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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 -
Frontiers in Endocrinology 2021Thymoma is a type of mediastinal malignant tumors which always associated with autoimmune diseases. Although surgery is the predominant treatment method for thymoma, the...
INTRODUCTION
Thymoma is a type of mediastinal malignant tumors which always associated with autoimmune diseases. Although surgery is the predominant treatment method for thymoma, the pathogenesis of thymoma and thymoma-associated autoimmune diseases is still unknown. However, the case study here provided a possible pathogenesis and treatment to cure the thymoma with autoimmune enteropathy and myocarditis.
CASE PRESENTATION
A thymoma case with autoimmune enteropathy and myocarditis undergoing surgery was reported. The symptoms and laboratory results of the patient had dramatically fluctuated after tumor resection and gradually alleviated. The whole exome sequencing found amplified in tumor cells. Immunohistochemistry indicated that thymoma cells were positive for MDM4. The result of drug sensitivity tests showed thymoma cells were highly sensitive to Nutlin-3a.
CONCLUSION
could play an important role in the pathogenesis of this thymoma case with autoimmune enteropathy and myocarditis. This discovery may provide a novel idea of pathogenesis and treatment for thymoma and autoimmune diseases.
Topics: Adult; Cell Cycle Proteins; Colon; Humans; Intestine, Small; Male; Myocarditis; Polyendocrinopathies, Autoimmune; Proto-Oncogene Proteins; Thymoma; Thymus Neoplasms; Young Adult
PubMed: 34054730
DOI: 10.3389/fendo.2021.661316 -
Expert Review of Clinical Immunology Aug 2016Celiac Disease is an autoimmune enteropathy with increasing incidence worldwide in both adults and children. It occurs as an inflammatory condition with destruction of... (Review)
Review
Celiac Disease is an autoimmune enteropathy with increasing incidence worldwide in both adults and children. It occurs as an inflammatory condition with destruction of the normal architecture of villi on consumption of gluten and related protein products found in wheat, barley and rye. However, the exact pathogenesis is not yet fully understood. A gluten-free diet remains the main modality of therapy to date. While some patients continue to have symptoms even on a gluten-free diet, adherence to this diet is also difficult, especially for the children. Hence, there is continued interest in novel methods of therapy and the current research focus is on the promising novel non-dietary modalities of treatment. Here, we critically reviewed the existing literature regarding the pathogenesis of celiac disease in children including the role of in-utero exposure leading to neonatal and infant sensitization and its application for the development of new therapeutic approaches for these patients.
Topics: Adult; Algorithms; Autoimmunity; Celiac Disease; Child; Chorionic Villi; Diet Therapy; Genetic Predisposition to Disease; Glutens; Humans; Infant; Infant, Newborn; Intestinal Mucosa
PubMed: 26999328
DOI: 10.1586/1744666X.2016.1168294 -
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 -
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 -
Gastroenterology Research Jun 2018An increasing number of drugs including monoclonal antibodies and small molecules, either anti-inflammatory or immunity-enhancing, have been developed to treat human... (Review)
Review
An increasing number of drugs including monoclonal antibodies and small molecules, either anti-inflammatory or immunity-enhancing, have been developed to treat human diseases and the number of medications in these classes is likely to expand in the future. The two most commonly used categories of such therapies are the anti-inflammatory group (anti- tumor necrosis factor (TNF) α, anti-interleukins/interleukin receptors, and anti-integrin bodies) and the anti-tumoral agents (immune checkpoint inhibitors, anti-CD20, and anti-endothelial growth factor). Although the anti-inflammatory biologics have brought about a revolutionary effect in the management of a variety of autoimmune disorders including rheumatologic diseases, inflammatory bowel disease, and inflammatory dermatological diseases, their ability to induce colitis in patients without a prior history of colitis or exacerbate quiescent colitis has been increasingly and unexpectedly recognized. While the use of immune-augmenting monoclonal antibody therapies results in a significant survival benefit in a subset of patients with malignancies, these monoclonal antibodies also have the ability to cause colitis through an apparent autoimmune mechanism. Colitis associated with these medications may demonstrate multiple histologic patterns including increased apoptosis (graft versus host disease (GVHD)-like), autoimmune enteropathy pattern, acute colitis pattern, ischemic colitis, inflammatory bowel disease pattern, either ulcerative colitis-like, Crohn's disease-like, or fulminant colitis-like. In addition, anti-inflammatory biologics are known to cause or reactivate latent infections such as tuberculosis and increase the risk for malignancies including high-grade lymphomas as well as indolent lymphoproliferative disorders. Thus, the differential diagnosis for colitis in patients receiving therapeutic anti-inflammatory biologics or anti-tumoral agents can be broad. Optimal diagnosis and treatment requires a multidisciplinary approach. This review aims to provide an overview of the literature on the clinical features, histology, and treatment of these newly recognized anti-inflammatory biologic and anti-tumoral immune therapy-induced colitises and hopes this outlines will raise the vigilance of all clinicians of these entities.
