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In order to describe the clinical features and the epidemiologic findings of 1,383 patients hospitalized in France for acute or chronic Q fever, we conducted a retrospective analysis based on 74,702 sera tested in our diagnostic center, National Reference Center and World Health Organization Collaborative Center for Rickettsial Diseases. The physicians in charge of all patients with evidence of acute Q fever (seroconversion and/or presence of IgM) or chronic Q fever (prolonged disease and/or IgG antibody titer to phase I of Coxiella burnetii > or = 800) were asked to complete a questionnaire, which was computerized. A total of 1,070 cases of acute Q fever was recorded. Males were more frequently diagnosed, and most cases were identified in the spring. Cases were observed more frequently in patients between the ages of 30 and 69 years. We classified patients according to the different clinical forms of acute Q fever, hepatitis (40%), pneumonia and hepatitis (20%), pneumonia (17%), isolated fever (17%), meningoencephalitis (1%), myocarditis (1%), pericarditis (1%), and meningitis (0.7%). We showed for the first time, to our knowledge, that different clinical forms of acute Q fever are associated with significantly different patient status. Hepatitis occurred in younger patients, pneumonia in older and more immunocompromised patients, and isolated fever was more common in female patients. Risk factors were not specifically associated with a clinical form except meningoencephalitis and contact with animals. The prognosis was usually good except for those with myocarditis or meningoencephalitis as 13 patients died who were significantly older than others. For chronic Q fever, antibody titers to C. burnetii phase I above 800 and IgA above 50 were predictive in 94% of cases. Among 313 patients with chronic Q fever, 259 had endocarditis, mainly patients with previous valvulopathy; 25 had an infection of vascular aneurysm or prosthesis. Patients with endocarditis or vascular infection were more frequently immunocompromised and older than those with acute Q fever. Fifteen women were infected during pregnancy; they were significantly more exposed to animals and gave birth to only 5 babies, only 2 with a normal birth weight. More rare manifestations observed were chronic hepatitis (8 cases), osteoarticular infection (7 cases), and chronic pericarditis (3 cases). Nineteen patients were observed who experienced first a documented acute infection, then, due to underlying conditions, a chronic infection. To our knowledge, we report the largest series of Q fever to date. Our results indicate that Q fever is a protean disease, grossly underestimated, with some of the clinical manifestations being only recently reported, such as Q fever during pregnancy, chronic vascular infection, osteomyelitis, pericarditis, and myocarditis. Our data confirm that chronic Q fever is mainly determined by host factors and demonstrate for the first time that host factors may also play a role in the clinical expression of acute Q fever.

Objective Recent studies have shown that in vitro endothelial cells are activated by antiphospholipid antibodies and may support leukocyte adhesion. We studied levels of soluble intercellular adhesion molecule 1 (sICAM-1, sCD54), soluble vascular cell adhesion molecule 1 (sVCAM-1, sCD106), and soluble E-selectin (soluble endothelial leukocyte adhesion molecule 1 [sELAM-1, sCD62E]) in sera from patients with primary antiphospholipid syndrome (primary APS), and compared them with those from patients with systemic lupus erythematosus–associated APS (SLE-APS) or pure SLE, as well as with those from 2 control groups composed of healthy volunteers and patients with thrombosis unrelated to autoimmune diseases. Methods Serum samples from 24 patients with primary APS, 15 patients with SLE-APS, 22 patients with pure SLE, 48 control patients with thrombosis, and 18 healthy volunteers were examined using enzyme-linked immunosorbent assays specific for sICAM-1, sVCAM-1, and sELAM-1. Results Serum levels of sVCAM-1, but not sICAM-1 or sELAM-1, were significantly increased in all patient study groups compared with thrombosis control patients and healthy volunteers, but did not differ between the groups of patients with primary APS, SLE-APS, or pure SLE. Concentrations of sVCAM-1 were significantly higher in primary APS or SLE-APS patients with severe, recurrent thrombosis and were negatively correlated with platelet counts in primary APS patients. In patients with primary APS, sVCAM-1 levels were higher if there was thrombotic kidney involvement and correlated with creatinemia. Conclusion Serum sVCAM-1 concentrations are increased in patients with primary APS, especially those with repeated thrombotic events or kidney involvement. These findings suggest that endothelial/monocyte interaction may be important in the pathogenesis of primary APS.

The authors report the observation of a 31-year-old woman who presented with total occlusion of digestive arteries and was successfully treated pharmacologically. A review of the literature shows that digestive arteries thrombosis is a rare condition in young women. Only 15 cases have been previously reported. Prognosis was poor with a mortality rate of 71%; 93% used oral contraception and more than 50% were smokers. All these cases were curiously similar. The authors accentuate the role of iloprost in the recovery of the patient. Digestive artery occlusion should be taken into consideration in the differential diagnosis of abdominal pain, especially in young women who smoke and take estrogen-containing birth control pills.

Introduction La maladie associee aux IgG4 (MAG-4) est une pathologie fibrosante et inflammatoire pouvant toucher un ou plusieurs organes au cours de son evolution. Elle est habituellement tres cortico-sensible, mais pose le probleme de rechutes frequentes ou de corticodependance justifiant l’utilisation d’immunosuppresseurs. Tres peu de donnees sont disponibles sur l’efficacite du rituximab (RTX) dans cette indication. Patients et methodes Analyse retrospective des observations de patients atteints de MAG-4 de la cohorte nationale francaise ayant beneficie d’un traitement par RTX. Les caracteristiques des patients, le type de localisation et les formes cliniques de la maladie, ainsi que les traitements recus ont ete analyses. L’indication retenue aupres des cliniciens pour l’utilisation du RTX, son efficacite et sa tolerance ont ete evalues. Resultats Vingt et un patients, sur les 121 patients declares dans la cohorte francaise de la MAG-4, ont beneficie d’un traitement par RTX. Des donnees completes etaient disponibles pour 17 d’entre eux, dont 11 avec un suivi par rapport au traitement par RTX superieur a 6 mois. Le nombre et le type d’atteintes d’organes etaient variables, mais les atteintes motivant le recours au RTX etaient surtout representees par l’atteinte renale (n = 6), l’atteinte aortique ou peri-aortique (fibrose retro-peritoneale) (n = 5) et l’atteinte des voies biliaires (cholangite) (n = 5). Hormis 1 patient traite par RTX en 1re ligne, le RTX etait administre dans tous les cas apres un traitement par corticoides ± immunosuppresseurs. Le RTX etait administre apres un delai d’evolution de la maladie median de 37 mois (extremes [5–180]). Dans les 11 cas avec un suivi > 6 mois apres l’administration, on retenait une reponse clinique dans 8/11 cas (73 %), avec baisse des IgG4 seriques dans 5/5 cas evaluables. Parmi les 8 patients ayant repondu au traitement, et avec un suivi median de 19 mois (extremes [1–72]), on notait une rechute dans 2 cas. L’arret de toute corticotherapie ou traitement immunosuppresseur n’etait possible, apres RTX, que dans 2 cas. Le traitement etait dans l’ensemble bien tolere. Discussion Le RTX semble efficace dans le traitement de la MAG-4, chez des patients refractaires ou cortico-dependants. Nous avons observe une reponse clinique chez pres de 75 % des patients. Ces chiffres sont discretement inferieurs aux resultats preliminaires rapportes par une equipe americaine dans un premier essai prospectif ouvert non comparatif (reponse chez pres de 90 % des patients). Ceci est peut-etre en rapport avec la duree d’evolution de la maladie avant traitement plus longue dans notre etude. Conclusion Le RTX constitue une alternative therapeutique au cours de la MAG4 particulierement dans les formes les plus systemiques de la maladie, les formes localisees severes, ou en cas de maladie refractaire, cortico-dependante ou en rechute. Le schema posologique optimal, et les modalites d’un eventuel traitement d’entretien restent a preciser.

