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Autoimmune syndrome

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https://www.readbyqxmd.com/read/29331748/safety-and-efficacy-of-anti-programmed-death-1-antibodies-in-patients-with-cancer-and-pre-existing-autoimmune-or-inflammatory-disease
#1
François-Xavier Danlos, Anne-Laure Voisin, Valérie Dyevre, Jean-Marie Michot, Emilie Routier, Laurent Taillade, Stéphane Champiat, Sandrine Aspeslagh, Julien Haroche, Laurence Albiges, Christophe Massard, Nicolas Girard, Stéphane Dalle, Benjamin Besse, Salim Laghouati, Jean-Charles Soria, Christine Mateus, Caroline Robert, Emilie Lanoy, Aurélien Marabelle, Olivier Lambotte
OBJECTIVE: Patients with autoimmune or inflammatory disease (AID) are susceptible to immune-related adverse events (irAEs) when treated with immune check-point inhibitors (ICIs). We decided to analyse the safety and effectiveness of anti-PD-1 antibodies in AID patients and look for an association between the presence of pre-existing AID and the clinical outcome. METHODS: In a prospective study of the REISAMIC registry of grade ≥2 irAEs occurring in ICI-treated patients, we studied the associations between pre-existing AID on one hand and irAE-free survival, overall survival and best objective response rate on the other...
January 10, 2018: European Journal of Cancer
https://www.readbyqxmd.com/read/29331587/il-23-il-17-immune-axis-in-guillain-barr%C3%A3-syndrome-exploring-newer-vistas-for-understanding-pathobiology-and-therapeutic-implications
#2
REVIEW
Monojit Debnath, Madhu Nagappa, Geetanjali Murari, Arun B Taly
Guillain Barré Syndrome (GBS) is a severe disorder of the peripheral nervous system with an inadequately known etiopathology. It is a post infectious immune mediated disorder, characterized by autoantibody production, complement activation as well as T reactivity against gangliosides. However, the precise etiopathogenesis remains poorly understood in a majority of the patients. Th17 cells, a recently identified lineage of Th cells have emerged as a predominant inducer of autoimmunity and inflammation in various immunological disorders...
January 10, 2018: Cytokine
https://www.readbyqxmd.com/read/29330115/pediatric-onset-evans-syndrome-heterogeneous-presentation-and-high-frequency-of-monogenic-disorders-including-lrba-and-ctla4-mutations
#3
Caroline Besnard, Eva Levy, Nathalie Aladjidi, Marie-Claude Stolzenberg, Aude Magerus-Chatinet, Olivier Alibeu, Patrick Nitschke, Stéphane Blanche, Olivier Hermine, Eric Jeziorski, Judith Landman-Parker, Guy Leverger, Nizar Mahlaoui, Gérard Michel, Isabelle Pellier, Felipe Suarez, Isabelle Thuret, Geneviève de Saint-Basile, Capucine Picard, Alain Fischer, Bénédicte Neven, Frédéric Rieux-Laucat, Pierre Quartier
Evans syndrome (ES) is defined by the combination of autoimmune hemolytic anemia and immune thrombocytopenia. Clinical presentation includes manifestations of immune dysregulation, found in primary immune deficiencies, autoimmune lymphoproliferative syndrome with FAS (ALPS-FAS), Cytotoxic T Lymphocyte Antigen-4 (CTLA-4) and Lipopolysaccharide-Responsive vesicle trafficking Beige-like and Anchor protein (LRBA) defects. We report the clinical history and genetic results of 18 children with ES after excluding ALPS-FAS...
January 9, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29328521/the-central-vein-sign-differentiates-ms-from-cns-inflammatory-vasculopathies
#4
Pietro Maggi, Martina Absinta, Matteo Grammatico, Luisa Vuolo, Giacomo Emmi, Giovanna Carlucci, Gregorio Spagni, Alessandro Barilaro, Anna Maria Repice, Lorenzo Emmi, Domenico Prisco, Vittorio Martinelli, Roberta Scotti, Niloufar Sadeghi, Gaetano Perrotta, Pascal Sati, Bernard Dachy, Daniel S Reich, Massimo Filippi, Luca Massacesi
OBJECTIVES: In multiple sclerosis (MS), MRI is a sensitive tool for detecting white matter lesions, but its diagnostic specificity is still suboptimal; ambiguous cases are frequent in clinical practice. Detection of perivenular lesions in the brain (the "central vein sign") improves the pathological specificity of MS diagnosis, but comprehensive evaluation of this MRI biomarker in MS-mimicking inflammatory and/or autoimmune diseases, such as CNS inflammatory vasculopathies, is missing...
