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LISTA COMPLETA DE DOENÇAS LISTA DE DOENÇAS AUTOIMUNES POR ESPECIALIDADES MÉDICAS REUMATO Doenças difusas do tecido conjuntivo, incluindo vasculites sistêmicas Síndrome anti-fosfolípide Síndrome HELLP (SAF, trombocitopenia, hemólise, elevação de enzimas hepáticas) Doença de Behçet Artrite reumatóide Artrite reumatóide juvenil Polimialgia reumática Poliarterite nodosa Arterite de células gigantes (arterite temporal) Arterite de Takayasu Granulomatose com poliangeite (Wegener) Granulomatose com poliangeite e eosinofilia (Churg-Strauss) Síndrome de Sjögren Polidermatomiosite Miosite por corpúsculos de inclusão Miosite associada a vastatinas Esclerodermia Espondiloartrites Artrite psoriásica Reumatismo palindrômico Fasciite eosinofílica Imunodeficiência autoimune Crioglobulinemia essencial mista Rayanud Artrites reativas Policondrite recidivante Chronic recurrent multifocal ostomyelitis (CRMO) Autoimmune angioedema Henoch-Schonlein purpura Leukocytoclastic vasculitis Cogans syndrome Kawasaki syndrome Lupus eritematoso sistêmico HEMATO Anemias autoimunes Anemia aplástica autoimune Anemia hemolítica Anemia perniciosa Púrpura trombocitopênica autoimune Síndrome de Evans (anemia hemolítica Coombs+, púrpura trombocitopênica autoimune, com neutropenia autoimune em 15% dos casos) Ooforite utoimune Mielofibrose autoimune GINECO Mastopatia granulomatosa autoimune Endometriose ENDOCRINO Diabetes mellitus tipo I Addison Hashimoto Graves Type I, II, & III autoimmune polyglandular syndromes GASTRO & HEPATOLOGIA Hepatite autoimune tipo 1 Hepatite autoimune tipo 2 Colangite biliar primária (ex-cirrose) Colangite autoimune Colangite esclerosante primária Doença celíaca Esofagite eosinofílica Pancreatite autoimune Doença de Crohn Retocolite ulcerativa NEFRO Glomerulonefrite Goodpasture Nefropatia por IgA Interstitial cystitis Microscopic polyangiitis Amyloidosis Anti-GBM/Anti-TBM nephritis Paroxysmal nocturnal hemoglobinuria (PNH) NEUROLOGIA Guillain-Barré Neuropatias desmielinizantes Dr. Carlos A von Muhlen: tember 2013, Vol. 34, No. 9 p. 453. Glycolipid antigens and autoantibodies in autoimmune neuropathies Hugh J. Willison and Carl S. Goodyear Institute of Infection, Immunity and Inflammation, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow G12 8TA, UK Autoantibodies to glycans present on glycolipids mediate the postinfectious paralytic disease, Guillain–Barre´ syndrome (GBS). These glycans are also found on lipooligosaccharides (LOSs) ofGBS-inducingmicrobes, suggesting molecular mimicry as a mechanism for disease induction.HowBlymphocytetolerancetoself-glycans is regulated during the initiation phase of the disease is currently under investigation. The discovery of antiglycolipid antibodies that bind to heteromeric glycolipid complexes has generated new insights in this field. Heteromeric complexes are structurally distinct glycolipids thatinteractto form newmolecular shapes capable of either enhancing or attenuating recognition by autoantibodies. Although the principles emerging from this phenomenon have a substantial impact on diagnostics methods,they also raise intriguing questions aboutthe diversity of innate antibody repertoires, mechanisms of tolerance, and autoantibody targeting of neural membranes. Miastenia gravis Esclerose múltipla Encefalomielite experimental alérgica Doença de Devic (neuromielite óptica) Encefalomielite perivenosa Narcolepsy Acute Disseminated Encephalomyelitis (ADEM) Acute necrotizing hemorrhagic leukoencephalitis PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus) Paraneoplastic cerebellar degeneration Transverse myelitis Chronic inflammatory demyelinating polyneuropathy (CIDP) Lambert-Eaton syndrome Encefalite Límbica Autoimune Paraneoplásica Hu CV2/CRMP5 Ma2 anfifisina Autoimune VGKC (voltage-gated potassium channel) receptores de glutamato tipo NMDA (células HEK-293 transfectadas com receptor NMDA) receptores de glutamato tipo AMPA receptores de glutamato tipo GABA ADEM Dr. Carlos A. von Muhlen: Dr. Carlos A. von Muhlen: Intern Med. 2016;55(21):3181-3184. Epub 2016 Nov 1. Two