Introduction Proprionibacterium acnes est un germe saprophyte de la peau principalement implique dans des infections osteo-articulaires chroniques de materiel ou de site operatoire. Nous decrivons un cas inhabituel d’infection osseuse a ce germe chez un patient immmunocompetent, sans antecedent chirurgical. Observation Il s’agit d’un patient de 65 ans se presentant dans notre service pour un aspect de grosse jambe gauche douloureuse evoluant depuis 2 ans. La douleur, d’horaire inflammatoire, a debute suite a un traumatisme minime de la face anterieure de jambe contre une barre de fer sans breche cutanee visible. Huit mois apres ce traumatisme, devant la persistance des douleurs, une scintigraphie osseuse avait ete realisee montrant un aspect evocateur d’hematome avec probable lesion tibiale. A l’examen dans le service, le patient presente un mollet gauche augmente de volume, chaud, sans fievre. Le bilan biologique montre un syndrome inflammatoire modere (CRP a 12), sans hyperleucocytose. Le bilan auto-immun et infectieux est negatif. Une IRM de jambe montre un aspect de periostite anteromediale du tibia d’allure evolutive, avec anomalies de signal au sein du muscle long flechisseur des orteils, des muscles fibulaires et du muscle long extenseur des orteils. Une biopsie chirurgicale est realisee. L’examen anatomopathologique retrouve un tissu osseux et musculaire inflammatoire, sans cellule tumorale. Deux prelevements sont positifs en culture a P. acnes. Devant l’absence d’autre hypothese diagnostique, un traitement antibiotique d’epreuve est debute par amoxicilline 6 g par jour pendant 3 mois. A 2 mois de la fin du traitement les douleurs ont regresse, la jambe a diminue de volume et une IRM de controle montre une disparition des anomalies de signal musculaire, et une stabilite des atteintes periostees. Discussion P. acnes est un pathogene emergent rapporte de maniere croissante dans les infections postoperatoires principalement au niveau membre superieur (epaule) et du rachis. La differenciation entre infection et colonisation repose sur l’isolement du germe en culture sur 2 prelevements au moins, ce qui est le cas pour notre patient. Le tableau clinique et le plus souvent torpide, sans signes inflammatoires systemiques francs. Il existe un seul cas dans la litterature d’osteomyelite tibiale chronique, chez un patient drepanocytaire. Chez notre patient on peut emettre l’hypothese d’une breche cutanee passee inapercue au moment du traumatisme, ayant permis la translocation de ce germe vers les parties molles, le muscle et le perioste. Conclusion Les internistes doivent savoir evoquer une infection a P. acnes devant un tableau de periostite ou myosite localisee chronique.

Objective. To characterize muscle metabolic anomalies associated with polymyalgia rheumatica (PMR) using P-31 magnetic resonance spectroscopy (MRS). Methods. Seventeen patients with PMR and 9 age-matched control subjects were investigated. The forearm flexor muscles were examined by P-31 MRS in a 4.7 T superconducting horizontal magnet (Biospec Bruker 47/30) during a rest-exercise-recovery protocol. The intracellular pH and the relative concentrations of phosphocreatine (PCr), inorganic phosphate (Pi), ATP, and phosphomonoesters were measured every minute during the protocol. Based on the PCr and pH timedependent changes during exercise and recovery, the rates of ATP production from PCr hydrolysis, glycogenolysis, and aerobic metabolism were calculated for each minute of exercise. Results. At rest, the metabolic parameters [PCr]: [Pi], pH, and [PCr]:[ATP] were not significantly different between the PMR patients and the control subjects. During exercise, the energetic cost and the contribution of anaerobic and oxidative pathways to energy supply were similar in the 2 groups, as were the recovery kinetics of the phosphorylated compounds and pH. Conclusion. Muscle MRS does not confirm the findings of histologic and biochemical studies that suggest an alteration of mitochondrial function in PMR. No other modifications in glycolytic or glycogenolytic pathways or in proton handling were found that could indicate an alteration of muscle energetics in patients with PMR.

To determine the prevalence of autoantibodies in patients with epilepsy and to find a possible relationship between antinuclear antibodies (ANA) and/or anticardiolipin (aCL) antibodies and epilepsy.One hundred sixty-three consecutive, unselected patients followed at the Centre Saint-Paul, a French medical center specialized in epilepsy, were included in the study. IgG and IgM class aCL antibodies were measured by an enzyme-linked immunosorbent assay (ELISA). IgG class ANA was detected by an indirect immunofluorescence technique with Hep2 cells as the substrate. Sera from 100 healthy blood donors, matched for age and sex, were used as controls.In 31 sera, IgG class a aCL antibodies were detected at a value higher than 17 GPL unit (19%, P = 0.0003); 10 of them had a value higher than 35 GPL unit. IgM class aCL antibodies were not detected at a significant value. For 6 of the 31 sera, there was a beta 2-glycoprotein I dependence. None of the patients with aCL antibodies in the serum had a past history of deep venous or arterial thrombosis. ANA were detected in the sera from 41 patients (25%, P0.005). The presence of autoantibodies in the serum was not statistically dependent on the type of epilepsy, the kind of antiepileptic drug, or the age or sex of the patients.Our study suggests that there is a relationship between epilepsy and aCL antibodies, even in the patients without systemic lupus erythematosus. Large prospective studies are needed to define the role of the aCL antibodies and ANA in pathophysiology of epilepsy.