January 12, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29327271/-paraneoplastic-neurological-syndromes-a-current-summary
#5
REVIEW
D De Simoni, R Höftberger
BACKGROUND: Paraneoplastic neurological syndromes (PNNS) are remote effects of a tumor and mediated by an altered immune reaction. In the last ten years, the spectrum of PNNS has changed profoundly with the discovery of a new category of neurological diseases that are associated with antibodies against surface or synaptic antigens. In contrast to classical PNNS, patients with surface receptor autoimmunity are often highly responsive to immunotherapy. OBJECTIVES: This article provides an update on the most relevant PNNS, focusing on specific syndromes associated with antibodies against classical onconeuronal antigens as well as surface and synaptic proteins...
January 11, 2018: Der Internist
https://www.readbyqxmd.com/read/29327071/diseases-of-complement-dysregulation-an-overview
#6
REVIEW
Edwin K S Wong, David Kavanagh
Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation. Although complement overactivation is common to all, cell surface alternative pathway dysregulation (aHUS), fluid phase alternative pathway dysregulation (C3G), or terminal pathway dysregulation (PNH) predominates resulting in the very different phenotypes seen in these diseases. The mechanism underlying the dysregulation also varies with predominant acquired autoimmune (C3G), somatic mutations (PNH), or inherited germline mutations (aHUS) predisposing to disease...
January 11, 2018: Seminars in Immunopathology
https://www.readbyqxmd.com/read/29326369/successful-treatment-of-postural-orthostatic-tachycardia-and-mast-cell-activation-syndromes-using-naltrexone-immunoglobulin-and-antibiotic-treatment
#7
Leonard B Weinstock, Jill B Brook, Trisha L Myers, Brent Goodman
A patient with severe postural orthostatic tachycardia syndrome (POTS) and mast cell activation syndrome (MCAS) received immunotherapy with low-dose naltrexone (LDN) and intravenous immunoglobulin (IVIg) and antibiotic therapy for small intestinal bacterial overgrowth (SIBO). A dramatic and sustained response was documented. The utility of IVIg in autoimmune neuromuscular diseases has been published, but clinical experience with POTS is relatively unknown and has not been reported in MCAS. As a short-acting mu-opioid antagonist, LDN paradoxically increases endorphins which then bind to regulatory T cells which regulate T-lymphocyte and B-lymphocyte production and this reduces cytokine and antibody production...
January 11, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29325681/is-hypothyroidism-in-idiopathic-pleuroparenchymal-fibroelastosis-a-novel-lung-thyroid-syndrome
#8
Nobuyasu Awano, Takehiro Izumo, Kensuke Fukuda, Mari Tone, Daisuke Yamada, Tamiko Takemura, Soichiro Ikushima, Toshio Kumasaka
BACKGROUND: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare type of interstitial pneumonia characterized by fibroelastosis. Patients with IPPFE as well as idiopathic interstitial pneumonia often have autoimmune diseases, which sometimes coincide with hypothyroidism (HypoT). However, there have been no reports on the association between IPPFE and HypoT. The purpose of this study was to evaluate the correlation between IPPFE and HypoT. We also examined the pathological features of the thyroid glands from autopsied cases...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29325172/pres-review-of-histological-features
#9
Nicholas Willard, Justin M Honce, B K Kleinschmidt-DeMasters
Posterior reversible encephalopathy syndrome was described in 1996 as a clinical-neuroimaging entity characterized by parieto-occipital watershed-region edema without overt infarction. It has been linked to hypertension, eclampsia, immunosuppressive therapies, infections, and autoimmune disorders. The condition usually has an acute onset, presents with seizures, and ameliorates within days. There have been few neuropathological studies, but in some cases, tissue damage may be more permanent.