Cases of Acute Disseminated Encephalomyelitis Following Vaccination Against Human Papilloma Virus. Sekiguchi K1, Yasui N, Kowa H, Kanda F, Toda T. Author information Abstract We herein present two cases of acute disseminated encephalomyelitis (ADEM) following vaccination against human papilloma virus (HPV). Case 1 experienced diplopia and developed an unstable gait 14 days after a second vaccination of Cervarix. Brain magnetic resonance imaging (MRI) showed an isolated small, demyelinating lesion in the pontine tegmentum. Case 2 experienced a fever and limb dysesthesia 16 days after a second vaccination of Gardasil. Brain MRI revealed hyperintense lesion in the pons with slight edema on a T2-weighted image. Both cases resolved completely. It is important to accumulate further data on confirmed cases of ADEM temporally associated with HPV vaccination. Astrocitopatia autoimmune Dr. Carlos A. von Muhlen: Dr. Carlos A. von Muhlen: Ann Neurol. 2017 Jan 24. doi: 10.1002/ana.24881. [Epub ahead of print] GFAP-IgG as Biomarker of Autoimmune Astrocytopathy: Analysis of 102 Patients. Flanagan EP1,2, Hinson SR2, Lennon VA1,2,3, Fang B2, Aksamit AJ1, Morris PP4, Basal E2, Honorat JA2, Alfugham NB1, Linnoila JJ1, Weinshenker BG1,2, Pittock S1,2, McKeon A1,2. Author information Abstract Objective A novel autoimmune CNS disorder with glial fibrillary acidic protein (GFAP)-IgG as biomarker was recently characterized. Here, 102 patients with GFAP-IgG positivity are described. Methods The 102 included patients had: 1) serum, CSF or both that yielded a characteristic astrocytic pattern of mouse tissue immunostaining; 2) confirmation of IgG reactive with specific GFAP isoforms (α, ε, or κ) by cell-based assays, and 3) clinical data available. Control specimens (865) were evaluated by tissue (542) and cell-based (323) assays. Results Median symptom-onset age was 44 years (range, 8-103) and 54% were women. The predominant phenotype (83 patients; 81%) was inflammation of meninges, brain or spinal cord, or all 3 (meningoencephalomyelitis). Among patients, highest specificity for those phenotypes was observed for CSF testing (94%), and highest sensitivity was for the GFAPα isoform (100%). Rare GFAP-IgG positivity was encountered in serum controls by tissue-based assay (0.5%) or cell-based assay (1.5%), and in CSF controls by cell-based assay (0.9%). Among patients, striking perivascular radial enhancement was found on brain MRI in 53%. Though cases frequently mimicked vasculitis, angiography was uniformly negative, and spinal imaging frequently demonstrated longitudinally-extensive myelitic lesions. Diverse neoplasms encountered were found prospectively in 22%. Ovarian teratoma was most common and was predicted best when both NMDA-R-IgG and AQP4-IgG coexisted (71%). Six patients with prolonged follow-up had brisk corticosteroid response, but required additional immune-suppression to overcome steroid-dependency. Interpretation GFAPα-IgG when detected in CSF, is highly specific for an immunotherapy-responsive autoimmune CNS disorder, sometimes with paraneoplastic cause. OTORRINO Surdez neurosensorial autoimune Castleman disease Meniere’s disease DERMATO Alopecia areata Dermatite herpetiforme Eritema nodoso Lichen planus Lichen sclerosus Linear IgA disease (LAD) Lupus discóide Pênfigo Penfigóide bolhoso Psoríase Pyoderma gangrenosum Urticária autoimune Vesiculobullous dermatosis Vitiligo PNEUMO Sarcoidose Alveolite fibrosante Proteinose alveolar autoimune CARDIO Pericardite autoimune Disautonomia autoimune Miocardite de células gigantes Miocardite autoimune Cardiomiopatia Hiperlipidemia autoimuneSíndrome pós-pericardiotomia Postmyocardial infarction syndrome Autoimmune dysautonomia Congenital heart block OFTALMO Uveites Esclerites e Epiesclerites Vasculites Doenças autoimunes da órbita Sarcoidose Vogt-Koyanagi-Harada Retinopatia autoimune Neurite óptica Ocular cicatricial pemphigoid Sympathetic ophthalmia Ligneous conjunctivitis Pars planitis (peripheral uveitis) AZOOR - acute zonal occult outer retinopathy MISCELÂNEA IgG4-related diseases Dr. Carlos A von Muhlen: Dr. Carlos A von