Context: Cyclophosphamide is used for renal and major extrarenal involvement in systemic lupus erythematosus (SLE) and is associated with a risk of premature ovarian failure. There are no data available about the relation between anti-Müllerian hormone (AMH) serum levels and the probability of subsequent pregnancy in SLE patients. Objective: We analyzed AMH levels and the probability of pregnancy in SLE women exposed to cyclophosphamide. Design and Setting: We conducted a matched cohort study in referral centers for SLE. Patients: Fifty-six cyclophosphamide-exposed SLE women younger than 40 years of age and 56 control SLE women matched for age within 6 months participated in the study. Main Outcome Measures: AMH was measured in samples from the PLUS study (ClinicalTrials.gov no. NCT00413361). All patients were interviewed in May 2012 regarding their obstetric status. Results: The mean age ± SD of the 112 patients was 31.6 ± 5.8 years. The mean AMH level was low (1.21 ± 1.01 ng/mL) and was significantly lower in patients exposed to cyclophosphamide (P = .03) and in patients older than 30 years (P = .02). During a median follow-up (interval between sampling and the interview) period of 4.2 (range, 2.5–4.8) years, 38 patients sought to become pregnant, and 32 (84.2%) succeeded. In the univariate analysis, the risk of failure was associated with cumulative cyclophosphamide dose (P = .007) and older age (P = .02), but not with AMH. Conclusion: We confirmed that AMH levels are low in SLE patients and decrease significantly with age and cyclophosphamide exposure. Nonetheless, the risk of failure to conceive was low and was predicted by cyclophosphamide exposure and age, but not by AMH levels.

Resume L’assimilation de plus en plus frequente de l’hospitalisation en psychiatrie a l’enfermement necessite une reflexion sur la portee de cette modalite de soin. L’objectif de ce travail est d’explorer le vecu et la perception des effets de cette experience au regard d’une perspective ethique. L’etude etait fondee sur une enquete par questionnaire realisee aupres de 169 patients au terme de leur sejour hospitalier. Une analyse statistique descriptive a ete realisee avec le logiciel SPSS 17.0 et les resultats examines au filtre des principes ethiques fondamentaux (autonomie, bienfaisance, non-malfaisance). Elle a mis en evidence que l’autonomisation est favorisee par l’existence de reperes dans le deroulement des soins (plus de 85 %), la liberte de circulation (82 %), l’information sur le projet therapeutique (83 %), et remise en cause surtout en matiere d’implication dans l’orientation des soins et d’information sur le mode d’hospitalisation (67 %) et la maladie (71 %). La bienfaisance est respectee tant au niveau de la disponibilite des soignants (94 %) que des effets benefiques reconnus et de l’utilite (90 %). Cependant, l’hospitalisation est une experience potentiellement chargee d’affects negatifs (vecu penible 50 %, impuissance 40 %) qui pourraient etre attenues par une ecoute plus active, ainsi qu’une information et une responsabilisation accrues des personnes hospitalisees.

European and American recommendations have recently been published for the treatment of proliferative lupus nephritis (LN). This study aimed to describe current practice in France. An electronic survey was sent to French nephrologists and internists via their scientific society between March and December 2012. One hundred and nine specialists (60 internists, 48 nephrologists and 1 rheumatologist), mostly from hospitals, completed the survey. Low-dose cyclophosphamide (Euro-Lupus) was the first induction immunosuppressive therapy used (67%), followed by mycophenolate mofetil (MMF) (20%) and high dose CYC (NIH, 9%). Maintenance immunosuppressive therapy after an induction with CYC was preferentially MMF (58%), versus 14% for azathioprine (AZA) and 25% using either MMF or AZA without preference. After an induction with MMF, maintenance treatment was mainly MMF (77%). Antimalarial drugs were prescribed systematically by 86% of specialists. In patients in stable remission, maintenance treatment was withdrawn after 2 years (40%), 3 years (25%) or more (34%). Low-dose corticosteroids were continued in the long-term by 54% of specialists. No difference was observed between nephrologists and internists, even in the prescription of antimalarials. Treatment of proliferative LN in France is homogenous enough and is consistent with recent international recommendations.

To evaluate the usefulness of 2-[18F]-fluoro-2-deoxy-d-glucose-positron emission tomography/computed tomography (FDG-PET/CT) in IgG4-related disease (IgG4-RD) for the staging of the disease and the followup under treatment.All patients included in the French IgG4-RD registry who underwent ≥1 FDG-PET/CT scan were included in the study. Clinical, biologic, pathologic, radiologic, and FDG-PET/CT qualitative and quantitative findings were retrospectively collected and analyzed.Twenty-one patients were included in the study and 46 FDG-PET/CT examinations were evaluated. At either diagnosis or relapse, all evaluated patients presented abnormal 18F-FDG uptake in typical IgG4-RD localizations. In most cases, FDG-PET/CT was more sensitive than conventional imaging to detect organ involvement, especially in arteries, salivary glands, and lymph nodes. In few cases (small-sized lesions and brain or kidney contiguous lesions), false-negative results were noted. Evaluation before and after treatment showed in most cases a good correlation of FDG-PET/CT results with treatment response and disease activity.This large retrospective study shows that FDG-PET/CT imaging is useful for the staging of IgG4-RD. Moreover, FDG-PET/CT is useful to assess the response to treatment during followup.

Immunoglobulin free light chains are produced in excess during normal antibody synthesis. Their evaluation is commonly used in case of a monoclonal gammopathy. In polyclonal hypergammaglobulinemia related to the Sjögren syndrome or systemic lupus, erythematosus serum free light chain levels are increased and could correlate with disease activity. We show here that theκ() andλ() free light chains and theκ : λratio () are increased in sixteen patients with IgG4-related disease when compared to healthy controls. The increase ofκandλfree light chains probably reflects the marked polyclonal B cell activation of the disease. We could not assess in this small cohort of patients a significative correlation of serum free light chain levels and disease activity or extension.

Autoimmune hemolytic anemia (AIHA) is rare and difficult to treat during chronic hepatitis C. We report herein the case of a hepatitis C patient with severe and resistant AIHA who experienced a good and sustained response with anti-CD20 monoclonal antibody treatment.

Inflammatory myopathy is a major clinical manifestation of the antisynthetase syndrome. Patients with anti Jo-1 antibodies can also present with other clinical manifestations as arthritis, mechanic's hands and lung fibrosis. As in dermatomyositis, polymyositis and overlap myopathies proximal muscle of the limbs are preferentialy involved. Associated fascia edema on magnetic resonance imagery studies with histological proven fasciitis has been reported. However peripheral limb involvement, reported here in two patients, as the isolated initial manifestation is unusual.

