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Life Science Alliance Jul 2024Celiac disease (CD) is an autoimmune enteropathy resulting from an interaction between diet, genome, and immunity. Although many patients respond to a gluten-free diet,...
Celiac disease (CD) is an autoimmune enteropathy resulting from an interaction between diet, genome, and immunity. Although many patients respond to a gluten-free diet, in a substantive number of individuals, the intestinal injury persists. Thus, other factors might amplify the ongoing inflammation. is a commensal fungus that is well adapted to the intestinal life. However, specific conditions increase pathogenicity. The hypothesis that may be a trigger in CD has been proposed after the observation of similarity between a fungal wall component and two CD-related gliadin T-cell epitopes. However, despite being implicated in intestinal disorders, may also protect against immune pathologies highlighting a more intriguing role in the gut. Herein, we postulated that a state of chronic inflammation associated with microbial dysbiosis and leaky gut are favorable conditions that promote pathogenicity eventually contributing to CD pathology via a mast cells (MC)-IL-9 axis. However, the restoration of immune and microbial homeostasis promotes a beneficial -MC cross-talk favoring the attenuation of CD pathology to alleviate CD pathology and symptoms.
Topics: Celiac Disease; Humans; Homeostasis; Candida albicans; Mast Cells; Gastrointestinal Microbiome; Dysbiosis; Candidiasis; Animals; Candida; Intestinal Mucosa
PubMed: 38719750
DOI: 10.26508/lsa.202302441 -
JPGN Reports Feb 2024Autoimmune enteropathy is a rare cause of chronic intractable diarrhea and is present in <1 in 100,000 infants. We report the case of a 9-month-old boy who presented...
Autoimmune enteropathy is a rare cause of chronic intractable diarrhea and is present in <1 in 100,000 infants. We report the case of a 9-month-old boy who presented with intractable diarrhea and vomiting. Genetic panel testing revealed a STAT3 heterozygous mutation in exon 6, suggesting infantile-onset multisystem autoimmune disease-1. The patient was initially treated with steroids and sulfasalazine. However, on tapering steroids, he had another episode of diarrhea and was subsequently put on baricitinib to which he responded.
PubMed: 38545278
DOI: 10.1002/jpr3.12038 -
BMC Nutrition Mar 2024Celiac disease (CD) is a systemic and autoimmune enteropathy of the gastrointestinal tract with malabsorption characteristics. The only effective treatment for CD is...
BACKGROUND/OBJECTIVES
Celiac disease (CD) is a systemic and autoimmune enteropathy of the gastrointestinal tract with malabsorption characteristics. The only effective treatment for CD is adhere strictly to a gluten-free diet (GFD) throughout life. We evaluated the dietary patterns in celiac disease patients and their association with dietary intakes and anthropometric measurements in Iran.
SUBJECTS/METHODS
This is a case-control study on 182 participants who were referred to the Khuzestan Celiac Association, Iran. Nutritional information was collected using a validated 147-item semi-quantitative food frequency questionnaire (FFQ). The software Stata (StataCorp, Version 14.0) was used to analyze the data. Principal component analysis (PCA) was used to obtain participants' dietary patterns.
RESULTS
A significant relationship was observed between age and body mass index (BMI) across quartiles of the healthy dietary pattern score (P < 0.001, P = 0.001, and P = 0.001, respectively), indicating that as age and BMI increased, participants demonstrated greater adherence to the healthy dietary pattern. Individuals with the highest adherence to the healthy dietary pattern had the lowest odds ratio for celiac disease (CD) (Q1: reference; Q2: 1.96, 95% CI: 0.84-4.55; Q3: 0.61, 95% CI: 0.27-1.42; Q4: 0.10, 95% CI: 0.03-0.33, P trend < 0.001), and this association remained significant after adjusting for BMI (adjusted P trend = 0.003) and energy intake (adjusted P trend < 0.001). Moreover, there was a significant association between the lowest odds ratio for CD and the highest adherence to the unhealthy dietary pattern after adjustment for energy intake (Q1: reference; Q2: 0.38, 95% CI: 0.13-1.12; Q3: 0.21, 95% CI: 0.06-0.71; Q4: 0.07, 95% CI: 0.02-0.29, adjusted P trend < 0.001). Additionally, a significant association was observed between the odds ratio for CD and the mixed dietary pattern score (Q1: reference; Q2: 6.01, 95% CI: 2.29-15.72; Q3: 2.47, 95% CI: 0.93-6.55; Q4: 4.84, 95% CI: 1.84-12.66, P trend = 0.02), and this association remained significant after adjustment for energy intake (adjusted P trend < 0.001).
CONCLUSIONS
The findings of the present study indicate that individuals who adhere to healthy dietary patterns have a lower incidence of celiac disease.
PubMed: 38448898
DOI: 10.1186/s40795-024-00849-7 -
Iranian Journal of Biotechnology Jul 2023Celiac disease (CD) is a gluten-sensitive chronic autoimmune enteropathy. A strict life-long gluten-free diet is the only efficient and accepted treatment until now....
BACKGROUND
Celiac disease (CD) is a gluten-sensitive chronic autoimmune enteropathy. A strict life-long gluten-free diet is the only efficient and accepted treatment until now. However, maintaining a truly gluten-free status is both difficult and costly, often resulting in a social burden for the person. Moreover, 2 to 5 percent of patients fail to improve clinically and histologically upon elimination of dietary gluten. Therefore, novel therapeutic approaches, including gluten degrading enzymes, are an unmet need of celiac patients.
