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Best Practice & Research. Clinical... Oct 2012Small intestinal diseases are a common, though often overlooked cause of diarrhoeal illness. Fully 1% of the Caucasian population are affected by coeliac disease and a... (Review)
Review
Small intestinal diseases are a common, though often overlooked cause of diarrhoeal illness. Fully 1% of the Caucasian population are affected by coeliac disease and a substantial portion of children living in poverty in the developing world are affected by environmental enteropathy. These are but two examples of the many diseases that cause mucosal injury to the primary digestive and absorptive organ in our body. While diarrhoea may be a common, though not universally seen symptom of small bowel mucosal disease, the consequent malabsorption can lead to substantial malnutrition and nutrient deficiencies. The small intestine, unlike the colon, has been relatively inaccessible, and systematic evaluation is often necessary to identify and treat small intestinal mucosal diseases that lead to diarrhoea. Immunodeficiency states, including HIV enteropathy, adult autoimmune enteropathy, drug-associated enteropathy, and tropical sprue continue to occur and require specific therapy. All patients with severe diarrhoea or diarrhoea associated with features suggestive of malabsorption may have a disease of the small intestinal mucosa that requires careful evaluation and targeted management.
Topics: Algorithms; Anti-Bacterial Agents; Blind Loop Syndrome; Celiac Disease; Chronic Disease; Diarrhea; Humans; Intestine, Small; Malabsorption Syndromes; Polyendocrinopathies, Autoimmune; Sprue, Tropical; Whipple Disease
PubMed: 23384804
DOI: 10.1016/j.bpg.2012.11.013 -
Medicina (Kaunas, Lithuania) Sep 2019Celiac disease (CD) is an autoimmune enteropathy that primarily affects the small intestine and is characterized by atrophy of intestinal villi. The manifestations of... (Review)
Review
Celiac disease (CD) is an autoimmune enteropathy that primarily affects the small intestine and is characterized by atrophy of intestinal villi. The manifestations of the disease improve following a gluten-free diet (GFD). CD is associated with various extra-intestinal diseases. Several skin manifestations are described in CD patients. The present paper reviews all CD-associated skin diseases reported in the literature and tries to analyze the pathogenic mechanisms possibly involved in these associations. Different hypotheses have been proposed to explain the possible mechanisms involved in every association between CD and cutaneous manifestations. An abnormal small intestinal permeability seems to be implicated in various dermatological manifestations. However, most of the associations between CD and cutaneous diseases is based on case reports and case series and a few controlled studies. To better assess the real involvement of the cutaneous district in CD patients, large multicentric controlled clinical trials are required.
Topics: Celiac Disease; Humans; Skin; Skin Diseases
PubMed: 31505858
DOI: 10.3390/medicina55090578 -
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 -
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 -
Clinical and Translational... Dec 2023Persistent villous atrophy (VA) is not uncommon in celiac disease (CeD) while patients take a gluten-free diet (GFD).
INTRODUCTION
Persistent villous atrophy (VA) is not uncommon in celiac disease (CeD) while patients take a gluten-free diet (GFD).
METHODS
We conducted a retrospective study with 122 serum samples collected from controls and patients with CeD either at the initial diagnosis or at the follow-up during endoscopy. These samples were assigned to 3 groups: nonceliac control, non-VA CeD (Marsh score 0-2), and VA CeD (Marsh score 3a-3c). We established an in-house multiplex assay to identify potential serological biomarkers for VA. We assessed autoantibodies reported to affect the small intestine, including IgA and IgG antibodies against tissue transglutaminase (tTG), interferons, villin, actin, autoimmune enteropathy-related 75 kDa antigen (AIE-75), and tryptophan hydroxylase (TPH)-1, as well as 27 cytokines. The apolipoproteins quantified included apo A1, apo B-100, and apo A4, which were produced predominantly by the intestinal epithelium or expressed specifically in villi.
RESULTS
Autoantibody levels were high only for tTG antibodies, which performed well in initial CeD diagnosis, but suboptimally for VA prediction during follow-up, because 14.6% of the follow-up patients with VA had low tTG-IgA. Increasing dilution improved tTG-IgA quantification, particularly when the antibody levels were extremely high but did not significantly improve VA detection. Among those with low tTG-IgA and persistent VA, high proinflammatory cytokines were observed in 2 patients. Median low-density lipoprotein cholesterol levels were significantly lower in the VA CeD group ( P = 0.03). Apolipoprotein levels were similar in patients with and without VA but diverged between those on a GFD or not.
DISCUSSION
tTG-IgA as a biomarker is suboptimal for VA prediction while on a GFD. Persistent VA is associated with low low-density lipoprotein cholesterol levels and partially related to persistent high proinflammatory cytokines.
Topics: Humans; Celiac Disease; Retrospective Studies; Transglutaminases; Biomarkers; Autoantibodies; Immunoglobulin A; Atrophy; Cytokines; Lipoproteins, LDL; Cholesterol
PubMed: 37753949
DOI: 10.14309/ctg.0000000000000639 -
Cureus May 2023Small bowel villous atrophy is most often caused by celiac disease in the Western world, but other diseases should be explored in patients without positive serology....
Small bowel villous atrophy is most often caused by celiac disease in the Western world, but other diseases should be explored in patients without positive serology. Adult-onset autoimmune enteropathy (AIE) is a rare cause of villous atrophy first known in children with T-cell dysregulation but also seen in adults with autoimmune predispositions. Here, an 82-year-old woman with autoimmune thyroiditis was admitted with weight loss and watery diarrhoea not responding to diet change. Endoscopy revealed villous atrophy both in the duodenum and in the ileum, but no positive celiac serology. A diagnosis of autoimmune enteropathy was made based on chronic diarrhoea not responding to diet change, autoimmune predisposition, villous atrophy, typical histological findings, and no evidence of immunodeficiency or medications causing villous atrophy. The patient was treated to good effect with corticosteroids but needed total parenteral nutrition while admitted. AIE should be considered in villous atrophy without positive celiac serology.
