China
Africa
Explore chapters and articles related to this topic
Immunomodulating Agents in Gastrointestinal Disease
Published in Thomas F. Kresina, Immune Modulating Agents, 2020
Samir A. Shah, Athos Bousvaros, A. Christopher Stevens
Autoimmune Enteropathy Autoimmune entropathy (AE) is an idiopathic intestinal inflammatory disease occurring in children, characterized by secretory diarrhea, villous atrophy, and evidence of other systemic autoimmune disease (including glomerulonephritis, diabetes, and adrenal insufficiency). Immune abnormalities identified in AE include T lymphocyte activation and antienterocyte antibodies. Whereas corticosteroids and azathioprine have limited efficacy in this disorder, CSA has improved villous histological characteristics, nutrient absorption, and growth [154,155].
Gastroenterology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
An acute onset of jaundice occurs in most patients. It may present insidiously with malaise, fatigue, anorexia and nausea. Hepatosplenomegaly is found in up to 80% of patients. Patients may develop symptoms secondary to other organ-specific autoimmune disorders (see ‘Autoimmune enteropathy’, page 263) or IBD.
Approach to Pediatric Diarrhea
Published in John F. Pohl, Christopher Jolley, Daniel Gelfond, Pediatric Gastroenterology, 2014
Karolina Maria Burghardt, Tanja Gonska
The intestinal barrier that is needed to regulate nutrient absorption can be defective and/or have increased permeability as a result of immunologic processes. Autoimmune enteropathy is characterized by immunoglobulin (Ig) G antibodies specific for components of the enterocyte brush border that initiate a cell-mediated immune dysregulation and damage of the epithelial barrier. Similarly, children with a mutation of the transcription factor FoxP3 (scurfin) which modulates CD4+ T cell proliferation have a propensity for having antienterocyte antibodies resulting in immune dysregulation polyendocrinopathy, enteropathy syndrome. Moreover, the state of inflammation that waxes and wanes in the intestine of patients with inflammatory bowel disease (IBD), ulcerative colitis (56.5), or Crohn disease (56.6) leads to a fluctuating degree of diarrhea.
Diagnostic and therapeutic challenge of unclassifiable enteropathies with increased intraepithelial CD103+ CD8+ T lymphocytes: a single center case series
Published in Scandinavian Journal of Gastroenterology, 2021
Christina Hartl, Jürgen Finke, Peter Hasselblatt, Wolfgang Kreisel, Annette Schmitt-Graeff
Chronic enteropathy associated with intraepithelial lymphocytosis (IEL) and villous atrophy (VA) of the duodenal mucosa and persistent diarrhea may result from many pathologies with celiac disease (CD) being the most prevalent underlying cause. In the absence of CD auto-antibodies and after exclusion of the clinically challenging diagnosis of seronegative CD, e.g., by exposure to gluten-free diet and HLA testing [1], a plethora of differential diagnoses have to be considered in patients with VA and IEL. Gastrointestinal pathology frequently occurs in primary immunodeficiency disorders (PID) such as the IPEX (immune dysregulation polyendocrinopathy enteropathy X-linked) or APECED syndromes (autoimmune polyendocrinopathy candidiasis ectodermal dystrophy) and CVID (common variable immunodeficiency) [2,3]. Potential causes also include medications such as olmesartan or immune checkpoint inhibitors, Crohn’s disease, infections (in particular, HIV infection, mycobacteriosis, giardiasis, Whipple’s disease), food intolerances or autoimmune enteropathy (AIE) [4,5]. The diagnostic work-up should therefore include blood tests for assessing malnutrition, ruling out seropositive or seronegative CD and testing for anti-enterocyte antibodies. In addition, microbiological examinations and endoscopies with duodenal biopsies are mandatory during diagnostic work-up to rule out infections, inflammatory bowel disease or lymphoproliferative disorders (LPD) [6,7].
Efficacy and safety of idelalisib for the treatment of indolent B-cell malignancies
Published in Expert Opinion on Pharmacotherapy, 2020
Piotr Smolewski, Dominika Rydygier
To summarize, it is recommended that a patient history of inflammatory bowel diseases or microscopic colitis of functional gastrointestinal disorders should be carefully collected in candidates with CLL or FL before the beginning of idelalisib treatment. Patients should be clinically monitored for the appearance of diarrhea or colitis during treatment. Any case of diarrhea should be tested to exclude infection as a cause. Colonoscopy and gastroenterological referrals with biopsy are recommended in the event of no response to treatment or bloody diarrhea. The knowledge of the histologic assessment is significant to distinguish enterocolitis from potential mimics, particularly graft-versus-host disease, autoimmune enteropathy, and cytomegalovirus/infectious enterocolitis [46]. Idelalisib dosing should be withheld when diarrhea grade 2 or 3 persists for more than 2 days. However, it should be continued and antibiotic therapy started in cases of grade 1 or 2 diarrhea of infective etiology, but when symptoms remain unresolved after 48 hours, steroids should be started after the exclusion of any infective etiology. In cases of satisfactory response is recommended to resume idelalisib with reduced dose, but in patients with unexplained and/or persistent gastrointestinal disorders, gastroenterological referral is advisable [39].
Two cases of monomorphic epitheliotropic intestinal T-cell lymphoma associated with coeliac disease
Published in Scandinavian Journal of Gastroenterology, 2019
Marco Vincenzo Lenti, Federico Biagi, Marco Lucioni, Antonio Di Sabatino, Marco Paulli, Gino Roberto Corazza
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), is an aggressive form of extranodal lymphoma arising from intraepithelial T-lymphocytes [1]. Unlike enteropathy-associated T-cell lymphoma (EATL) [2], MEITL is characterised by an epitheliotropic infiltration of monomorphic, CD8/CD56 positive, small- to medium-sized T-cells, in the absence of inflammation [1]. Although previous studies presumed, on a clinical basis only, an association with coeliac disease (CD) [3,4], this has been officially denied by the latest World Health Organisation classification of lymphoid neoplasms, and MEITL is now considered a sporadic form of lymphoma [1]. However, that of enteropathy-associated lymphomas is a grey area with blurred boundaries: EATL, known to be associated with CD, has been described in two patients with autoimmune enteropathy [5,6], and a case of MEITL concomitant to ulcerative colitis has been recently reported [7].