{"id":"http://purl.bioontology.org/ontology/MDRFRE/20000045","prefLabel":"Lupus érythémateux systémique (SMQ)","notation":"20000045","semanticType":["http://purl.bioontology.org/ontology/STY/T185"],"cui":["C1869047"],"definition":["* Affection autoimmune inflammatoire chronique affectant plusieurs systèmes du corps selon différents degrés de sévérité et caractérisé par divers anticorps o Anticorps anti-ADN double brin et anti-polypeptides Sm o Anticorps antiphospholipides (comprenant l'anticoagulant lupique, les anticorps anticardiolipine et ceux produisant des résultats faux positifs pour la syphilis) o L'activation complémentaire entraîne la formation de complexe immun * Les médicaments peuvent déclencher les symptômes de novo du lupus érythémateux systémique (LES) ou exacerber les symptômes existants * La sévérité et la manifestation des symptômes varient selon les présentations cliniques o Symptômes systémiques : anorexie, perte de poids, malaise, myalgie, arthralgies et fièvre o Manifestations cutanées (par exemple, érythème maculaire en ailes de papillon, lésions discoïdes, lésions maculopapulaires, érythème des côtés des paumes, lésions maculaires rouges ou pourpres sur la face palmaire des doigts, alopécie, érythème périunguéal, lésions de la muqueuse, purpura et photosensibilité o Polyarthrite o Néphrite - Protéinurie, glomérulonéphrite membranoproliférative diffuse, urines anormales (hématurie, pyurie, cylindres urinaires) ou augmentation de la créatinine sérique ; hypertension et syndrome néphrotique peuvent survenir o Manifestations du système nerveux central - Céphalées, épilepsie, psychoses, syndrome de cerveau organique et changements de la personnalité, accident vasculaire cérébral ou crises ischémiques transitoires o Autres manifestations/complications - péricardite, myocardite, endocardite fibrineuse (Libman-Sachs) et insuffisances de valves - Pleurésie avec ou sans effusion - Anémie, leucopénie et thrombocytopénie dues à l'hémolyse - Douleurs abdominales, nausées, vomissements et diarrhée - syndrome de Gougerot-Sjögren, conjonctivite non précisée, vascularite rétinienne et névrite optique"],"label":[],"synonym":[],"obsolete":false,"prefixIRI":null,"subClassOf":[],"xref":null,"inScheme":[],"created":null,"modified":null,"memberOf":[],"altLabelXl":[],"hiddenLabelXl":[],"parents":[],"prefLabelXl":[],"isInActiveScheme":[null],"isInActiveCollection":[],"properties":{"https://data.bioontology.org/metadata/def/prefLabel":["20000045"],"http://purl.bioontology.org/ontology/MDRFRE/SMQ_ALGO":["A or Sum(Category Term Weight)>6"],"http://www.w3.org/1999/02/22-rdf-syntax-ns#type":["http://www.w3.org/2002/07/owl#Class"],"http://bioportal.bioontology.org/ontologies/umls/tui":["T185"],"http://purl.bioontology.org/ontology/MDRFRE/SMQ_LEVEL":["1"],"http://purl.bioontology.org/ontology/MDRFRE/SOS":["Le système de \"pondération\" des termes larges a été développé empiriquement et tient compte de la fréquence des termes et de la probabilité de leur rapport avec l'apparition documentée du LES chez les patients recevant ces médicaments. L'algorithme s'applique de la manière suivante : Les PT mentionnés dans chaque cas sont groupés selon les catégories prédéterminées. S'il y a plusieurs PT de la même catégorie, elle n'est comptée qu'une seule fois. Chaque catégorie représentée dans le cas contribue au score total par son facteur de pondération empiriquement prédéterminé (par exemple, trois pour les catégories Affection hématologique, Sérite et Affection immunologique). Le score total du cas est donc la somme de toutes les catégories présentes dans le cas, affectées de leur pondération. Si cette somme est supérieure à six, le cas sera considérée comme suspect de LES. Par exemple, un cas comportant des PT dans les catégories Affection hématologique (3), Affection immunologique (3) et Affection rénale (1) aura un score de 7 (3 + 3 + 1) et sera classé comme cas potentiel de LES dans la recherche à portée large."],"http://www.w3.org/2004/02/skos/core#notation":["20000045"],"http://purl.bioontology.org/ontology/MDRFRE/SMQ_STATUS":["A"],"http://purl.bioontology.org/ontology/MDRFRE/SMQ_SOURCE":["1. Guidelines for referral and management of systemic lupus erythematosus in adults. American College of Rheumatology Ad Hoc Committee on Systemic Lupus Erythematosus Guidelines. Arthritis Rheum 1999;42:1785-96. 