February 1, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29325166/drug-safety-and-immunogenicity-of-tumour-necrosis-factor-inhibitors-the-story-so-far
#10
Meghna Jani, William G Dixon, Hector Chinoy
TNF-α inhibitor (TNFi) therapies have transformed the treatment of several rheumatic musculoskeletal diseases. However, the majority of TNFi's are immunogenic and consequent anti-drug antibodies formation can impact on both treatment efficacy and safety. Several controversies exist in the area of immunogenicity of TNFis and drug safety. While anti-drug antibodies to TNFis have been described in association with infusion reactions; serious adverse events (AEs) such as thromboembolic events, lupus-like syndrome, paradoxical AEs, for example, vasculitis-like events and other autoimmune manifestations have also been reported...
January 8, 2018: Rheumatology
https://www.readbyqxmd.com/read/29324900/internal-carotid-artery-stenosis-a-novel-surgical-model-for-moyamoya-syndrome
#11
Jill M Roberts, Michael E Maniskas, Justin F Fraser, Gregory J Bix
Moyamoya is a cerebrovascular disorder characterized by progressive stenosis of the intracranial internal carotid arteries. There are two forms: Disease and Syndrome, with each characterized by the sub-population it affects. Moyamoya syndrome (MMS) is more prominent in adults in their 20's-40's, and is often associated with autoimmune diseases. Currently, there are no surgical models for inducing moyamoya syndrome, so our aim was to develop a new animal model to study this relatively unknown cerebrovascular disease...
2018: PloS One
https://www.readbyqxmd.com/read/29322703/impact-of-electrophysiological-and-clinical-variants-and-timing-of-plasmapheresis-on-outcome-of-guillain-barr%C3%A3-syndrome
#12
Bipin Amin, Himanshu Meghnathi, M D Gajjar, Tarak Patel, Jignesh Vanani, Nidhi Gupta, Anil Chauhan
Introduction: Guillain-Barré syndrome (GBS) is an autoimmune polyneuropathy causing acute flaccid paralysis and it is known to improve with plasmapheresis. Objective: To study effects of electrophysiological type of GBS, clinical variant of GBS and time taken for initiation of plasmapheresis on outcome of disease. Methods: 50 consecutive patients of GBS attending tertiary care hospital underwent clinical examination and electrophysiological studies...
November 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29321641/antiphospholipid-syndrome
#13
REVIEW
Karen Schreiber, Savino Sciascia, Philip G de Groot, Katrien Devreese, Soren Jacobsen, Guillermo Ruiz-Irastroza, Jane E Salmon, Yehuda Shoenfeld, Ora Shovman, Beverley J Hunt
Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the presence of antiphospholipid antibodies, such as lupus anticoagulant, anticardiolipin antibodies and anti-β2-glycoprotein 1 antibodies. APS can present with a variety of clinical phenotypes, including thrombosis in the veins, arteries and microvasculature as well as obstetrical complications. The pathophysiological hallmark is thrombosis, but other factors such as complement activation might be important. Prevention of thrombotic manifestations associated with APS includes lifestyle changes and, in individuals at high risk, low-dose aspirin...
January 11, 2018: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/29317827/optimal-management-of-cogan-s-syndrome-a-multidisciplinary-approach
#14
REVIEW
Vittorio D'Aguanno, Massimo Ralli, Marco de Vincentiis, Antonio Greco
Cogan's syndrome (CS) is a rare disorder characterized by nonsyphilitic interstitial keratitis (IK) and audio-vestibular symptoms. CS affects mainly young Caucasian adults, mostly during their first three decades of age, and may develop into typical and atypical variants. Typical CS manifests primarily with IK and hearing loss, whereas atypical CS usually presents with inflammatory ocular manifestations in association with audio-vestibular symptoms but mostly different Ménière-like symptoms and, more frequently, with systemic inflammation (70%), of which vasculitis is the pathogenic mechanism...
2018: Journal of Multidisciplinary Healthcare
https://www.readbyqxmd.com/read/29317049/autoimmunity-against-a-glycolytic-enzyme-as-a-possible-cause-for-persistent-symptoms-in-lyme-disease
#15
Paolo Maccallini, Serena Bonin, Giusto Trevisan
Some patients with a history of Borrelia burgdorferi infection develop a chronic symptomatology characterized by cognitive deficits, fatigue, and pain, despite antibiotic treatment. The pathogenic mechanism that underlines this condition, referred to as post-treatment Lyme disease syndrome (PTLDS), is currently unknown. A debate exists about whether PTLDS is due to persistent infection or to post-infectious damages in the immune system and the nervous system. We present the case of a patient with evidence of exposure to Borrelia burgdorferi sl and a long history of debilitating fatigue, cognitive abnormalities and autonomic nervous system issues...
