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Autoimmune enteropathy

Stefan Tukaj, Anna Görög, Konrad Kleszczyński, Detlef Zillikens, Sarolta Kárpáti, Michael Kasperkiewicz
Inflammation-induced heat shock proteins (HSPs) and hypovitaminosis D have been reported to impact immune responses and to be associated with autoimmune diseases including celiac disease (CD), a gluten-sensitive enteropathy mediated by autoantibodies against tissue transglutaminase (TG2). Recently, we provided evidence for a role of autoantibodies to HSPs in patients with dermatitis herpetiformis (DH), an inflammatory skin disease induced by underlying latent CD. In this study, we aimed at investigating the humoral autoimmune response to HSPs and vitamin D status in CD patients (n=15) presenting without the cutaneous disease manifestation...
October 16, 2016: Journal of Steroid Biochemistry and Molecular Biology
Giuseppe Losurdo, Mariabeatrice Principi, Andrea Iannone, Enzo Ierardi, Alfredo Di Leo
Celiac disease (CD) is the most common autoimmune enteropathy, triggered by a deregulated immune response to gliadin. It has been hypothesized that human intestinal microbiota may interfere with the pathogenesis of the disease and in the clinical course of CD. In the present review, we analyzed the microbiota alterations observed in the course of CD, how they may influence the pathogenesis of CD, and the possible applications for a microbiota modulation in CD. In detail, most of the current literature underlined that the dysbiosis in CD is hallmarked by an increase in gram-negative and Bacteroidetes species, and by a decrease in Bifidobacteria and Lactobacilli...
November 2016: Journal of Clinical Gastroenterology
Shahrzad Bakhtiar, Frank Ruemmele, Fabienne Charbit-Henrion, Eva Lévy, Frédéric Rieux-Laucat, Nadine Cerf-Bensussan, Peter Bader, Ulrich Paetow
Monogenic primary immunodeficiency syndromes can affect one or more endocrine organs by autoimmunity during childhood. Clinical manifestations include type 1 diabetes mellitus, hypothyroidism, adrenal insufficiency, and vitiligo. Lipopolysaccharide (LPS)-responsive beige-like anchor protein (LRBA) deficiency was described in 2012 as a novel primary immunodeficiency, predominantly causing immune dysregulation and early onset enteropathy. We describe the heterogeneous clinical course of LRBA deficiency in two siblings, mimicking an autoimmune polyendocrine disorder in one of them in presence of the same underlying genetic mutation...
2016: Frontiers in Pediatrics
Gholamreza Azizi, Hassan Abolhassani, Mohammad Hosein Asgardoon, Tina Alinia, Reza Yazdani, Javad Mohammadi, Nima Rezaei, Hans D Ochs, Asghar Aghamohammadi
INTRODUCTION: Common variable immunodeficiency (CVID) comprises a large heterogeneous group of patients with primary antibody deficiency. AREAS COVERED: The affected patients are characterized by increased susceptibility to infections and low levels of serum immunoglobulin. However, enteropathy, granulomatous organ infiltrates, malignancy, inflammatory and autoimmune conditions are also prevalent. The concomitance of immunodeficiency and autoimmunity appears to be paradoxical and creates difficulties in the management of autoimmune complications affecting these patients...
September 16, 2016: Expert Review of Clinical Immunology
Antonella Gallo, Giovanna Passaro, Antonio Gasbarrini, Raffaele Landolfi, Massimo Montalto
Alterations of intestinal microflora may significantly contribute to the pathogenesis of different inflammatory and autoimmune disorders. There is emerging interest on the role of selective modulation of microflora in inducing benefits in inflammatory intestinal disorders, by as probiotics, prebiotics, synbiotics, antibiotics, and fecal microbiota transplantation (FMT). To summarize recent evidences on microflora modulation in main intestinal inflammatory disorders, PubMed was searched using terms microbiota, intestinal flora, probiotics, prebiotics, fecal transplantation...
August 28, 2016: World Journal of Gastroenterology: WJG
Ru-Ying Fan, Xiao-Wei Wang, Li-Jun Xue, Ran An, Jian-Qiu Sheng
Cronkhite-Canada syndrome (CCS) is a rare but serious protein-losing enteropathy, but little is known about the mechanism. Further more, misdiagnosis is common due to non-familiarity of its clinical manifestation. A 40-year-old male patient was admitted to our hospital because of diarrhea and hypogeusia associated with weight loss for 4 mo. On physical examination, skin pigmentation, dystrophic nail changes and alopecia were noted. He had no alike family history. Laboratory results revealed low levels of serum albumin (30...
August 16, 2016: World Journal of Clinical Cases
Julio Maria Fonseca Chebli, Liliana Andrade Chebli, Pedro Duarte Gaburri, Áureo Augusto de Almeida Delgado, Thais Mansur Ghetti Costa
No abstract text is available yet for this article.
