. IgG4-related diseases are systemic and rare autoimmune illnesses. They often are connected with chronic inflammation and fibrotic reaction that can occur in any organ of the body. The most typical feature of these diseases is a mononuclear infiltrate with IgG4-positive plasma cells and self-sustaining inflammatory response. In this review, we focus especially upon the hepatopancreatobiliary system, autoimmune pancreatitis and IgG4-related sclerosing cholangitis. The cooperation of the gastroenterologist, radiologist, surgeon and histopathologist is crucial for establishing correct diagnoses and appropriate treatment, especially in IgG4 hepatopancreatobiliary diseases.
PubMed: 34945093
DOI: 10.3390/jcm10245796 -
Nutrients Mar 2020Celiac disease (CD) is a chronic autoimmune enteropathy triggered by the ingestion of gluten in genetically predisposed individuals. At the time of diagnosis, the...
Celiac disease (CD) is a chronic autoimmune enteropathy triggered by the ingestion of gluten in genetically predisposed individuals. At the time of diagnosis, the frequency of nonalcoholic fatty liver disease (NAFLD) and nonalcoholic steatohepatitis in individuals with CD appears to be similar to that of the general population, although a lower body mass index and a lower rate of hypercholesterolemia and type 2 diabetes mellitus are observed at diagnosis in CD patients. The effect of a gluten-free diet (GFD) in individuals with these liver and metabolic disorders is still a matter of debate. The aim of this study was to investigate the links between a GFD and metabolic/liver disorders in CD patients. A systematic electronic search of the literature from January 2009 to December 2019 was performed using Medline, Web of Science, Scopus, and the Cochrane Library. Only papers written in English concerning metabolic and liver disorders in adult patients with CD were included. Out of 1195 citations, 14 eligible studies were identified. Increases in the frequency of NAFLD, weight gain, and alterations of the lipid profile suggest that important changes happen in celiac patients on a GFD, though the physiopathology of these conditions is unclear. Although a GFD is the only effective treatment available for CD, liver function, body weight, and metabolic and nutritional profiles should be monitored in patients on a GFD.
Topics: Adult; Body Mass Index; Celiac Disease; Comorbidity; Diabetes Mellitus, Type 2; Diet, Gluten-Free; Female; Humans; Hypercholesterolemia; Male; Metabolic Diseases; Non-alcoholic Fatty Liver Disease; Nutritional Physiological Phenomena
PubMed: 32231050
DOI: 10.3390/nu12040940 -
Digestive Diseases and Sciences Mar 2019Autoimmune enteropathy (AIE) is a complex disease affecting both children and adults. Although associated with significant morbidity and mortality, the pathophysiology... (Review)
Review
Autoimmune enteropathy (AIE) is a complex disease affecting both children and adults. Although associated with significant morbidity and mortality, the pathophysiology of the disease and its treatment have not been well characterized. This study aims to review the medical literature available on this rare but clinically significant ailment, to help establish a better understanding of its pathophysiology and enumerate the available diagnostic and treatment modalities. A literature search was conducted on PubMed using key terms related to autoimmune enteropathy and intractable diarrhea, with no restrictions on the date of publication or language. We found a total of 98 reports of AIE published in the form of case reports and case series. The evidence reviewed suggests that AIE is a multifaceted disorder that requires a high index of suspicion in the appropriate clinical setting to be able to make an early diagnosis. Current evidence supports the use of supportive care to correct nutritional and metabolic deficiencies, and immunosuppressives and immunomodulators as directed therapies. Hematopoietic stem cell transplant is an aggressive, but successful curative modality for patients with AIE as part of immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome. Cumulative clinical experience with management of AIE has allowed improved outcomes in transplanted and non-transplanted AIE patients even though morbidity and mortality with are still high in patients with this condition. More research is needed to further define the role of new therapies for AIE, and a central registry with participation of multiple institutions might help share and standardize care of patients with this rare but serious condition.