OBJECTIVES
To evaluate the function of sunn pest prolyl endoprotease for gluten and gliadin hydrolysis in vitro.
MATERIALS AND METHODS
The spPEP was expressed as a recombinant protein in , and its catalytic activity was assessed by SDS-PAGE and RP-HPLC analyses.
RESULTS
Production of a 100-kDa spPEP protein was confirmed by SDS-PAGE and western blot analysis. Also, we demonstrate that spPEP efficiently degrades gluten and α-gliadin (30-40 kDa) in vitro under conditions similar to the GI and is resistant to pepsin and trypsin.
CONCLUSION
The gathered data demonstrated that spPEP might be a novel candidate for Oral Enzymatic Therapy (OET) in CD and other gluten-related disorders.
PubMed: 38344704
DOI: 10.30498/ijb.2023.347693.3420 -
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 -
Journal of Community Hospital Internal... 2023Autoimmune lupus enteritis in systemic lupus erythematosus (SLE) is a rare manifestation that comprises of gastrointestinal tract inflammation with supportive images...
Autoimmune lupus enteritis in systemic lupus erythematosus (SLE) is a rare manifestation that comprises of gastrointestinal tract inflammation with supportive images and/or biopsy findings. We report a unique case of widespread lupus enteritis occurring late in the disease process and in isolation without additional features of active SLE but in the presence of SLE serological activity. There was no clear evidence of active mesenteric vasculitis, intestinal pseudo-obstruction, protein-losing enteropathy, or coagulopathy by imaging or histopathology. This is the first reported case of an SLE patient with pan-gastrointestinal involvement of lupus enteritis responding to Belimumab, with complete resolution of the gastrointestinal syndrome and no further recurrence of gastrointestinal events. Rapid diagnosis and prompt immunomodulatory treatment of lupus-related enteritis are critical to avoid potentially life-threatening complications.
PubMed: 37868240
DOI: 10.55729/2000-9666.1185 -
Revista de Gastroenterologia de Mexico... 2024Celiac disease (CD) is an autoimmune enteropathy that develops in genetically susceptible individuals. The typical gastrointestinal manifestation is diarrhea but...
INTRODUCTION AND AIMS
Celiac disease (CD) is an autoimmune enteropathy that develops in genetically susceptible individuals. The typical gastrointestinal manifestation is diarrhea but symptoms of dyspepsia, such as epigastric pain, nausea, or satiety, can sometimes appear. Previous studies have reported that the prevalence of CD in patients with dyspepsia can be as high as 7%. The aim of the present study was to evaluate CD seroprevalence in subjects with dyspeptic symptoms and a control group in a Mexican population.
MATERIAL AND METHODS
A case-control study was conducted on blood donors that answered the PAGI-SYM questionnaire for dyspepsia and in whom IgA antibodies to tissue transglutaminase 2 (IgA anti-tTG2) and IgG antibodies to deamidated gliadin peptide (IgG anti-DGP) were determined. CD seroprevalence in subjects with dyspeptic symptoms and in asymptomatic subjects was compared.
RESULTS
A total of 427 subjects (76.3% men), with a mean patient age of 34 years (range of 18-65 years) were included. Of those participants, 87 (20.3%) had symptoms of dyspepsia (group A) and 340 (79.6%) were asymptomatic (group B). Antibodies were positive in one (1.15%) of the group A subjects (1/87, 95% CI 0.2-6 %), whereas they were positive in 4 (1.18%) of the group B subjects (4/340, 95% CI 0.4-2.9%, p = 0.59).
CONCLUSIONS
CD seroprevalence in the study population with dyspeptic symptoms (1%) was not different from that of the control population. Thus, CD screening in Mexican patients with dyspepsia is not justified.
Topics: Humans; Male; Adult; Female; Mexico; Middle Aged; Seroepidemiologic Studies; Celiac Disease; Case-Control Studies; Adolescent; Young Adult; Dyspepsia; Aged; Immunoglobulin G; Immunoglobulin A; Transglutaminases; Protein Glutamine gamma Glutamyltransferase 2; Autoantibodies
PubMed: 37833136
DOI: 10.1016/j.rgmxen.2023.05.011 -
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... 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 Jul 2023Autoimmune enteropathy (AIE) is a differential diagnosis of incurable chronic diarrhea, malnutrition, and weight loss. This type of diarrhea is associated with protein...
Autoimmune enteropathy (AIE) is a differential diagnosis of incurable chronic diarrhea, malnutrition, and weight loss. This type of diarrhea is associated with protein enteropathy that usually affects the small intestine. The diagnosis of AIE is based on chronic diarrhea, malabsorption, specific histological result, antibodies against enterocytes, and excluding similar conditions. In this case, a 28-year-old female presented with diarrhea, lower limb edema, weight loss, and electrolyte imbalances. Endoscopic examination demonstrated duodenal villous atrophy, while duodenal biopsies revealed villous blunting, scattered intraepithelial lymphocytes, and crypt hyperplasia in the lamina propria. The patient was treated with immunosuppressive treatment including methylprednisolone and azathioprine, achieving clinical remission.
PubMed: 37637519
DOI: 10.7759/cureus.42538