Épidémiologie de la maladie de Horton en Haute-Savoie de 2007 à 2012

La maladie de Horton est la première vascularite en fréquence chez les patients de plus de 50 ans. Son incidence, étroitement influencée par un gradient Nord-Sud inversé, est proche en France de 9,4 par an pour 100 000 patients de plus de 50 ans. L objectif principal de notre étude était de calculer cette incidence dans notre département de la Haute-Savoie et d en évaluer l évolution sur 6 ans afin de confirmer ou non une impression clinique d épidémie . Il s agissait donc d une étude rétrospective observationnelle des nouveaux-cas diagnostiqués histologiquement. Nous avons mis en évidence 82 nouveaux cas et une incidence de la maladie en nette augmentation sur les six ans de l étude, celle-ci passant de 3.1 nouveaux cas par an(en 2007) à 8.1 (en 2011) et 8.3(en 2012) cas par an pour 100 000 habitants de plus de 50 ans. Notre nombre de biopsies annuelles a doublé durant la période, en moyenne 21 % de ces biopsies étaient positives, un pic diagnostic en Octobre (15 % des diagnostics) a été mis en évidence, nos patients diagnostiqués présentaient en général les symptômes classiques de la maladie. Nous retrouvons ainsi sur les deux dernières années, probablement davantage grâce à un meilleur diagnostic de la maladie de Horton en Haute-Savoie que par le biais d une véritable épidémie, une incidence proche de celle antérieurement décrite. Nos efforts doivent se poursuivre pour optimiser son diagnostic et permettre un traitement optimal des patients.GRENOBLE1-BU Médecine pharm. (385162101) / SudocSudocFranceF

Prise en charge des syndromes inflammatoires inexpliqués en médecine générale (enquête pratique auprès de 80 généralistes de Haute-Savoie)

Le syndrome inflammatoire inexpliqué (SII) est défini par la persistance d une CRP supérieure à 10 mg/l, pendant au moins 3 semaines, sans qu aucun point d appel ne se dégage après un interrogatoire et un examen clinique rigoureux, et après un bilan biologique et radiologique de 1ère intention. Il nous a paru important d étudier qualitativement la démarche diagnostique du médecin généraliste face à un SII; pour voir si cette démarche est conforme aux recommandations de la littérature récente, et voir comment le relai au spécialiste se passe. 80 médecins ont ainsi répondu à notre questionnaire en octobre 2011. Notre étude montre que peu confrontés à ce motif de consultation, ils se sentent parfois en difficulté dans cette démarche. 2 médecins sur 5 connaissent les 3 grands cadres étiologiques infections, maladies de système, cancers. La 1/2 des médecins évoquent la maladie de Horton chez les plus de 50 ans. Concernant le bilan étiologique, les médecins généralistes demandent bien la NFS, l EPS, le bilan hépatique, le ionogramme, mais omettent fréquemment l ECBU, et les hémocultures. La radiographie pulmonaire et l échographie abdominale sont presque toujours prescrites mais le scanner thoraco-abdomino-pelvien dans seulement 50% des cas. Le spécialiste est sollicité dans 100% des cas, mais après un délai souvent trop long. Il nous semble que la prise en charge de ces patients, et le réseau ville-hôpital pourraient être améliorés, en proposant un arbre décisionnel, accompagné du numéro d appel direct du spécialiste référent de l hôpital le plus proche. L élaboration de ce support pourrait faire l objet d un nouveau travail de thèse.The inflammation of unknown origin (IUO) is defined by the persistence of serum CRP levels greater than 10 mg / l, for at least 3 weeks, with no clinically identified cause after a thorough interview and clinical examination, and no guidance provided by biological and radiological tests. It seemed important to us to qualitatively study the diagnostic approach of a GP in front of IUO. We wanted to know if their approach follows the recommendations, as found in the bibliography; and how they passe the relay to the specialist. 80 GPs responded to our questionnaire in October 2011. Our study shows that GPs manage few consultations for this reason, and they sometimes feel difficulty in this situation. Two doctors in five spontaneously mention the three major etiological frameworks - infections, systemic diseases, cancers. Almost half of doctors suggest temporal arteritis in patients over 50 years. When the general practitioner researches the cause, he prescribes the laboratory tests advisable: full blood count, the serum protein elestrophoresis, the transaminases, and the creatinine, But he often omits to prescribe the urine culture, and omits systematically the blood cultures. Chest radiography and abdominal ultrasound are almost always prescribed. But chest abdominal and pelvic CAT-scan is done only in 50% of cases. The specialist advice is sought in 100% of cases, but often too late. It seems that the management of these patients, and town-hospital network, could be improved by offering to GPs a decision tree, with the direct call number of the referring internist or specialist from the nearest hospital. This tree could be the subject of a new thesis.GRENOBLE1-BU Médecine pharm. (385162101) / SudocSudocFranceF

