, Les patients avaient un âge médian de 30 ans (valeurs extrêmes : 8 -68 ans), p.87
, Leur suivi médian était de 100,5 mois (valeurs extrêmes : 1 -504 mois)
, Les patients présentaient un titre d'AAN médian de 1/1280 (valeurs extrêmes : 1/160 -1/1280)
, 8%) : il s'agissait d'Ac anti-ADN natif pour 66 patients (70,2%), des Ac anti-SSa et/ou SSb pour 37 patients (39,4%), des Ac anti-Sm pour 12 patients (12,8%), des Ac anti, On retrouvait d'autres auto-Ac chez 75 patients, vol.79
, 20 patients avaient un LA (22,0%), 15 patients avaient un Ac anti-bêta2GP1 (16,5%) et 23 avaient un aCL, vol.38
, Quarante-six patients (48,9%) présentaient une consommation du complément (C3 et/ou C4)
, 6%) avaient une anomalie à l'électrophorèse des protéines sériques dont 49 (56,3%) avaient une hypergammaglobulinémie polyclonale, Cinquante et un patients (sur les 87 testés, vol.58
, 2%) : il s'agissait d'arthralgies, concernant au moins 2 articulations, de rythme inflammatoire (dérouillage matinal ? 30 minutes) pour 67 d'entre eux (71,3%), d'arthrites (synovite impliquant au moins 2 articulations avec douleurs de rythme inflammatoire) dans 15 cas, La principale manifestation clinique du LES était rhumatologique, chez 82 patients, vol.87
, On retrouvait des lésions de lupus cutané aigu chez 43 patients (45,7%), de lupus cutané subaigu chez 6 patients (6,4%) et de lupus cutané chronique chez 10 patients (10,6%), La principale manifestation extra-rhumatologique était cutanéo-muqueuse et concernait 71 patients (75,5%)
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, Résumé Les anticorps (Ac) anti-NuMA représentent des auto-Ac identifiés sur une fluorescence particulière sur les cellules en mitose lors de la recherche d'Ac antinucléaires
, Soixante-six (73,3%) d'entre eux avaient une fluorescence anti-NuMA1 et 24 patients (26,7%) avaient une fluorescence anti-NuMA2. Parmi ces 90 patients, 70 (77,7%) souffraient d'une maladie systémique et 61 (67,8%) avaient une maladie auto-immune : syndrome de Sjögren primitif, Sur une cohorte rétrospective de 90 patients porteurs d'Ac anti-NuMA, nous avons identifié que la prévalence de ces Ac était de 0,36%
, Quatre patients (4,4%) présentaient une pathologie cancéreuse et quatre patients (4,4%) avaient une maladie infectieuse. Douze patients (13,3%) souffraient de maladies diverses
, Lorsque l'on comparait les caractéristiques entre les patients porteurs d'Ac anti-NuMA1 par rapport à ceux porteurs d'Ac anti-NuMA2, on notait un titre d'Ac antinucléaires plus élevé (1/789,7 versus 1/457,5), une association plus fréquente à d'autres auto-Ac (26,2% versus 15,9%) et une association plus fréquente aux maladies auto-immunes (72,4%, versus 60,2%) chez les patients avec Ac anti-NuMA1. Leur comparaison a permis de montrer que les patients avec des maladies auto-immunes et des anti-NuMA étaient plus jeunes (52,1 ans versus 54,7 ans), davantage des femmes (85,3% versus 66,3%) et avaient un titre d'Ac antinucléaires plus important (1/1192,3 versus 1/595,5), une association à d'autres auto-Ac, En réalisant une revue exhaustive de la littérature, nous avons identifié 12 études rapportant les caractéristiques de patients porteurs d'Ac anti-NuMA, qui présentaient en moyenne les mêmes caractéristiques que les patients de notre cohorte, vol.29
étant les deux maladies auto-immunes les plus fréquemment retrouvées chez les patients porteurs d'Ac anti-NuMA, nous avons décidé d'étudier l'impact de la présence d'Ac anti-NuMA sur le phénotype de ces patients par rapport à ceux qui ne possèdent pas de tels Ac ,
, Nous avons observé que les patients avec Ac anti-NuMA étaient moins sujets au syndrome sec ophtalmique (70% versus 89,1%, p = 0,031) ou à des complications du syndrome sec (15% versus 39,4%, p = 0,045), moins porteurs d'Ac anti-ENA (40% versus 68,6%, p = 0,022), notamment d'Ac anti-SSa et/ou SSb (40% versus 66,4%, p = 0,027), et avaient reçu moins de traitements (nombre médian de lignes de traitement : 1 versus 2, p < 0,01), 30% versus 24,8%), neurologiques du SSp (15% versus 5,8%), et moins de syndrome sec buccal (80% versus 86,1%), de manifestations rhumatologiques
, 019) et avaient moins d'auto-Ac (50,0% versus 85,0%, p < 0,01) en particulier moins d'Ac anti-ADN natifs (42,9% versus 75,0%, p = 0,025) et d'anti-SSa/SSb (7,1% versus 45,0%, p < 0,01), moins de consommation du complément (21,4% versus 53,8%, p = 0,040), moins d'hypergammaglobulinémie polyclonale (20,0% versus 61,0%, p = 0,019) et plus de thrombopénie (35,7% versus 12,5%, p = 0,044) que les patients sans Ac anti-NuMA. Les patients avec anti-NuMA avaient reçu moins de traitement par antipaludéen de synthèse, Nous avons alors comparé 14 patients avec un LES et des Ac anti-NuMA et 80 patients atteints de LES sans anti-NuMA. Nous avons pu noter que les patients avec anti-NuMA ne présentaient pas de glomérulonéphrite lupique (0% versus 28, vol.8, p.0
en évidence que les patients présentant un Ac anti-NuMA avaient un profil phénotypique et évolutif particulier qui pourrait être lié au rôle particulier de ces auto ,
,
, Au moment d'être admis(e) à exercer la médecine, je promets et je jure d'être fidèle aux lois de l'honneur et de la probité