Autoimmune enteropathy

An autoimmune disorder that primarily affects infants and young children. It is characterized by severe gastrointestinal symptoms, such as diarrhea and vomiting , that result in MALNUTRITION.

Networked: 231 relevant articles (7 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Diarrhea
2.	Inflammatory Bowel Diseases (Inflammatory Bowel Disease)
3.	Graft vs Host Disease (Graft-Versus-Host Disease)
4.	Cytomegalovirus Infections (Inclusion Disease)
5.	Rupture

Experts

1.	Fasano, Alessio: 10 articles (01/2021 - 02/2006)
2.	Kobayashi, Ichiro: 6 articles (01/2022 - 08/2004)
3.	Alaedini, Armin: 5 articles (01/2021 - 02/2008)
4.	Serena, Gloria: 4 articles (01/2021 - 08/2015)
5.	Corazza, Gino R: 4 articles (04/2018 - 12/2009)
6.	Sapone, Anna: 4 articles (03/2011 - 02/2006)
7.	Cerf-Bensussan, Nadine: 3 articles (01/2022 - 01/2015)
8.	Elli, Luca: 3 articles (01/2021 - 06/2016)
9.	Green, Peter H: 3 articles (01/2021 - 12/2016)
10.	Uhde, Melanie: 3 articles (01/2021 - 12/2016)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Autoimmune enteropathy:

1.	Cyclosporine (Ciclosporin)FDA LinkGeneric 11/01/1991 - "Cyclosporin A is useful in the treatment of autoimmune enteropathy. " 11/01/1991 - "Two children with autoimmune enteropathy were treated with cyclosporin A for eight months. " 12/01/1990 - "Successful treatment of autoimmune enteropathy with cyclosporine." 11/01/1991 - "Response to autoimmune enteropathy to cyclosporin A therapy." 01/01/2003 - "This constitutes the first report of effective treatment of adult autoimmune enteropathy with tacrolimus, a substance with a similar mode of action to cyclosporine, but with fewer side effects and improved bioavailability."
2.	Tacrolimus (Prograf)FDA LinkGeneric 01/01/2003 - "Adult autoimmune enteropathy treated successfully with tacrolimus." 07/01/1996 - "No long-term follow-up of patients with autoimmune enteropathy treated with tacrolimus is currently available." 07/01/1996 - "Treatment of pediatric autoimmune enteropathy with tacrolimus (FK506)." 05/01/1997 - "Autoimmune enteropathy in a pediatric patient: partial response to tacrolimus therapy." 02/01/2022 - "A 28-year-old male with common variable immunodeficiency disease, atopic dermatitis, autoimmune gastroenteropathy, inflammatory polyarthropathy, primary bone marrow failure, colon cancer, and family history of Lynch syndrome was admitted to our hospital for an acute flare of autoimmune enteropathy secondary to subtherapeutic tacrolimus levels. "
3.	Adrenal Cortex Hormones (Corticosteroids)IBA 10/01/2019 - "She was diagnosed with autoimmune enteropathy, and treatment consisted of an extended corticosteroid taper, with the resolution of symptoms." 09/01/2012 - "Mesenchymal stromal cell infusions as rescue therapy for corticosteroid-refractory adult autoimmune enteropathy." 04/01/2018 - "The final diagnosis of autoimmune enteropathy (AIE) was made based on histopathologic findings of endoscopic biopsy from duodenal mucosa after excluding neoplastic disease, inflammatory bowel disease, and infectious diarrhea, etc. Chronic diarrhea and oliguria alleviated after the administration of corticosteroids. " 05/01/2021 - "After many laboratory, endoscopy and radiological tests, she was diagnosed with autoimmune enteropathy (AE) and achieved clinical remission with corticosteroids and azathioprine."
4.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 02/01/2021 - "This is a case of adult-onset autoimmune enteropathy (AIE) in a 46-year-old man with multiple autoimmune conditions who presented with worsening disease process after receiving cyclophosphamide. " 02/01/2021 - "Acute Flare of Adult-Onset Autoimmune Enteropathy Associated With Cyclophosphamide." 05/01/2003 - "Remission of severe autoimmune enteropathy after treatment with high-dose cyclophosphamide."
5.	Abatacept (Orencia)FDA Link 01/01/2014 - "Abatacept: a new treatment option for refractory adult autoimmune enteropathy." 01/01/2022 - "Case Report: "Primary Immunodeficiency"-Severe Autoimmune Enteropathy in a Pediatric Heart Transplant Recipient Treated With Abatacept and Alemtuzumab." 01/01/2022 - "In this case report, we describe a clinical course of a child with severe autoimmune enteropathy after a heart transplant in infancy and detail a treatment approach with abatacept and alemtuzumab. "
6.	Azathioprine (Imuran)FDA LinkGeneric 05/01/2021 - "After many laboratory, endoscopy and radiological tests, she was diagnosed with autoimmune enteropathy (AE) and achieved clinical remission with corticosteroids and azathioprine."
7.	Immune Checkpoint InhibitorsIBA 03/01/2017 - "Clinical history, lack of prominent intra-epithelial lymphocytes and crypt rupture may help to distinguish PD-1 inhibitor gastroenterocolitis from mimics, which include other medication effect, inflammatory bowel disease, graft-versus-host disease, cytomegalovirus infection and autoimmune enteropathy."
8.	vedolizumabIBA 01/01/2018 - "This case is the first to show success in treating autoimmune enteropathy with vedolizumab and provides physicians with an additional therapeutic option when limited by a patient's comorbidities and side effects of other drugs." 01/01/2018 - "New Treatment Option for Autoimmune Enteropathy: A Rare Case of Intractable Diarrhea Treated with Vedolizumab."
9.	AntibodiesIBA 12/01/2009 - "Anti-goblet cell antibodies for the diagnosis of autoimmune enteropathy?" 07/01/2000 - "A small proportion of these patients seem to have an adult form of autoimmune enteropathy, characterized by the presence of antienterocyte antibodies. " 10/01/1999 - "Autoimmune enteropathy with goblet cell antibodies." 06/01/1999 - "Autoimmune enteropathy with goblet-cell antibodies." 10/01/1995 - "Autoimmune enteropathy with anti-goblet cell antibodies."
10.	T-Cell Antigen Receptors (T-Cell Receptor)IBA 07/01/1996 - "We have evaluated the expression of different molecular forms of T cell antigen receptor (TcR) in duodenal biopsies of pediatric patients with different forms of villous atrophy: celiac disease, autoimmune enteropathy, intractable diarrhea of unknown origin, and severe cow milk intolerance. " 07/01/2000 - "Based on this recent evidence, patients with refractory sprue should be screened for antienterocyte antibodies and have T-cell receptor and monoclonal antibody studies performed; this could facilitate identification of cases of adult-onset autoimmune enteropathy and those of cryptic T-cell lymphoma. "