Occurrence of focal myositis during Behçet's disease: The identification of a specific vasculitis‐associated focal myopathy

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Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich’s syndrome): frequency, clinical implication and prognosis

International audienceBackground: Episodic Angioedema with eosinophilia (EAE, Gleich's syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia and frequent elevated serum Immunoglobin M.Methods: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France.Results: Thirty patients were included with a median age at diagnosis of 41 years [5-84]. The median duration of each crisis was 5.5 days [1-90] with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%) among which 5 (17%) showed evidence of clonal TCR γ gene rearrangement. Median follow-up duration was 53 months [31-99]. The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio 4.15 [CI 95% 1.18-14.66; p=0.02). At last follow-up, 3 patients (10%) were able to withdraw all treatments and 11 (37%) were in clinical and biological remission with less than 10 mg of daily prednisone.Conclusion: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare

Evaluation of lupus anticoagulant, damage, and remission as predictors of pregnancy complications in lupus women: the French GR2 study

International audienceObjectives: The specific roles of remission status, lupus low disease activity state (LLDAS), and damage accrual on the prognosis of pregnancies in women with systemic lupus erythematosus (SLE) are unknown. We analysed their impact on maternal flares and adverse pregnancy outcomes (APOs).Methods: We evaluated all women (≥18 years) with SLE enrolled in the prospective GR2 study with an ongoing singleton pregnancy at 12 weeks (one pregnancy/woman). Several sets of criteria were used to define remission, disease activity, and damage. APOs included: fetal/neonatal death, placental insufficiency with preterm delivery, and small-for-gestational-age birth weight. First trimester maternal and disease features were tested as predictors of maternal flares and APOs.Results: The study included 238 women (98.3% on hydroxychloroquine) with 230 live births. Thirty-five (14.7%) patients had at least one flare during the second/third trimester. At least one APO occurred in 34 (14.3%) women.Hypocomplementemia in the first trimester was the only factor associated with maternal flares later in pregnancy (p = 0.02), while several factors were associated with APOs. In the logistic regression models, damage by SLICC-Damage Index (OR 1.8, 95% CI: 1.1-2.9 for model 1 and OR 1.7, 95% CI: 1.1-2.8 for model 2) and lupus anticoagulant (LAC, OR 4.2, 95% CI: 1.8-9.7 for model 1; OR 3.7, 95% CI: 1.6-8.7 for model 2) were significantly associated with APOs.Conclusion: LAC and damage at conception were predictors of APOs, and hypocomplementemia in the first trimester was associated with maternal flares later in pregnancy in a cohort of pregnant patients with well-controlled SLE.Clinical trial registration number: ClinicalTrials.gov, https://clinicaltrials.gov, NCT02450396

“Idiopathic Eosinophilic Vasculitis”: Another Side of Hypereosinophilic Syndrome? A Comprehensive Analysis of 117 Cases in Asthma-Free Patients