Muhlen: Immunoglobulin G4-related disease (IgG4-RD) is characterized by fibrous swelling of affected organs, elevations in serum IgG4 concentrations, and responsiveness to glucocorticoid treatment1. Affected tissues display similar histological features: diffuse lymphoplasmacytic infiltration by numerous IgG4-positive plasma cells, occasional eosinophils, storiform fibrosis, and obliterative phlebitis2. Dr. Carlos A. von Muhlen: Dr. Carlos A. von Muhlen: Intern Med. 2016;55(21):3181-3184. Epub 2016 Nov 1. Two Cases of Acute Disseminated Encephalomyelitis Following Vaccination Against Human Papilloma Virus. Sekiguchi K1, Yasui N, Kowa H, Kanda F, Toda T. Author information Abstract We herein present two cases of acute disseminated encephalomyelitis (ADEM) following vaccination against human papilloma virus (HPV). Case 1 experienced diplopia and developed an unstable gait 14 days after a second vaccination of Cervarix. Brain magnetic resonance imaging (MRI) showed an isolated small, demyelinating lesion in the pontine tegmentum. Case 2 experienced a fever and limb dysesthesia 16 days after a second vaccination of Gardasil. Brain MRI revealed hyperintense lesion in the pons with slight edema on a T2-weighted image. Both cases resolved completely. It is important to accumulate further data on confirmed cases of ADEM temporally associated with HPV vaccination. Dr. Carlos A. von Muhlen: Dr. Carlos A. von Muhlen: Ann Neurol. 2017 Jan 24. doi: 10.1002/ana.24881. [Epub ahead of print] GFAP-IgG as Biomarker of Autoimmune Astrocytopathy: Analysis of 102 Patients. Flanagan EP1,2, Hinson SR2, Lennon VA1,2,3, Fang B2, Aksamit AJ1, Morris PP4, Basal E2, Honorat JA2, Alfugham NB1, Linnoila JJ1, Weinshenker BG1,2, Pittock S1,2, McKeon A1,2. Author information Abstract Objective A novel autoimmune CNS disorder with glial fibrillary acidic protein (GFAP)-IgG as biomarker was recently characterized. Here, 102 patients with GFAP-IgG positivity are described. Methods The 102 included patients had: 1) serum, CSF or both that yielded a characteristic astrocytic pattern of mouse tissue immunostaining; 2) confirmation of IgG reactive with specific GFAP isoforms (α, ε, or κ) by cell-based assays, and 3) clinical data available. Control specimens (865) were evaluated by tissue (542) and cell-based (323) assays. Results Median symptom-onset age was 44 years (range, 8-103) and 54% were women. The predominant phenotype (83 patients; 81%) was inflammation of meninges, brain or spinal cord, or all 3 (meningoencephalomyelitis). Among patients, highest specificity for those phenotypes was observed for CSF testing (94%), and highest sensitivity was for the GFAPα isoform (100%). Rare GFAP-IgG positivity was encountered in serum controls by tissue-based assay (0.5%) or cell-based assay (1.5%), and in CSF controls by cell-based assay (0.9%). Among patients, striking perivascular radial enhancement was found on brain MRI in 53%. Though cases frequently mimicked vasculitis, angiography was uniformly negative, and spinal imaging frequently demonstrated longitudinally-extensive myelitic lesions. Diverse neoplasms encountered were found prospectively in 22%. Ovarian teratoma was most common and was predicted best when both NMDA-R-IgG and AQP4-IgG coexisted (71%). Six patients with prolonged follow-up had brisk corticosteroid response, but required additional immune-suppression to overcome steroid-dependency. Interpretation GFAPα-IgG when detected in CSF, is highly specific for an immunotherapy-responsive autoimmune CNS disorder, sometimes with paraneoplastic cause. Pancreatite autoimune Paquimeningite hipertrófica Dressler’s syndrome Evans syndrome POEMS syndrome Mooren’s ulcer Mucha-Habermann disease Restless legs syndrome Retroperitoneal fibrosis Schmidt syndrome Balo disease Susac’s syndrome Tolosa-Hunt syndrome Parry Romberg syndrome Parsonnage-Turner syndrome Sperm & testicular autoimmunity Stiff person syndrome Cold agglutinin disease Vasculites Autoanticorpos associados a câncer REUMATO fator reumatóide IgG / IgA / IgM anti-CCP FAP anti-queratina anti-Sa MCV (vimentina mutada citrulinada) Sheet3