Introduction Les explorations de symptomatologies vertigineuses sont souvent laborieuses des que ceux-ci ne sont ni d’origine peripherique ni d’origine centrale. L’hypothese psychosomatique est parfois proposee notamment chez une personne jeune. Nous rapportons un cas d’epilepsie vestibulaire de diagnostic difficile. Observation Un jeune de 35 ans etait adresse en consultation pour une asthenie inexpliquee et des malaises atypiques evoluant depuis 2013. Ces malaises survenaient notamment lors de discussions soutenues ou de stimulation visuelle. Le stress et les emotions declenchaient egalement ces sensations d’instabilite. Il etait gene dans sa vie quotidienne au point d’avoir arreter les competitions de planche a voile. Il avait comme seul antecedent une thyroidite d’Hashimoto avec des anticorps anti-TPO tres positifs equilibree par opotherapie substitutive. L’examen clinique ne retrouvait pas de syndrome vestibulaire central ou peripherique. Le patient signalait par ailleurs des troubles de l’attention et parfois des troubles de l’elocution mais toujours en contexte anxiogene. L’IRM cerebrale etait normale. L’electroencephalogramme realise mettait en evidence des anomalies irritatives soit sous la forme de bouffees assez degradees de pointes et d’ondes lentes, soit la presence d’activites pointues dans la region temporo-parieto-occipitale a predominance droite. Ces anomalies etaient confirmees sur l’electroencephalogramme de sieste. L’instauration d’un traitement par benzodiazepine a permis la resolution des crises. Discussion Les epilepsies vestibulaires se rencontrent plus chez le sujet jeune et sont generalement d’evolution favorable [1] . Elles peuvent se traduire par des manifestations d’instabilite comme chez notre patient ou de grands vertiges en fonction de la topographie de la zone epileptogene. Elles peuvent reveler des cavernomes temporales et justifient une imagerie cerebrale [2] . Conclusion Les epilepsies vestibulaires peuvent se reveler par des manifestations atypiques minimes mais invalidants. L’association de signes de la lignee epileptique (manifestations neurologiques autres transitoires) permet d’evoquer le diagnostic et d’instaurer une therapeutique specifique.

The lupus anticoagulant-hypoprothrombinemia syndrome (LAHS)--the association of acquired factor II deficiency and lupus anticoagulant--is a rare disease drastically different from antiphospholipid syndrome in that it may cause predisposition not only to thrombosis but also to severe bleeding. We performed a retrospective study of 8 patients with LAHS referred to 6 French tertiary care centers between January 2003 and February 2011, and a literature review retrieving all related articles published between 1960 and April 2011. Including our 8 new cases, LAHS has been reported in 74 cases. The disease mostly occurs in young adults, with a female to male sex ratio of 1.4. Associated conditions mostly include autoimmune diseases such as systemic lupus erythematosus and infectious diseases. Bleeding is a frequent feature (89% of cases), while arterial and/or venous thrombosis is less common (13%). Factor II level is severely decreased at diagnosis (median value, 11%; range, 1%-40%). LAHS associated with autoimmune diseases is more persistent than LAHS associated with infection, and hemorrhagic complications are more common. Corticosteroids should be considered the first-line treatment, but the thrombotic risk strongly increases during treatment because of the improvement of factor II level. Despite the fact that 50% of patients develop severe bleeding, the mortality rate is

Introduction L’analyse precise des causes et des comorbidites associees au deces chez les patients atteints de drepanocytose est importante pour chaque centre afin de reconnaitre les situations a risque et d’ameliorer notre prise en charge hospitaliere. Patients et methodes Nous avons realise une etude retrospective portant sur l’ensemble des patients atteints d’un syndrome drepanocytaire majeur, enfants et adultes, suivis au CHU de Marseille et decedes entre 2005 et 2015. Nous avons analyse les causes du deces en distinguant ceux principalement lies : – aux complications aigues de la drepanocytose (crise vaso-occlusive, syndrome thoracique aigu, anemie) ; – a une complication septique ; – aux defaillances chroniques d’organes secondaires a la drepanocytose ; – a une cause autre ou non identifiee. Resultats Sur la periode etudiee, 10 deces sont rapportes dans notre centre. Deux enfants sont decedes (âge au deces : 10 et 14– ans), tous deux dans un contexte septique (infection digestive severe et meningite a pneumocoque). Dans les deux cas, les vaccinations recommandees et notamment anti-pneumococcique etaient a jour. Aucun ne presentait de complication chronique de la maladie. Huit adultes sont decedes (âge moyen et median au deces : 40– ans, extremes : 19–69– ans). Parmi ceux-ci, un seul patient est decede d’une complication aigue de la drepanocytose (embolie graisseuse secondaire a une necrose medullaire etendue). Cinq patients sont decedes sur des complications chroniques d’organes. Parmi eux, 4 avaient une cirrhose evoluee : les causes du deces etaient 1 choc septique dans les suites d’une transplantation hepatique, 1 choc hemorragique dans les suites immediates d’une ponction-biopsie hepatique, 1 choc hemorragique avec defaillance multiviscerale dans un contexte de crise vaso-occlusive, 1 decompensation d’une encephalopathie hepatique ; le cinquieme deces etait lie a une defaillance cardiaque droite sur une hypertension pulmonaire severe. Enfin, 2 patients sont decedes de causes autres ou non identifiees : l’un est decede de mort subite sur arret cardiaque sans origine evidente retrouvee a l’autopsie, en dehors de tout contexte de vaso-occlusion ou de defaillance chronique d’organe ; le deuxieme est decede au volant de sa voiture sans cause precise retrouvee chez un patient avec des antecedents d’hypertension pulmonaire et d’hemorragie meningee anevrysmale. Aucun patient decede ne beneficiait d’un traitement par hydroxyuree. Trois patients adultes beneficiaient d’un programme regulier d’echanges transfusionnels et 4 patients adultes avaient beneficie en urgence dans les semaines precedant le deces d’echanges erythrocytaires (dont 3 en raison de l’aggravation aigue d’une insuffisance d’organe chronique). Hormis 4 d’entre eux, tous les patients sont decedes en service de reanimation. Discussion Si les complications aigues de la drepanocytose representaient autrefois une des principales causes de deces, l’analyse des causes et comorbidites associees aux deces chez les patients drepanocytaires enfants et adultes de la cohorte marseillaise retrouve une faible representation des deces secondaires aux phenomenes aigus de vaso-occlusion. Si les deux deces pediatriques sont lies a des infections severes, 5 des 8 adultes decedent des consequences directes d’une complication chronique d’organe de la maladie, representee dans notre cohorte essentiellement par l’hepatopathie multifactorielle de la drepanocytose, et ce en depit de la prise en charge chronique et des therapeutiques d’urgence (echanges erythrocytaires, suppleance d’organes, transplantation hepatique, traitement d’hypertension pulmonaire). Conclusion Si la mortalite des enfants atteints de syndrome drepanocytaire majeur est encore associee a la survenue d’infections severes, celle des adultes semble dominee par les defaillances d’organes compliquant l’evolution chronique de la maladie. Cette etude souligne l’importance de la mise en place de strategies diagnostiques et therapeutiques precoces et adaptees afin de prevenir l’installation progressive d’insuffisances d’organes terminales.