Topics: Adolescent; Autoimmunity; Child; Child, Preschool; Diagnosis, Differential; Female; Hematopoietic Stem Cell Transplantation; Humans; Immunosuppressive Agents; Infant; Male; Nutritional Support; Polyendocrinopathies, Autoimmune; Predictive Value of Tests; Treatment Outcome
PubMed: 30415406
DOI: 10.1007/s10620-018-5364-1 -
Archives of Pathology & Laboratory... Oct 2015Olmesartan is an antihypertensive medication belonging to the angiotensin II receptor blocker class of drugs that has recently been associated with severe enteropathy.... (Review)
Review
Olmesartan is an antihypertensive medication belonging to the angiotensin II receptor blocker class of drugs that has recently been associated with severe enteropathy. Olmesartan-associated enteropathy is uncommon and may be difficult to recognize because of its clinical and histologic similarities to other clinical entities, including celiac sprue and autoimmune enteropathy. The purpose of this article is to review the clinical and histologic findings of olmesartan-associated enteropathy that have been reported in the literature and to discuss clinical entities to consider in the differential diagnosis of olmesartan-associated enteropathy.
Topics: Antihypertensive Agents; Celiac Disease; Diagnosis, Differential; Diarrhea; Gastrointestinal Tract; Humans; Imidazoles; Intestinal Diseases; Polyendocrinopathies, Autoimmune; Tetrazoles; Withholding Treatment
PubMed: 26414468
DOI: 10.5858/arpa.2015-0204-RA -
Revista Espanola de Enfermedades... Nov 2023Autoimmune enteropathy is a rare intestinal disease. Here we report an elderly female with autoimmune enteropathy and primary biliary cholangitis who presented with...
Autoimmune enteropathy is a rare intestinal disease. Here we report an elderly female with autoimmune enteropathy and primary biliary cholangitis who presented with chronic diarrhea, malnutrition and severe hypokalemia and metabolic acidosis. Enteroscopy showed atrophied small intestinal villi with scallop-like and fissure-like changes. Hormone treatment relieved diarrhea. Four months later, she developed primary biliary cholangitis, and the liver function returned to normal after treatment with ursodeoxycholic acid, corticosteroids and immunosuppressants.
Topics: Aged; Humans; Female; Liver Cirrhosis, Biliary; Polyendocrinopathies, Autoimmune; Atrophy; Diarrhea; Rare Diseases
PubMed: 37771301
DOI: 10.17235/reed.2023.9901/2023 -
Clinical and Translational... Aug 2021Adult-onset autoimmune enteropathy (AIE) is a rare cause of severe chronic diarrhea because of small intestinal villous atrophy. We report on patients with adult-onset...
INTRODUCTION
Adult-onset autoimmune enteropathy (AIE) is a rare cause of severe chronic diarrhea because of small intestinal villous atrophy. We report on patients with adult-onset AIE in an European referral center.
METHODS
Retrospective study including patients diagnosed with AIE in the Amsterdam UMC, location VUmc, between January 2003 and December 2019. Clinical, serological, and histological features and response to treatment were reported. The specificity of antienterocyte antibodies (AEA) was evaluated by examining the prevalence of AEA in (i) controls (n = 30) and in patients with (ii) AIE (n = 13), (iii) celiac disease (CD, n = 52), (iv) refractory celiac disease type 2 (n = 18), and (v) enteropathy-associated T-cell lymphoma (EATL, n = 10).
RESULTS
Thirteen AIE patients were included, 8 women (62%), median age of 52 years (range 23-73), and 6 (46%) with an autoimmune disease. AEA were observed in 11 cases (85%), but were also found in CD (7.7%), refractory celiac disease type 2 (16.7%), and EATL (20%). Ten patients (77%) were human leukocyte antigen DQ2.5 heterozygous. Total parenteral nutrition was required in 8 cases (62%). Steroids induced clinical remission in 8 cases (62%). Step-up therapy with rituximab, cyclosporine, infliximab, and cladribine in steroid-refractory patients was only moderately effective. Four patients died (31%), but 4 (31%) others are in long-term drug-free remission after receiving immunosuppressive treatment, including 1 patient who underwent autologous stem cell transplantation.
DISCUSSION
Adult-onset AIE is a rare but severe enteropathy that occurs in patients susceptible for autoimmune disease. Four patients (31%) died secondary to therapy-refractory malabsorption, while immunosuppressive therapy leads to a long-lasting drug-free remission in one-third of patients.