September 2016: Pancreas
Maria Giovanna Danieli, Federica Pulvirenti, Valeria Rocchi, Ramona Morariu, Isabella Quinti
Hyaluronidase-facilitated subcutaneous immunoglobulin (fSCIg) is a new immunoglobulin product for replacement therapy in patients with primary antibody deficiencies (PAD). The pre-administration of recombinant human hyaluronidase associated with 10% immunoglobulin allowed the infusion of larger (up to 600 ml) amounts of immunoglobulin at a single infusion site, enabling patients to receive the necessary treatment in a single monthly dose. Here, we report the effectiveness and the tolerability of fSCIg in patients with severe PAD-related comorbidities: refractory autoimmune thrombocytopenia; systemic granulomatous disease; severe enteropathy, and Type I diabetes...
September 2016: Immunotherapy
Melanie Uhde, Mary Ajamian, Giacomo Caio, Roberto De Giorgio, Alyssa Indart, Peter H Green, Elizabeth C Verna, Umberto Volta, Armin Alaedini
OBJECTIVE: Wheat gluten and related proteins can trigger an autoimmune enteropathy, known as coeliac disease, in people with genetic susceptibility. However, some individuals experience a range of symptoms in response to wheat ingestion, without the characteristic serological or histological evidence of coeliac disease. The aetiology and mechanism of these symptoms are unknown, and no biomarkers have been identified. We aimed to determine if sensitivity to wheat in the absence of coeliac disease is associated with systemic immune activation that may be linked to an enteropathy...
July 25, 2016: Gut
Moira Paroni, Andrea Magarotto, Silvia Tartari, Giulia Nizzoli, Paola Larghi, Giulia Ercoli, Umberto Gianelli, Massimiliano Pagani, Luca Elli, Sergio Abrignani, Dario Conte, Jens Geginat, Flavio Caprioli
OBJECTIVES: To provide a functional and phenotypic characterization of immune cells infiltrating small intestinal mucosa during non-IPEX autoimmune enteropathy (AIE), as to gain insights on the pathogenesis of this clinical condition. METHODS: Duodenal biopsies from a patient with AIE at baseline and following drug-induced remission were analyzed by immunohistochemistry, immunofluorescence, and flow cytometry, and results were compared with those obtained from patients with active celiac disease, ileal Crohn's disease and healthy controls...
2016: Clinical and Translational Gastroenterology
Alberto Caminero, Heather J Galipeau, Justin L McCarville, Chad W Johnston, Steve P Bernier, Amy K Russell, Jennifer Jury, Alexandra R Herran, Javier Casqueiro, Jason A Tye-Din, Michael G Surette, Nathan A Magarvey, Detlef Schuppan, Elena F Verdu
BACKGROUND & AIMS: Partially degraded gluten peptides from cereals trigger celiac disease (CD), an autoimmune enteropathy occurring in genetically susceptible persons. Susceptibility genes are necessary but not sufficient to induce CD, and additional environmental factors related to unfavorable alterations in the microbiota have been proposed. We investigated gluten metabolism by opportunistic pathogens and commensal duodenal bacteria and characterized the capacity of the produced peptides to activate gluten-specific T-cells from CD patients...
October 2016: Gastroenterology
Karol Sestak, Hazel Thwin, Jason Dufour, David X Liu, Xavier Alvarez, David Laine, Adam Clarke, Anthony Doyle, Pyone P Aye, James Blanchard, Charles P Moehs
Celiac disease (CD) is an autoimmune disorder that affects approximately three million people in the United States. Furthermore, non-celiac gluten sensitivity (NCGS) affects an estimated additional 6% of the population, e.g., 20 million in the U.S. The only effective treatment of CD and NCGS requires complete removal of gluten sources from the diet. While required adherence to a gluten-free diet (GFD) is extremely difficult to accomplish, efforts to develop additional supportive treatments are needed. To facilitate these efforts, we developed a gluten-sensitive (GS) rhesus macaque model to study the effects of novel therapies...
2016: Nutrients
Umberto Volta, Giacomo Caio, Elisa Boschetti, Fiorella Giancola, Kerry J Rhoden, Eugenio Ruggeri, Paola Paterini, Roberto De Giorgio
BACKGROUND: Although serological tests are useful for identifying celiac disease, it is well established that a minority of celiacs are seronegative. AIM: To define the prevalence and features of seronegative compared to seropositive celiac disease, and to establish whether celiac disease is a common cause of seronegative villous atrophy. METHODS: Starting from 810 celiac disease diagnoses, seronegative patients were retrospectively characterized for clinical, histological and laboratory findings...