Introduction Les encephalites limbiques auto-immunes representent un groupe nosologique heterogene d’atteintes dysimmunes du systeme nerveux central dont le traitement n’est pas codifie en raison de leur rarete. Les differentes lignes therapeutiques proposees en phase aigue reposent sur la corticotherapie a forte dose, les immunoglobulines intraveineuses et les seances d’echange plasmatique. L’efficacite de ces differents traitements, rapportee dans quelques observations, est tres inconstante. Nous rapportons le cas d’un patient porteur d’une encephalite limbique a anti-CASPR2 ayant repondu de maniere spectaculaire aux seances d’echanges plasmatiques. Observation Un patient de 65 ans est hospitalise pour prise en charge diagnostique d’une confusion avec amnesie anterograde evoluant depuis 1 mois, associee a des mouvements anormaux a type de myoclonies diffuses. L’histoire commence 8 mois auparavant avec une modification de l’humeur (irritabilite inhabituelle) notee par l’entourage du patient. Progressivement apparait une alteration de l’etat general avec perte de 4 kg faisant decouvrir une masse mediastinale. L’exerese conclut a un thymome benin. A j1 postoperatoire, apparait un etat confusionnel avec amnesie a predominance anterograde nette, des myoclonies diffuses quasiment permanentes, et des troubles de la deglutition. Les premiers examens realises ne permettent pas de retrouver d’anomalies expliquant sa degradation clinique (pas d’anomalies metaboliques, electro-encephalogramme normal, imagerie par resonance magnetique cerebrale normale, electromyogramme normal). L’examen du liquide cephalo-rachidien ne retrouvait pas d’hypercellularite, ni hyperproteinorachie, ni de synthese intrathecale. Le dosage de la proteine 14,3.3 est normal. Le PET-TDM cerebral retrouve un aspect compatible avec le diagnostic d’encephalite limbique, a savoir un hypermetabolisme putaminal bilateral, s’associant a un hypometabolisme cerebral diffus marque. Le traitement est debute sur ces arguments. La recherche d’anticorps associee aux encephalites limbiques reviendra positive plus tard pour les anticorps anti-CASPR2 confirmant le diagnostic de maniere formelle. Sur le plan therapeutique, une corticotherapie a forte dose est debutee (methylprednisolone 1 g/jour sur 5 jours) sans efficacite clinique evidente. Un relais par des seances d’echanges plasmatiques est entrepris a un rythme de 3 seances par semaine pendant 2 semaines puis 1 seance par semaine pendant 2 semaines soit un total de 8 seances. Une amelioration clinique franche est notee 7 jours apres le debut des echanges plasmatiques avec disparition des myoclonies et ensuite de l’amnesie anterograde et de l’etat confusionnel. Un traitement d’entretien par rituximab 1 G a 15 jours d’intervalle est instaure. Conclusion Les encephalites limbiques representent un groupe nosologique d’atteinte dysimmune du systeme nerveux central mieux comprises sur le plan immunologique depuis une dizaine avec la mise en evidence d’anticorps specifiques notamment anti-canaux potassiques (VGKC) qui ciblent des antigenes membranaires neuronaux. Les encephalites limbiques a anti-CASPR2 font parties integrantes du sous-groupe des anti-VGKC [avec les Anti-Leucine-Rich Glioma-Inactivated Protein 1 (LGI1)]. Si la clinique est bien decrite, le traitement quant a lui reste tres empirique avec des etudes pour la plupart retrospectives incluant un faible nombre de patients. La litterature rapporte une vingtaine de cas ayant repondu a des seances d’echanges plasmatiques, ce traitement etant desormais propose en 2e ligne therapeutique apres echec de la corticotherapie ou des immunoglobulines. Cette observation conforte l’efficacite des echanges plasmatiques dans le traitement des encephalites limbiques a anticorps anti-VGKC.

IgG4-related systemic disease is now recognized as a systemic disease that may affect various organs. The diagnosis is usually made in patients who present with elevated IgG4 in serum and tissue infiltration of diseased organs by numerous IgG4+ plasma cells, in the absence of validated diagnosis criteria. We report the clinical, laboratory, and histologic characteristics of 25 patients from a French nationwide cohort. We also report the treatment outcome and show that despite the efficacy of corticosteroids, a second-line treatment is frequently necessary. The clinical findings in our patients are not different from the results of previous reports from Eastern countries. Our laboratory and histologic findings, however, suggest, at least in some patients, a more broad polyclonal B cell activation than the skewed IgG4 switch previously reported. These observations strongly suggest the implication of a T-cell dependent B-cell polyclonal activation in IgG4-related systemic disease, probably at least in part under the control of T helper follicular cells.

Objective. Scleroderma renal crisis (SRC) is a severe manifestation of SSc, whose prognosis remains severe, despite treatment with angiotensin-converting-enzyme inhibitor and dialysis. This study was undertaken to describe SRC characteristics, prognosis and outcome, and evaluate the responsibility of CSs in its occurrence.Methods. Analysis concerned 91 SSc patients with SRC who were compared with 427 non-SRC–SSc patients taken as controls.Results. Among the 91 SRC patients, 71 (78.0%) had high blood pressure, 53 (58.2%) hypertensive encephalopathy and 51 (56.0%) thrombotic microangiopathy; 64 (70.3%) had received CSs before or concomitantly with SRC vs 156 (36.5%) non-SRC–SSc patients (P < 0.001). Treated SRC patients also received more prednisone 29.3 (28.4) vs 3.6 (9.9) mg than controls (P < 0.001). SRC clinical outcomes were poor: 49 (53.8%) patients required dialysis, which was definitive for 38. Thirty-seven (40.7%) SRC patients died vs 10.8% of the controls (P < 0.001). Death was most frequent among dialysed patients who never recovered renal function (22 vs 2) and 13 never-dialysed SRC patients died.Conclusions. Although SRC prognosis has improved markedly, SRC remains a severe manifestation of SSc, despite treatment with angiotensin-converting enzyme inhibitor and dialysis. CSs contributed significantly to SRC occurrence.