PubMed: 37398811
DOI: 10.7759/cureus.39677 -
Nutrients Jan 2010The prevalence of celiac disease (CD), an autoimmune enteropathy, characterized by chronic inflammation of the intestinal mucosa, atrophy of intestinal villi and several... (Review)
Review
The prevalence of celiac disease (CD), an autoimmune enteropathy, characterized by chronic inflammation of the intestinal mucosa, atrophy of intestinal villi and several clinical manifestations has increased in recent years. Subjects affected by CD cannot tolerate gluten protein, a mixture of storage proteins contained in several cereals (wheat, rye, barley and derivatives). Gluten free-diet remains the cornerstone treatment for celiac patients. Therefore the absence of gluten in natural and processed foods represents a key aspect of food safety of the gluten-free diet. A promising area is the use of minor or pseudo-cereals such as amaranth, buckwheat, quinoa, sorghum and teff. The paper is focused on the new definition of gluten-free products in food label, the nutritional properties of the gluten-free cereals and their use to prevent nutritional deficiencies of celiac subjects.
Topics: Celiac Disease; Diet, Gluten-Free; Edible Grain; Food Labeling; Food Safety; Humans; Malnutrition
PubMed: 22253989
DOI: 10.3390/nu20100016 -
Revista Espanola de Enfermedades... Jan 2024Celiac disease (CD) is a chronic autoimmune enteropathy triggered by gluten intake. Celiac hepatitis is the most common hepatic manifestation of CD, it usually responds... (Observational Study)
Observational Study
Celiac disease (CD) is a chronic autoimmune enteropathy triggered by gluten intake. Celiac hepatitis is the most common hepatic manifestation of CD, it usually responds to a gluten-free diet (GFD) and is sometimes the only manifestation in paucisymptomatic CD. Through this descriptive observational study, we determined the prevalence of liver abnormalities upon diagnosis of CD. A total of 140 patients were included. The prevalence of alterations in liver markers at diagnosis of CD was 47%. In 2.9% of patients, liver abnormalities were the only manifestation at diagnosis. A higher prevalence of liver alterations was found in those patients who presented a more severe histological alteration (MARSH 3c).
Topics: Humans; Celiac Disease; Liver Diseases; Diet, Gluten-Free; Biopsy; Inflammatory Bowel Diseases
PubMed: 37204091
DOI: 10.17235/reed.2023.9516/2023 -
Archives of Pathology & Laboratory... Jan 2018- Patients who receive an upper gastrointestinal endoscopic examination frequently have biopsies taken from the duodenum. Accurate interpretation of duodenal biopsies is... (Review)
Review
CONTEXT
- Patients who receive an upper gastrointestinal endoscopic examination frequently have biopsies taken from the duodenum. Accurate interpretation of duodenal biopsies is essential for patient care. Celiac disease is a common clinical concern, but pathologists need to be aware of other conditions of the duodenum that mimic celiac disease.
OBJECTIVE
- To review the normal histologic features of duodenal mucosa and describe the clinical and histologic findings in celiac disease and its mimics, listing the differentiating features of biopsies with villous atrophy and epithelial lymphocytosis.
DATA SOURCES
- The study comprises a literature review of pertinent publications as of November 30, 2016.
CONCLUSIONS
- Celiac disease is a common cause of abnormal duodenal histology. However, many of the histologic features found in the duodenal biopsy of patients with celiac disease are also present in other conditions that affect the small bowel. Diagnostic precision may be enhanced by obtaining a careful patient history and by ancillary laboratory testing, particularly for the presence of antitissue transglutaminase antibodies.
Topics: Angiotensin II Type 1 Receptor Blockers; Anti-Inflammatory Agents, Non-Steroidal; Biopsy; Blind Loop Syndrome; Celiac Disease; Duodenitis; Graft vs Host Disease; Humans; Immunosuppressive Agents; Inflammatory Bowel Diseases; Intestinal Mucosa; Milk Hypersensitivity; Polyendocrinopathies, Autoimmune; Soybean Proteins
PubMed: 28758791
DOI: 10.5858/arpa.2016-0608-RA -
Histopathology Jan 2007Intestinal malabsorption results from a wide variety of causes, which can most easily be organized into three groups. Maldigestion arises from problems with mixing or... (Review)
Review
Intestinal malabsorption results from a wide variety of causes, which can most easily be organized into three groups. Maldigestion arises from problems with mixing or with digestive mediators, and includes post-gastrectomy patients and those with deficiencies of pancreatic or intestinal enzymes, or of bile salts. Mucosal and mural causes of malabsorption are abundant, and include gluten-sensitive enteropathy, tropical sprue, autoimmune enteropathy, and HIV/AIDS-related enteropathy, as well as mural conditions such as systemic sclerosis. Finally, microbial causes of malabsorption include bacterial overgrowth, Whipple's disease, and numerous infections or infestations that are most frequently seen in immunocompromised patients. An overview of the most common and interesting entities in each of these categories follows, along with a discussion of current concepts. Mucosal conditions and microbial causes of malabsorption are given special attention.
Topics: Humans; Intestinal Absorption; Intestinal Mucosa; Malabsorption Syndromes
PubMed: 17204022
DOI: 10.1111/j.1365-2559.2006.02547.x