Therapies and Procedures

1.	Aftercare (After-Treatment) 05/01/2003 - "Remission of severe autoimmune enteropathy after treatment with high-dose cyclophosphamide."
2.	Therapeutics 07/01/2023 - "A Combination Therapy in a Rare Case of Adult-Onset Autoimmune Enteropathy." 01/01/2019 - "Autoimmune Enteropathy: An Updated Review with Special Focus on Stem Cell Transplant Therapy." 01/01/2011 - "The discovery of the molecular background for autoimmune enteropathy provides important new potential opportunities for diagnosis and therapy. " 01/01/2021 - "Limited awareness among pediatricians of autoimmune enteropathy (AIE) caused by destruction of the gut endocrine cells in APECED patients delays diagnosis and appropriate therapy. " 11/29/2011 - "Adult onset autoimmune enteropathy (AIE) is a rare condition characterized by diarrhea refractory to dietary therapy diagnosed in patients with evidence of autoimmune conditions. "
3.	Gluten-Free Diet 01/01/2023 - "The clinical picture, lack of response to a gluten-free diet and endoscopic and histopathologic findings were compatible with autoimmune enteropathy. " 04/01/2015 - "In severe cases of childhood coeliac disease not responding to a gluten free diet, autoimmune enteropathy and refractory coeliac disease must be taken into account. " 01/01/2020 - "Celiac disease, an autoimmune enteropathy, occurs in susceptible individuals and is treatable with a gluten-free diet. " 06/01/2016 - "Celiac disease is the most common autoimmune enteropathy in Western countries, and is usually associated with a good response to the gluten free diet and an excellent prognosis. " 12/01/2006 - "Autoimmune enteropathy should be always ruled out in patients with a villous atrophy unresponsive to a gluten-free diet, autoimmune manifestations and negativity of coeliac disease markers."
4.	Diet Therapy (Therapy, Diet) 11/01/2020 - "Autoimmune enteropathy is an extremely rare condition characterized by an abnormal intestinal immune response which typically manifests within the first 6 months of life as severe, intractable diarrhea that does not respond to dietary modification. " 09/01/2012 - "Adult autoimmune enteropathy (AIE) is a rare cause of malabsorption syndrome unresponsive to dietary restriction. " 04/01/2018 - "We aim to describe the case of a 41-year-old woman complaining of severe malabsorption syndrome, who was diagnosed with autoimmune enteropathy based on the presence of flat intestinal mucosa unresponsive to any dietary restriction and positivity for enterocyte autoantibodies. " 03/01/2014 - "Autoimmune enteropathy (AIE) is a rare cause of small bowel villous atrophy, characterized by malabsorption, unresponsiveness to dietary restriction, circulating autoantibodies to enterocytes, and an overall predisposition to autoimmunity. " 07/12/1997 - "Autoimmune enteropathy is a condition described in children and characterised by villous atrophy, which is unresponsive to any dietary restrictions, and by the presence of enterocyte autoantibodies. "
5.	Parenteral Nutrition 02/27/2021 - "We performed a comprehensive search of published literature from January 2010 to the present by selecting the following search strings: "intestinal failure" OR "home parenteral nutrition" OR "short bowel syndrome" OR "chronic pseudo-obstruction" OR "chronic intestinal pseudo-obstruction" OR "autoimmune enteropathy" OR "long-term parenteral nutrition". " 05/01/2014 - "To evaluate the effects of glutamine-supplemented parenteral nutrition (PN) and probiotics in adult autoimmune enteropathy (AIE) patients. " 05/01/2014 - "Glutamine-supplemented parenteral nutrition and probiotics in four adult autoimmune enteropathy patients." 07/01/2009 - "The term autoimmune enteropathy (AIE) was applied to a form of "intractable diarrhoea" with serum gut autoantibodies, characterized by male predominance, early onset, poor response to parenteral nutrition and several autoimmune diseases, mainly type 1 diabetes. "