September 2016: Digestive and Liver Disease
Simon Murch
Recent diagnostic advances have demonstrated that celiac disease is relatively common although most patients have less florid symptoms than previously recognised. The mucosal lesion of this autoimmune disorder depends on both adaptive and innate immune responses. The characteristic antibodies to tissue transglutaminase-2 (tTG-2) and deamidated gliadin peptides may be produced in persons possessing the relevant HLA-DQ genotypes if intact gliadin peptides can penetrate the epithelial barrier to reach antigen presenting cells...
June 8, 2016: Indian Journal of Pediatrics
Behdad Navabi, Julia Elizabeth Mainwaring Upton
BACKGROUND: Eosinophilia is not an uncommon clinical finding. However, diagnosis of its cause can be a dilemma once common culprits, namely infection, allergy and reactive causes are excluded. Primary immunodeficiency disorders (PID) are among known differentials of eosinophilia. However, the list of PIDs typically reported with eosinophilia is small and the literature lacks an inclusive list of PIDs which have been reported with eosinophilia. This motivated us to review the literature for all PIDs which have been described to have elevated eosinophils as this may contribute to an earlier diagnosis of PID and further the understanding of eosinophilia...
2016: Allergy, Asthma, and Clinical Immunology
Bianca Tesi, Peter Priftakis, Fredrik Lindgren, Samuel C C Chiang, Nikolaos Kartalis, Alexandra Löfstedt, Esther Lörinc, Jan-Inge Henter, Jacek Winiarski, Yenan T Bryceson, Marie Meeths
PURPOSE: Autosomal recessive mutations in LRBA, encoding for LPS-responsive beige-like anchor protein, were described in patients with a common variable immunodeficiency (CVID)-like disease characterized by hypogammaglobulinemia, autoimmune cytopenias, and enteropathy. Here, we detail the clinical, immunological, and genetic features of a patient with severe autoimmune manifestations. METHODS: Whole exome sequencing was performed to establish a molecular diagnosis...
July 2016: Journal of Clinical Immunology
Katharina Anne Scherf, Rachele Ciccocioppo, Miroslav Pohanka, Kvetoslava Rimarova, Radka Opatrilova, Luis Rodrigo, Peter Kruzliak
Celiac disease (CD) is an autoimmune enteropathy initiated and sustained by the ingestion of gluten in genetically susceptible individuals. It is caused by a dysregulated immune response toward both dietary antigens, the gluten proteins of wheat, rye, and barley, and autoantigens, the enzyme tissue transglutaminase (TG2). The small intestine is the target organ. Although routine immunochemical protocols for a laboratory diagnosis of CD are available, faster, easier-to-use, and cheaper analytical devices for CD diagnosis are currently unavailable...
June 2016: Molecular Biotechnology
Umberto Volta, Maria Gloria Mumolo, Giacomo Caio, Elisa Boschetti, Rocco Latorre, Fiorella Giancola, Paola Paterini, Roberto De Giorgio
A 62-year-old woman complaining of severe malabsorption was diagnosed with celiac disease based on the findings of flat, small intestinal mucosa and HLA-DQ2 positivity, although celiac serology was negative. This diagnosis was questioned due to the lack of clinical and histological improvement after a long period of strict gluten-free diet. The detection of enterocyte autoantibodies guided to the correct diagnosis of autoimmune enteropathy, leading to a complete recovery of the patient following an appropriate immunosuppressive treatment...
2016: Gastroenterology and Hepatology From Bed to Bench
Eva Lévy, Marie-Claude Stolzenberg, Julie Bruneau, Sylvain Breton, Bénédicte Neven, Sylvie Sauvion, Mohammed Zarhrate, Patrick Nitschké, Alain Fischer, Aude Magérus-Chatinet, Pierre Quartier, Frédéric Rieux-Laucat
LRBA (lipopolysaccharide-responsive and beige-like anchor protein) deficiency associates immune deficiency, lymphoproliferation, and various organ-specific autoimmunity. To date, prevalent symptoms are autoimmune cytopenias and enteropathy, and lymphocytic interstitial lung disease. In 2 siblings from a consanguineous family presenting with early onset polyautoimmunity, we presumed autosomal recessive inheritance and performed whole exome sequencing. We herein report the first case of early-onset, severe, chronic polyarthritis associated with LRBA deficiency...
July 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Federica Branchi, Martina Locatelli, Carolina Tomba, Dario Conte, Francesca Ferretti, Luca Elli
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. However, a minority of patients develop complications of the disease, such as refractory celiac disease, ulcerative jejunoileitis and neoplastic complications such as adenocarcinoma of the small bowel and enteropathy associated T cell lymphoma. Neoplastic complications described in association with celiac disease have a high mortality rate, due to their aggressive behavior and to the usual advanced stage at the time of diagnosis...
June 2016: Digestive and Liver Disease
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