Exposure to sunlight is one of the environmental factors involved in the pathogenesis of systemic lupus erythematosus. We investigated whether there is seasonal variation in the incidence of cutaneous and noncutaneous severe lupus flares in southern France.We retrospectively reviewed clinical and biological data from all SLE patients hospitalized for a flare of the disease during a two year period in our centre and collected corresponding meteorological data from the official website of MeteoFrance.Forty one patients, mean age 36.7 ± 13.8 years, were included. Twenty-six patients (63.4%) had kidney biopsy performed, showing in all cases proliferative nephritis, associated with membranous nephritis in 9 (22%). We found a clear seasonal pattern for overall lupus flares with 39% of flares occurred in Spring. Among patients without any cutaneous involvement, this seasonal pattern was still observed (p=0.024). Patients under antimalarials presented flares significantly later in the sunny season than those without (respectively median in July versus May, p=0.044). There were strong positive correlations between occurrence of lupus flares and maximum temperature increase (ρ=0.87, p0.001), minimum temperature increase (ρ=0.87, p0.001), and duration of sunshine increase (ρ=0.78, p=0.003). These correlations were also observed in patients with renal flares.We confirmed a seasonal pattern for lupus flares among patients living in Southern France, with most flares in spring, in correlation with an increase in temperature and duration of sunshine. A similar seasonal pattern was observed in patients with no cutaneous involvement and with visceral involvement.

vascularity. Am J Reprod Immunol 2004;51:257–68. 45. Wong M, Toh L, Wilson A, Rowley K, Karschimkus C, Prior D, et al. Reduced arterial elasticity in rheumatoid arthritis and the relationship to vascular disease risk factors and inflammation. Arthritis Rheum 2003;48:81–9. 46. Cooper GS. Unraveling the etiology of systemic autoimmune diseases: peering into the preclinical phase of disease. J Rheumatol 2009;36:1853–5. 47. Leslie D, Lipsky P, Notkins AL. Autoantibodies as predictors of disease. J Clin Invest 2001;108:1417–22. 48. Majka DS, Holers VM. Can we accurately predict the development of rheumatoid arthritis in the preclinical phase? [editorial]. Arthritis Rheum 2003;48:2701–5. 49. Rantapaa-Dahlqvist S, de Jong BA, Berglin E, Hallmans G, Wadell G, Stenlund H, et al. Antibodies against cyclic citrullinated peptide and IgA rheumatoid factor predict the development of rheumatoid arthritis. Arthritis Rheum 2003;48:2741–9.

BackgroundRecently, metagenomic studies have identified viable Pepper mild mottle virus (PMMoV), a plant virus, in the stool of healthy subjects. However, its source and role as pathogen have not been determined.Methods and findings21 commercialized food products containing peppers, 357 stool samples from 304 adults and 208 stool samples from 137 children were tested for PMMoV using real-time PCR, sequencing, and electron microscopy. Anti-PMMoV IgM antibody testing was concurrently performed. A case-control study tested the association of biological and clinical symptoms with the presence of PMMoV in the stool. Twelve (57%) food products were positive for PMMoV RNA sequencing. Stool samples from twenty-two (7.2%) adults and one child (0.7%) were positive for PMMoV by real-time PCR. Positive cases were significantly more likely to have been sampled in Dermatology Units (pConclusionsOur study identified a local source of PMMoV and linked the presence of PMMoV RNA in stool with a specific immune response and clinical symptoms. Although clinical symptoms may be imputable to another cofactor, including spicy food, our data suggest the possibility of a direct or indirect pathogenic role of plant viruses in humans.

purpose: The coexistence of mitochondrial myopathy and polymyalgia rheumatica without giant cell arteritis is an interesting association. The frequency of this association was assessed in a prospective study. patients and methods: Muscle biopsy specimens were obtained from 15 patients with polymyalgia rheumatica. When ragged red fibers (RRF) were observed, histochemical, ultrastructural, and biochemical studies were performed. results: In five cases, we found the typical appearance of mitochondrial myopathy, with the presence of numerous RRF. Histochemical and biochemical results confirmed these mitochondrial myopathies, showing miscellaneous deficiencies of mitochondrial respiratory chain enzymes. conclusion: Persistence of histologic and biochemical abnormalities after steroid treatment in two patients seems to indicate that a subclinical mitochondrial myopathy preceded polymyalgia rheumatica. How a mitochondrial myopathy could induce or facilitate the emergence of a polymyalgia rheumatica remains unknown.

Anti-RNA polymerase III antibodies (anti-RNAP III) have been reported as potential immune markers of Systemic Sclerosis (SSc). Until now, their clinical use was disregarded because of technical difficulties to perform immunoprecipitation. Recently, ELISA kits became commercially available allowing an easy detection of anti-RNAP III. We intended to clarify the relevance of these antibodies in the diagnosis of SSc by ELISA detection.The prevalence of anti-RNAP III was analyzed using two ELISA kits in 50 consecutive SSc patients from Marseilles in South of France. Controls included 66 patients with other systemic autoimmune diseases, 34 viral diseases and 50 healthy subjects. Positive results with at least one ELISA kit were controlled by immunoprecipitation which is the reference assay.In this study, positivity for anti-centromere and/or anti-topoisomerase I antibodies was observed in 84% of SSc patients. The prevalence of anti-RNAP III in SSc patients was 0% to 6% (3/50) depending on the ELISA kit and only 2% by immunoprecipitation. Concerning controls, two rheumatoid arthritis patients were positive using ELISA (6%), including one with immunoprecipitation confirmation. No anti-RNAP III was detected in systemic lupus erythematosus patients. Three blood donors and one viral disease control were positive using ELISA, but all were negative by immunoprecipitation.Anti-RNAP III was rarely detected in a French population of SSc patients. Their prevalence was even lower than the one observed in rheumatoid arthritis controls. Therefore local immunologic profiles should be established before deciding a change in clinical practice for SSc immune screening.