Topics: Adult; Aged; Atrophy; Autoantibodies; Chronic Disease; Diarrhea; Duodenum; Enterocytes; Fatty Acid-Binding Proteins; Female; HLA-DQ Antigens; Humans; Immunosuppressive Agents; Male; Middle Aged; Netherlands; Parenteral Nutrition; Polyendocrinopathies, Autoimmune; Retrospective Studies; T-Lymphocyte Subsets; Tertiary Care Centers; Young Adult
PubMed: 34333499
DOI: 10.14309/ctg.0000000000000387 -
BMC Pediatrics Nov 2023Autoimmune enteropathy (AIE) defined by intractable diarrhoea and nonceliac enteropathy with villous atrophy, is a rare digestive disease. Case reports of this disease... (Review)
Review
BACKGROUND
Autoimmune enteropathy (AIE) defined by intractable diarrhoea and nonceliac enteropathy with villous atrophy, is a rare digestive disease. Case reports of this disease are sporadic and the clinical characteristics of AIE is seldom discussed.
PURPOSE
We evaluate the clinical, laboratory, histopathological features, response to therapy and outcome of AIE in children.
METHOD
We conducted a retrospective analysis of five children with AIE in our hospital. A comprehensive search of MEDLINE was performed using PubMed, through keywords of "autoimmune enteropathy, pediatric or children". The clinical manifestations, endoscopic results, pathological results, and medication therapy of these children were collected and the cases were divided into two groups, infants (≤ 1 year old) and children (> 1 year old).
RESULTS
Five cases treated in our department: one case took eight years to make the final diagnosis; one case was positive for anti-intestinal epithelial cell (AE) antibody; three cases showed crypt apoptosis in histopathology; and two cases showed celiac-like changes. All cases were responsive to glucocorticoid therapy in the early stage of treatment, while three cases required immunosuppressant maintenance. After reviewing the literature, we performed a statistical analysis of 50 cases with a male-to-female ratio of 31:19. Among them, 35 patients (70%) were within 1 year of age, and their clinical manifestations were mainly watery stool (43 cases, 86%), weight loss (28 cases, 56%), abdominal distension (3 cases, 6%), serum AE or anti-goblet cell (AG) antibody positivity (32 cases, 64%), other immune-related antibodies (21 cases, 42%), gene mutations (9 cases, 18%), and family history (21 cases, 42%). All the children showed different degrees of intestinal villous atrophy. Thirty-seven (74%) of the children were treated early, and their clinical symptoms were relieved. Comparing the cases between different age groups, it was found that the mortality rate of children with onset in infancy was higher (P < 0.05), and there was no difference in other autoimmune diseases, AE antibody positivity rates, and other antibodies between the two groups. In addition to survival rate between different age group (P = 0. 005), there was no difference in sex, autoantibody positivity rate, single gene mutation, or family history between the two groups (P > 0.05) through analysis of mortality and clinical remission cases.
CONCLUSION
Endoscopic examination and mucosal pathological examination should be performed to diagnose AIE in children with watery stool and weight loss who fail to be treated with diet therapy. Immunotherapy is the core of medical management of AIE and can improve prognosis. Children with a poor prognosis in infancy should be actively treated to reduce mortality rates associated with AIE.
Topics: Infant; Humans; Child; Male; Female; Retrospective Studies; Polyendocrinopathies, Autoimmune; Intestinal Diseases; Diarrhea; Atrophy; Weight Loss
PubMed: 38017413
DOI: 10.1186/s12887-023-04435-x -
Clinical Microbiology Reviews Jul 2014Celiac disease (CD) is a common chronic autoimmune enteropathy caused by gluten intake. To date, the only therapy for CD is the complete exclusion of dietary sources of... (Review)
Review
Celiac disease (CD) is a common chronic autoimmune enteropathy caused by gluten intake. To date, the only therapy for CD is the complete exclusion of dietary sources of grains and any food containing gluten. It has been hypothesized that the intestinal microbiota is somehow involved in CD. For this reason, probiotics are appearing as an interesting adjuvant in the dietetic management of CD. This review aims to discuss the characteristics of the microbiota in CD subjects and the use of probiotics as a novel therapy for CD. Comparisons between children with CD and controls show that their microbiota profiles differ; the former have fewer lactobacilli and bifidobacteria. Specific probiotics have been found to digest or alter gluten polypeptides. It has also been demonstrated that some bacterial species belonging to the genera Lactobacillus and Bifidobacterium exert protective properties on epithelial cells from damage caused by gliadin.
Topics: Animals; Celiac Disease; Humans; Intestines; Microbiota; Probiotics
PubMed: 24982318
DOI: 10.1128/CMR.00106-13 -
Frontiers in Pediatrics 2022
PubMed: 35273932
DOI: 10.3389/fped.2022.856920