PubMed: 29915627
DOI: 10.14740/gr1041w -
World Journal of Gastroenterology Sep 2014The gastrointestinal tract is responsible for a multitude of digestive and immune functions which depend upon the balanced interaction of the intestinal microbiota,... (Review)
Review
The gastrointestinal tract is responsible for a multitude of digestive and immune functions which depend upon the balanced interaction of the intestinal microbiota, diet, gut barrier function, and mucosal immune response. Disruptions in one or more of these factors can lead to intestinal disorders or enteropathies which are characterized by intestinal inflammation, increased gut permeability, and reduced capacity to absorb nutrients. Enteropathy is frequently associated with human immunodeficiency virus (HIV) infection, inflammatory bowel disease, autoimmune enteropathy, radiation enteritis, and irritable bowel syndrome (IBS), where pathologic changes in the intestinal tract lead to abdominal discomfort, bloating, abnormal bowel function (e.g., diarrhea, urgency, constipation and malabsorption). Unfortunately, effective therapies for the management of enteropathy and restoring intestinal health are still not available. An accumulating body of preclinical studies has demonstrated that oral administration of plasma- or serum-derived protein concentrates containing high levels of immunoglobulins can improve weight, normalize gut barrier function, and reduce the severity of enteropathy in animal models. Recent studies in humans, using serum-derived bovine immunoglobulin/protein isolate, demonstrate that such protein preparations are safe and improve symptoms, nutritional status, and various biomarkers associated with enteropathy. Benefits have been shown in patients with HIV infection or diarrhea-predominant IBS. This review summarizes preclinical and clinical studies with plasma/serum protein concentrates and describes the effects on host nutrition, intestinal function, and markers of intestinal inflammation. It supports the concept that immunoglobulin-containing protein preparations may offer a new strategy for restoring functional homeostasis in the intestinal tract of patients with enteropathy.
Topics: Administration, Oral; Animals; Dietary Proteins; Humans; Immunoglobulins; Inflammation Mediators; Intestinal Absorption; Intestinal Diseases; Intestinal Mucosa; Intestines; Microbiota; Nutritional Status; Nutritional Support; Permeability; Treatment Outcome
PubMed: 25206275
DOI: 10.3748/wjg.v20.i33.11713 -
Intestinal Research Jan 2022Autoimmune enteropathy (AIE) is a rare disease, characterized by intractable diarrhea, villous atrophy of the small intestine, and the presence of circulating...
Autoimmune enteropathy (AIE) is a rare disease, characterized by intractable diarrhea, villous atrophy of the small intestine, and the presence of circulating anti-enterocyte autoantibodies. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, and mutations in FOXP3, which is a master gene of regulatory T cells (Tregs), are major causes of AIE. Recent studies have demonstrated that mutations in other Treg-associated genes, such as CD25 and CTLA4, show an IPEX-like phenotype. We present the case of a 13-year-old girl with CTLA4 haploinsufficiency, suffering from recurrent immune thrombocytopenic purpura and intractable diarrhea. We detected an autoantibody to the AIE-related 75 kDa antigen (AIE-75), a hallmark of the IPEX syndrome, in her serum. She responded well to a medium dose of prednisolone and a controlled dose of 6-mercaptopurine (6-MP), even after the cessation of prednisolone administration. Serum levels of the soluble interleukin-2 receptor and immunoglobulin G (IgG) were useful in monitoring disease activity during 6-MP therapy. In conclusion, autoimmune-mediated mechanisms, similar to the IPEX syndrome, may be involved in the development of enteropathy in CTLA4 haploinsufficiency. Treatment with 6-MP and monitoring of disease activity using serum levels of soluble interleukin-2 receptor and IgG is suggested for such cases.
PubMed: 33476510
DOI: 10.5217/ir.2020.00041 -
Nutrients Aug 2018Increased antibody reactivity towards self-antigens is often indicative of a disruption of homeostatic immune pathways in the body. In celiac disease, an autoimmune... (Review)
Review
Increased antibody reactivity towards self-antigens is often indicative of a disruption of homeostatic immune pathways in the body. In celiac disease, an autoimmune enteropathy triggered by the ingestion of gluten from wheat and related cereals in genetically predisposed individuals, autoantibody reactivity to transglutaminase 2 is reflective of the pathogenic role of the enzyme in driving the associated inflammatory immune response. Autoantibody reactivity to transglutaminase 2 closely corresponds with the gluten intake and clinical presentation in affected patients, serving as a highly useful biomarker in the diagnosis of celiac disease. In addition to gastrointestinal symptoms, celiac disease is associated with a number of extraintestinal manifestations, including those affecting skin, bones, and the nervous system. Investigations of these manifestations in celiac disease have identified a number of associated immune abnormalities, including B cell reactivity towards various autoantigens, such as transglutaminase 3, transglutaminase 6, synapsin I, gangliosides, and collagen. Clinical relevance, pathogenic potential, mechanism of development, and diagnostic and prognostic value of the various identified autoantibody reactivities continue to be subjects of investigation and will be reviewed here.
Topics: Autoantibodies; Autoantigens; Celiac Disease; GTP-Binding Proteins; Gangliosides; Genetic Predisposition to Disease; Glutens; Humans; Prognosis; Protein Glutamine gamma Glutamyltransferase 2; Synapsins; Transglutaminases; Wheat Hypersensitivity
PubMed: 30127251
DOI: 10.3390/nu10081123 -
ACG Case Reports Journal Nov 2019Autoimmune enteropathy is a rare condition seen in adults with limited therapeutic options available. It manifests with profuse diarrhea and malnourishment. The...
Autoimmune enteropathy is a rare condition seen in adults with limited therapeutic options available. It manifests with profuse diarrhea and malnourishment. The diagnosis is made through a combination of clinical, serologic, and histologic parameters. The cornerstone of therapy revolves around nutritional optimization and immunosuppression, most commonly in the form of corticosteroids. Alternate therapies, such as antitumor necrosis factor agents, can be considered if there is an inadequate response to steroids. We report a case of autoimmune enteropathy that was successfully treated with adalimumab, a rare treatment for an infrequent disease.
PubMed: 32309471
DOI: 10.14309/crj.0000000000000265