To the Editor: In November 2007, a 69-year-old man with fever was hospitalized at the Northern Hospital in Marseilles, France. He also had diabetes, high blood pressure, and alcohol and tobacco addictions. In September 2007, he had received a diagnosis of laryngeal cancer, which required 2 chemotherapy treatments through a central venous catheter (CVC), the second of which he had received 6 days before his November visit. Prostatic cancer, diagnosed 1.5 years earlier, had been treated by radiotherapy. At the time of the November admission, he had leukopenia (1.77 × 109 leukocytes/L with 0.49 × 109 polymorphonuclear cells/L) and an elevated C-reactive protein level (151 mg/L). The patient was admitted with a preliminary diagnosis of drug-induced febrile granulocytosis; the origin of his fever remained unclear. Blood for culture was first collected from a peripheral vein and on the next day was collected from a peripheral vein and from the CVC. Gram-positive rods grew in the aerobic bottle from the CVC sample. The microorganism was identified by biochemical tests using API Coryne strip (bioMerieux, Marcy-l’Etoile, France) as Rhodococcus spp. (94% similarity). The day after hospital admission, the patient was empirically treated with intravenous ticarcillin-clavulanate, 5 g 3×/day; ciprofloxacin, 200 mg 2×/day; and granulocyte colony–stimulating factor. One day later, fever resolved and the polymorphonuclear cell count was within normal limits. Oral antimicrobial drug therapy was continued for 1 week. Bacterial identification of the strain was performed by 16S rRNA sequencing. We obtained a 1,464-bp sequence, which was found to differ at only 2 nt positions from that of Gordonia sputi (GenBank accession no. {"type":"entrez-nucleotide","attrs":{"text":"X80634","term_id":"562305","term_text":"X80634"}}X80634). We concluded that our patient had catheter-related bacteremia caused by G. sputi because he was immunocompromised and had a CVC. We ruled out a contaminant because the organism did not belong to the normal flora of human skin and because fever resolved after treatment with antimicrobial drugs. The genus Gordona was first described in 1971, for coryneform bacteria isolated from sputum of patients with pulmonary disease or from soil (1). It is a member of the mycolic acid–containing group consisting of genera Corynebacterium, Dietzia, Gordonia, Mycobacterium, Nocardia, Rhodococcus, and Tsukamurella. The genus has been revised several times by rearrangements with the genera Rhodococcus and Nocardia, and the name Gordona was changed to Gordonia in 1997. The genus belongs to suborder Corynebacterineae within the order Actinomycetales and currently contains 27 recognized species; only 7 have been described in human disease. Species are identified by molecular analysis. Gordonia spp. cause a wide spectrum of disease in humans ( 2–10; Table). Neurologic and vascular infections in immunocompromised and immunocompetent patients have been reported. Cutaneous and respiratory infections, otitis externa, osteitis, and arthritis have reportedly occurred only in immunocompetent patients. Bacteria have most often been isolated from blood samples. Bacteremia has started from underlying disease such as a sequestrated lung (4) or acute cholecystis (5) or has been related to coronary artery surgery (2) and frequently to CVCs (2,3,6). Catheter removal has been recommended for treatment of Gordonia spp. infections in children (6), but the recommendation varied for adults. Six cases of infection in children have been described (6), appearing as bacteremia, ventriculitis, and brain abscess. Table Summary of clinical reports involving Gordonia spp.* Microbiologic diagnosis of Gordonia spp. remains difficult. Biochemical profiles can lead to incorrect identification of isolates as Rhodococcus spp. (2,4–6,9) and sometimes Corynebacterium spp. (3) or Nocardia spp. (6). Identification at the genus and species levels is presently obtained by 16S rRNA sequence comparisons. No recommendation for antimicrobial drug susceptibility testing has been unanimously approved, but these microorganisms seem to be susceptible to many antimicrobial drugs (6). Previous studies suggest a combination of penicillins and aminoglycosides as a suitable therapy for Gordonia-related bacteremia (3). Carbapenem or fluoroquinolone in combination with an aminoglycoside can also be used (6). Antimicrobial drug susceptibility is similar to that of Rhodococcus spp., for which Gordonia spp. are usually incorrectly identified. However, although vancomycin is often used to treat Rhodococcus spp. infections, in a previous study 11% of Gordonia spp. isolates were resistant (6). Treatment must therefore be evaluated specifically for each patient. Gordonia spp. are environmental bacteria whose implication in human disease seems to be increasing. Phenotypic identification of bacteria included in this genus is difficult, and they are often poorly identified as Rhodococcus spp. or Corynebacterium spp. Molecular identification of Gordonia spp. by using 16S rRNA gene sequence comparison enables their characterization in human disease because the method is more accurate. The fact that these bacteria are often associated with medical devices highlights their role as nosocomial agents. Gram-positive bacilli must, therefore, not be systematically considered as contaminants, especially if associated with medical devices, and should be thoroughly identified by molecular methods in addition to biochemical tests.

The evidence of NK cell implication in human diseases has initially been shown for antiviral immunity and tumor surveillance. Nowadays, increased attention is being paid to the aspect of NK cells and innate immunity in studies on autoimmune diseases. However, despite a growing knowledge on NK cell function and regulation, their role in human autoimmune disease still remains controversial. In animal models, studies of NK cells have shown conflicting results toward a disease-promoting or disease-protective role. Similarly, in human diseases, available data suggest an unequivocal role of NK cells. Yet, the understanding of NK cell implication in human diseases is far from being achieved. We review here the current knowledge on NK cell biology in human autoimmune and inflammatory diseases and discuss their possible mechanisms of action in these complex pathologies.

DAP12 is an ITAM-bearing transmembrane adaptor originally identified on the surface of Natural Killer cells. A broad expression among other immune cells was later found in myeloid and lymphoid cells. However, data on DAP12 expression pattern rely only on immunoblot and microarray analysis. Here, we describe the generation and the characterization of an anti-DAP12 monoclonal antibody. Using this novel reagent, we show that DAP12 expression is restricted to innate immune cells in basal condition. Since a decreased expression of DAP12 has been suggested in NK cells of systemic lupus erythematosus patients, we have further investigated the NK cell receptor repertoire and leukocyte expression of DAP12 in these patients and no major changes were detectable when compared to controls.