International audienceBackground: The absence of asthma may rule out a diagnosis of eosinophilic granulomatosis with polyangiitis in patients with hypereosinophilic syndrome (HES) and features of vasculitis.Objective: To describe eosinophilic vasculitis (EoV) as a possible manifestation of HES in asthma-free patients.Methods: We screened our hospital database and the literature for patients with HES who met the following 4 criteria: (1) histopathological or clinical features of EoV (biopsy-proven vasculitis with predominant eosinophilic infiltration of the vessel wall and/or features of vasculitis with tissue and/or blood hypereosinophilia [absolute eosinophil count >1.5 G/L]); (2) no other obvious causes of reactive eosinophilia, organ damage, and vasculitis; (3) the absence of antineutrophil cytoplasmic antibodies; and (4) the absence of current asthma.Results: Ten of our 83 (12%) asthma-free patients with HES and 107 additional cases in the literature met the criteria for EoV. After a critical analysis of the patients' clinical and laboratory characteristics and outcomes, we identified 41 cases of single-organ EoV (coronary arteritis, n = 29; temporal arteritis, n = 8; cerebral vasculitis, n = 4). Of the remaining 76 patients with EoV, the most frequent manifestations (>10%) were cutaneous vasculitis (56%), peripheral neuropathy (24%), thromboangiitis obliterans-like disease (16%), fever (13%), central nervous system involvement (13%), deep venous thrombosis (12%), and nonasthma lung manifestations (12%). Blood hypereosinophilia more than 1.5 G/L was observed in 79% of patients, and necrotizing vasculitis was observed in 44%.Conclusions: Our results suggest that idiopathic EoV (HES-associated vasculitis) can be classified as an eosinophilic-rich, necrotizing, systemic form of vasculitis that affects vessels of various sizes in asthma-free patients

The Lymphoid Variant of Hypereosinophilic Syndrome

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Characterisation of a high-risk profile for maternal thrombotic and severe haemorrhagic complications in pregnant women with antiphospholipid syndrome in France (GR2): a multicentre, prospective, observational study

International audienceBackgroundProspective data about the risks of thrombotic and severe haemorrhagic complications during pregnancy and post partum are unavailable for women with antiphospholipid syndrome. We aimed to assess thrombotic and haemorrhagic events in a prospective cohort of pregnant women with antiphospholipid syndrome.MethodsThis multicentre, prospective, observational study was done at 76 centres in France. To be eligible for this study, women had to have diagnosis of antiphospholipid syndrome; have conceived before April 17, 2020; have an ongoing pregnancy that had reached 12 weeks of gestation; and be included in the study before 18 weeks of gestation. Exclusion criteria were active systemic lupus erythematosus nephropathy, or a multifetal pregnancy. Severe haemorrhage was defined as the need for red blood cell transfusion or maternal intensive care unit admission because of bleeding or invasive procedures, defined as interventional radiology or surgery, to control bleeding. The GR2 study is registered with ClinicalTrials.gov, NCT02450396.FindingsBetween May 26, 2014, and April 17, 2020, 168 pregnancies in 27 centres met the inclusion criteria for the study. 89 (53%) of 168 women had a history of thrombosis. The median term at inclusion was 8 weeks gestation. 16 (10%) of 168 women (95%CI 5–15) had a thrombotic (six [4%] women; 95% CI 1–8) or severe haemorrhagic event (12 [7%] women; 95% CI 4–12). There were no deaths during the study. The main risk factors for thrombotic events were lupus anticoagulant positivity at inclusion (six [100%] of six women with thrombosis vs 78 [51%] of 152 of those with no thrombosis; p=0·030) and placental insufficiency (four [67%] of six women vs 28 [17%] of 162 women; p=0·013). The main risk factors for severe haemorrhagic events were pre-existing maternal hypertension (four [33%] of 12 women vs 11 [7%] of 156 women; p=0·014), lupus anticoagulant positivity at inclusion (12 [100%] of 12 women vs 72 [49%] of 146 women; p<0·0001) and during antiphospholipid history (12 [100%] of 12 women vs 104 [67%] of 156 women; p=0·019), triple antiphospholipid antibody positivity (eight [67%] of 12 women vs 36 [24%] of 147 women; p=0·0040), placental insufficiency (five [42%] of 12 women vs 27 [17%] of 156 women; p=0·038), and preterm delivery at 34 weeks or earlier (five [45%] of 11 women vs 12 [8%] of 145 women; p=0·0030).InterpretationDespite treatment adhering to international recommendations, a proportion of women with antiphospholipid syndrome developed a thrombotic or severe haemorrhagic complication related to pregnancy, most frequently in the post-partum period. Lupus anticoagulant and placental insufficiency were risk factors for these life-threatening complications. These complications are difficult to prevent, but knowledge of the antenatal characteristics associated with them should increase awareness and help physicians manage these high-risk pregnancies