January 2018: Medical Hypotheses
https://www.readbyqxmd.com/read/29316339/reply-x-chromosome-short-arm-involvement-in-autoimmune-diseases-comment-on-the-report-by-sharma-et-al
#16
R Hal Scofield, Rohan Sharma, Valerie M Harris
Studying Klinefelter's syndrome (male 47,XXY) and triple X (female 4,7XXX), we propose that the number of X chromosomes mediates the female bias of systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS). Our recent paper described SLE and SS patients with extremely rare X chromosome aneuploides in which distal Xp was triplicated, implicating genes lying on the short arm of X1 . Brooks points out that X-linked chronic granulomatosus disease (X-CGD) can also have gene duplication from Xp21.2 to Xp terminus, and some female carriers of X-CDG have a cutaneous lupus-like illness...
January 5, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29316023/sj%C3%A3-gren-s-syndrome-x-chromosome-dose-effect-an-epigenetic-perspective
#17
REVIEW
Jean-Luc C Mougeot, Braxton Noll, Farah K Bahrani Mougeot
Sjögren's syndrome (SS) is a chronic autoimmune disease affecting exocrine glands leading to mouth and eyes dryness. The extent to which epigenetic DNA methylation changes are responsible for an X-chromosome dose effect has yet to be determined. Our objectives were to (i) describe how epigenetic DNA methylation changes could explain an X-chromosome dose effect in SS for women with normal 46,XX genotype and (ii) determine the relevant relationships to this dose effect, between X-linked genes, genes controlling X-chromosome inactivation (XCI) and genes encoding associated transcription factors, all of which are differentially expressed and/or differentially methylated in the salivary glands of SS patients...
January 9, 2018: Oral Diseases
https://www.readbyqxmd.com/read/29313588/paraneoplastic-inverse-myasthenic-syndrome-as-a-presentation-of-bronchogenic-carcinoma
#18
G S Chowdhary, Malav Jhala
Tumours may produce growth factors and cytokines responsible for signs and symptoms distant to the primary or metastatic site. This may be the first sign of a malignancy and its recognition may be critical for early cancer detection. Moreover, proper diagnosis spares the patient of extensive and expensive search for an alternate cause of the neurological dysfunction. In neurological paraneoplastic syndromes like Lambert Eaton Myasthenic syndrome associated with small cell lung cancer, evidence of autoimmunity against presynaptic neuro-muscular junction by anti voltage gated calcium channel anti bodies is well documented...
September 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29311560/a-central-role-for-pi3k-akt-signaling-pathway-in-linking-samhd1-deficiency-to-the-type-i-interferon-signature
#19
Changhoon Oh, Jeongmin Ryoo, Kiwon Park, Baek Kim, Michele B Daly, DongYeon Cho, Kwangseog Ahn
The autoimmune disorder Aicardi-Goutières syndrome (AGS) is characterized by a constitutive type I interferon response. SAMHD1 possesses both dNTPase and RNase activities and mutations in SAMHD1 cause AGS; however, how SAMHD1-deficiency causes the type I interferon response in patients with AGS remains unknown. Here, we show that endogenous RNA substrates accumulated in the absence of SAMHD1 act as a major immunogenic source for the type I interferon response. Reconstitution of SAMHD1-negative human cells with wild-type but not RNase-defective SAMHD1 abolishes spontaneous type I interferon induction...
January 8, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29311288/coronary-embolism-among-st-segment-elevation-myocardial-infarction-patients-mechanisms-and-management
#20
Batric Popovic, Nelly Agrinier, Nidhal Bouchahda, Samuel Pinelli, Charles Henry Maigrat, Pierre Adrien Metzdorf, Christine Selton Suty, Yves Juillière, Edoardo Camenzind
BACKGROUND: Coronary artery embolism (CE) is recognized as an important nonatherosclerotic cause of ST-segment-elevation myocardial infarction. The objective was to describe clinical characteristics and long-term outcomes and to identify risks factors of CE in a large consecutive series of ST-segment-elevation myocardial infarction patients. METHODS AND RESULTS: We studied 1232 consecutive patients who presented with de novo ST-segment-elevation myocardial infarction...
January 2018: Circulation. Cardiovascular Interventions
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