International audience; Microparticles are small vesicles playing a crucial role in cell communication by promoting prothrombotic and proinflammatory responses. However, the molecular mechanisms underlying their release are still elusive. We previously established that thrombin promoted the generation of endothelial microparticles (EMPs). In the present study, gene profiling identified TRAIL/Apo2L, a cytokine belonging to the tumor necrosis factor-␣ superfamily, as a target of thrombin. Thrombin increased the expression of cell-associated and soluble forms of TRAIL (sTRAIL) in HMEC-1 cells and human umbilical vein endothelial cells (HUVECs). Blocking TRAIL by specific antibodies or by small interfering RNA reduced both the number and the procoagulant activity of EMPs released by thrombin. Consistent with an involvement of sTRAIL in thrombin-induced EMP release, we showed that (1) exogenously added sTRAIL generated procoagulant EMPs; (2) supernatants from thrombin-stimulated endothelial cells induced EMP release by HMEC-1 cells and HUVECs, whereas those recovered from TRAIL knockdown endothelial cells displayed no effect. TRAIL/ TRAIL-R2 complex mediated EMP release by initiating the recruitment of adaptor proteins and the activation of nuclear factor B. Moreover, sTRAIL modulated intercellular adhesion molecule-1 and interleukin-8 expression induced by thrombin by a downstream pathway involving nuclear factor B activation. Our data reveal a novel mechanism controlling EMP release and identify TRAIL as a key partner in the pathway linking coagulation and inflammation elicited by thrombin. (Circ Res. 2009;104:943-951.)

Objectives: The aim of this study was to assess whether non-specific clinical signs or biological results can identify patients with a high probability of infective endocarditis (IE) to improve outcome. Patients and methods: All patients tested for IE were included in a cohort and classified according to the modified Duke criteria. Patients with rejected endocarditis served as controls. Univariate and multivariate analyses were performed, and a score was calculated by adding 1 when a variable independently associated with IE (excluding major Duke criteria) was present and 0 when the variable was absent. A second score for patients with prior valvular damage (PVD) was also used. Scores were evaluated using the ROC curve method. Results: IE was diagnosed in 402 of 2039 participants (19.7%). By multivariate analysis, PVD, fever, emboli, stroke, splenomegaly, finger clubbing, leucocytosis and erythrocyte sediment rate >50 were independently associated with IE. The rate of IE increased significantly from 4% (10/254) for a score of 0 to 83% (10/12) for a score of 6 in all patients, and from 9.5% (23/241) to 100% (10/10) in patients with PVD. The area under the ROC curve was 0.75 for the first score and 0.7 for the second. In a prospective study of 117 patients with suspicion of IE, the proportion of confirmed IE was 19% and the area under the ROC curve was 0.72. Conclusions: This simple score can be used to identify patients with a high probability of IE, in the emergency room or on admission, to speed up diagnosis, or to initiate empirical antimicrobial therapy without replacing the modified Duke criteria.

Objective Expression of class I major histocompatibility complex (MHC) molecules on the surface of muscle cells is a biologic feature of idiopathic inflammatory myopathies (IIM). Class I MHC–transgenic mouse models support a causative role for class I MHC expression by muscle cells in the pathogenesis of IIM. The muscle lesions are characterized by leukocyte infiltration. We undertook this study to analyze the expression in muscle lesions of various class I MHC–specific receptors on leukocytes and natural killer (NK) cells. Methods We generated a panel of cell transfectants to control the immunofluorescence analysis of class I MHC receptor expression. We then analyzed the expression of CD158 (killer cell Ig-like receptors [KIRs]) and CD85j (leukocyte Ig-like receptor 1, Ig-like transcript 2) on muscle sections prepared from 14 patients with IIM (5 with dermatomyositis [DM], 5 with polymyositis [PM], and 4 with sporadic inclusion body myositis [IBM]). Results We could not detect the presence of NK cells in inflammatory lesions. However, the class I MHC receptor CD85j, but no KIRs, was expressed by inflammatory cells infiltrating muscle lesions in IIM. Conclusion CD85j is expressed in PM and sporadic IBM at the sites of partial invasion and in DM in perivascular inflammation, paving the way for dissecting the role of CD85j in the pathogenesis of inflammatory myopathies.

One out of 7 children has visual defects. Visual system is incriminated in 80% of children learning. The earlier the detection of visual defects is discovered the more successful the treatment can be. Our work aimed at defining what the reality about visual defects screening was in GP's consulting rooms. We received 87 answers out of 200 questionnaires sent to GP practicing in the Bouches-du-Rhône. This survey revealed a lack of information on screening for visual disorders in children among surveyed physicians, which had a clear impact on their practice; 84 of them were not aware of the Anaes guidelines concerning screening for visual disorders in children. At the close of this project, we developed a simple document (double-sided A4 paper) summarizing the required material as well as screening procedures that can be easily performed in general practice offices. A website offering the same information was designed as well. The next step will consist in assessing among general practitioners the benefits of these documents and their impact on routine practice.

Monthly high-dose intravenous administration of human polyclonal immunoglobulins (IVIG) has been shown to be effective as an adjuvant treatment for dermatomyositis. We report a patient with dermatomyositis treated with high doses of immunoglobulins by subcutaneous infusion (SCIG). SCIG was used because of the lack of peripheral and central vein access to continue effective IVIG therapy. The treatment was administered at home, was well tolerated, and was associated to the stabilization of the disease after a 1-year follow-up. Thus, our report suggests that SCIG could be an alternative to IVIG in the treatment of dermatomyositis.

Resume Introduction Le purpura thrombopenique auto-immun s'accompagne d'anticorps antiglycoproteines plaquettaires, en particulier le complexe GPIIb/IIIa. La persistance de ces autoanticorps a la phase de guerison peut parfois conduire a une thrombopathie auto-immune acquise que nous illustrons par la description d'un nouveau cas. Exegese Nous rapportons le cas d'une femme traitee par splenectomie pour un purpura thrombopenique auto-immun et developpant 5 ans apres un syndrome ecchymotique, alors que son taux plaquettaire etait normal. L'allongement du temps de saignement et un defaut d'agregabilite plaquettaire in vitro ont ete notes. Il existait depuis la thrombopenie initiale des anticorps antiglycoproteines plaquettaires GPIIb/IIIa associes a un anticoagulant circulant lupique et une gammapathie monoclonale d'allure benigne. La responsabilite des antiGPIIb/IIIa a ete confirmee par leur activite hypoagregante en presence de plaquettes temoins. Conclusion Cette thrombopathie acquise auto-immune simulant une maladie de Glanzmann est a rattacher a la persistance d'anticorps antiglycoproteines plaquettaires GPIIb/IIIa.

SummaryA multicenter study was set up to evaluate the prevalence, clinical and biological significance of antiphosphatidylethanolamine antibodies (aPE) in thrombotic patients with or without the main known clinical and biological risk factors for thrombosis. APE and antibodies, defined as the laboratory criteria of antiphospholipid syndrome (APS) -lupus anticoagulant, anticardiolipin and anti-beta2-GPI antibodies were measured in 270 patients with thrombosis (234 venous and 37 arterial) and 236 matched controls. APE were found in 15% of thrombotic patients compared to 3% of controls (p