2. Hellman DB, Stone JH. Arthritis and musculoskeletal disorders. In: Tierney, Jr. LM, McPhee SJ, Papadakis MA, editors. Current medical diagnosis and treatment. 43rd ed. New York: McGraw-Hill Companies, Inc.; 2004. Available via intranet at STAT!Ref Online Medical Database. Teton Data Systems, Jackson, Wyo. (Version 3.3.6, 2004). 3. Hahn BH. Disorders of the immune system, connective tissues, and joints. In: Kasper DL, Fauci AS, Longo DL, Braunwald E, Hauser SL, Jameson JL, editors. Harrison's principles of internal medicine. 16th ed. New York: McGraw-Hill Companies; 2005. Available via intranet at STAT!Ref Online Medical Database. Teton Data Systems, Jackson, Wyo. (Version 3.3.6, 2004). 4. Diffuse connective tissue disease. In: Beers MH, Berkow R, editors. The merck manual of diagnosis and therapy. 11th ed. Whitehouse Station: Merck Research Laboratories; 1999. Available via intranet at STAT!Ref Online Medical Database. Teton Data Systems, Jackson, Wyo. (Version 3.3.6, 2004). 5. Trethewey P. Systemic Lupus Erythematosus. Dimens Crit Care Nurs 2004;23:111-5. 6. CIOMS Publication, \"Reporting Adverse Drug Reactions: Definitions of Terms and Criteria for their Use\", pp 19 - 20, 1999. 7. The Merck Manual, Seventeenth Edition, pp 426 - 430, 1999 8. \"Harrison's Principles of Internal Medicine\", 14th edition, pp 1874 - 1880, 1998 9. Stratton MA Drug-induced systemic lupus erethemetosis. Clin Pharm 1985: 4:567-63 10. Yung R, Chang S, Hemati N et al. Mechanisms of drug-induced lupus. IV. Comparison of procainamide and hydralazine with analogs in vitro and in vivo. Arthitis Rheum 1997; 40: 1436-43. 11. Cush JJ, Goldings EA. Drug-induced lupus: clinical spectrum and pathogenesis. Am J Med Sci 1985; 290: 36-45."],"http://bioportal.bioontology.org/ontologies/umls/hasSTY":["http://purl.bioontology.org/ontology/STY/T185"],"http://bioportal.bioontology.org/ontologies/umls/cui":["C1869047"],"http://www.w3.org/2004/02/skos/core#prefLabel":["Lupus érythémateux systémique (SMQ)"],"http://www.w3.org/2004/02/skos/core#definition":["* Affection autoimmune inflammatoire chronique affectant plusieurs systèmes du corps selon différents degrés de sévérité et caractérisé par divers anticorps o Anticorps anti-ADN double brin et anti-polypeptides Sm o Anticorps antiphospholipides (comprenant l'anticoagulant lupique, les anticorps anticardiolipine et ceux produisant des résultats faux positifs pour la syphilis) o L'activation complémentaire entraîne la formation de complexe immun * Les médicaments peuvent déclencher les symptômes de novo du lupus érythémateux systémique (LES) ou exacerber les symptômes existants * La sévérité et la manifestation des symptômes varient selon les présentations cliniques o Symptômes systémiques : anorexie, perte de poids, malaise, myalgie, arthralgies et fièvre o Manifestations cutanées (par exemple, érythème maculaire en ailes de papillon, lésions discoïdes, lésions maculopapulaires, érythème des côtés des paumes, lésions maculaires rouges ou pourpres sur la face palmaire des doigts, alopécie, érythème périunguéal, lésions de la muqueuse, purpura et photosensibilité o Polyarthrite o Néphrite - Protéinurie, glomérulonéphrite membranoproliférative diffuse, urines anormales (hématurie, pyurie, cylindres urinaires) ou augmentation de la créatinine sérique ; hypertension et syndrome néphrotique peuvent survenir o Manifestations du système nerveux central - Céphalées, épilepsie, psychoses, syndrome de cerveau organique et changements de la personnalité, accident vasculaire cérébral ou crises ischémiques transitoires o Autres manifestations/complications - péricardite, myocardite, endocardite fibrineuse (Libman-Sachs) et insuffisances de valves - Pleurésie avec ou sans effusion - Anémie, leucopénie et thrombocytopénie dues à l'hémolyse - Douleurs abdominales, nausées, vomissements et diarrhée - syndrome de Gougerot-Sjögren, conjonctivite non précisée, vascularite rétinienne et névrite 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