Prise en charge de la maladie de Horton en ambulatoire

La maladie de Horton est une vascularité des artères de moyen et de gros calibre des sujets de plus de 50 ans, sensible à la corticothérapie, dont l'incidence et la prévalence sont en constante augmentation. Nous avons élaboré un questionnaire anonyme auprès de 409 médecins généralistes en Auvergne et Midi-Pyrénées afin d'explorer leurs habitudes de prise en charge. D'autre part, nous avons décrit 2 observations illustrant la grande variabilité de cette maladie et les difficultés thérapeutiques chez des patients souvent polypathologiques. Cette pathologie est bien connue par la majorité des médecins généralistes. Plusieurs signes cliniques dont l'amaurose transitoire, une baisse d'acuité visuelle, la cécité, la diplopie, les troubles oculomoteurs, la nécrose du cuir chevelu et de la langue, représentant toute la gravité de cette pathologie doivent être recherchés systématiquement afin de déterminer les modalités thérapeutiques et de suivi. La décroissance de la corticothérapie doit se faire progressivement, avec un suivi clinique et biologique rigoureux afin de surveiller la survenue d'une éventuelle rechute, ou l'apparition d'une corticodépendance. L'arrêt peut être discuté à partir de 5mg de prednisone en l'absence d'arguement pour une insuffisance surrénalienne. Les effets iatrogènes de la corticothérapie doivent être prévenus le plus rapidement possible afin de réduire la morbi-mortalité liée à la corticothérapie et ainsi réduire le nombre et la durée des hospitalisations. Les médecins généralistes sont en première ligne dans le dépistage de la maladie de Horton. Ils ont un rôle fondamental dans la gestion des complications du traitement.CLERMONT FD-BCIU-Santé (631132104) / SudocSudocFranceF

La grossesse au cours du lupus systémique (étude rétrospective de 27 cas)

Le lupus systémique est une pathologie fortement influencée par le milieu hormonal. La grossesse devient donc une période à risque au cours de la vie d'une patiente lupique. Notre étude rétrospective a porté sur 27 grossesses chez 20 femmes suivies pour un lupus systémique (SLE) au CHU de clermont-Ferrand. Un SLE a été diagnostiqué au cours d'une grossesse, lors de la découverte d'un bloc auriculo-ventriculaire (CHB). 14 femmes ont eu 1 grossesse, 3 deux grossesses, 1 trois grossesses, 1 a eu une grossesse gémellaire après le diagnostic de lupus systémique. 9 patientes avaient une corticothérapie, inférieure à 20 mg/jour dans tous les cas. 7 patientes étaient sous aspirine, 15 sous hydroxychloroquine, 1 sous azathioprine. 8 femmes étaient porteuses d'anticorps antiSSA/SSB. un anticoagulant circulant était observé chez 6 patientes, des anticorps anticardiolipines chez 11 femmes. un syndrome des antiphospholipides était associé au lupus chez l'une d'entre elles. Nous avons mis en évidence 9 poussées de la maladie pendant la grossesse (8 peu sévères et une grave) le plus souvent au cours du 3ème trimestre : 3 poussées rénales, 2 poussées cutanées, 1 poussée articulaire et 4 poussées uniquement biologiques, nécessitant une modification thérapeutique dans 5 cas. Le taux de poussée était plus important lorsque le lupus était actif à la conception et s'il existe un antécédent de néphrite lupique. Il n'y a eu aucune perte fœtale. Sont survenues, 1 HELLP syndrome, 2 CHB. 5 enfants sont nés prématurément, 8 étaient hypotrophes. Le taux de prématurité et d'hypotrophie était plus important chez les mères avec antécédent d'atteinte rénale. 4 enfants ont séjournés en réanimation (surveillance d'un CHB dans 2 cas, détresse respiratoire et prématurité dans les 2 autres cas). 2 enfants ont eu des infections néonatales. Il n'y a eu aucune malformation congénitale décrite lorsque l'hydroxychloroquine était poursuivie. Les 2 enfants atteints de cHB ont bénéficiés de l'implantation d'un pace maker, l'un à 5 mois, l'autre à 7 jours. Une cardiomyopathie s'est développée dans 1 cas. Aucun allongement du QTc ni allongement du PR n'ont été observés sur les électrocardiogrammes des enfants issus de mères antiSSA/SSB. La grossesse est de bon pronostic lorsqu'elle est planifiée au cours d'un lupus en rémission.CLERMONT FD-BCIU-Santé (631132104) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Adherence to treatment in systemic lupus erythematosus patients

International audienceAdherence is defined as "the extent to which a person's behaviour coincides with medical or health advice." Poor adherence to therapeutic regimens is a common and expensive problem in patients with chronic diseases including systemic lupus erythematosus (SLE) and is associated with a higher risk of flares, morbidity, hospitalisations and poor renal outcome. Non-adherence to the treatment is multifactorial for most patients and varies according to unintentional or intentional patterns. The rates of non-adherence in SLE patients range from 3% to 76% depending on the assessment methods, which are all subject to limitations. Indeed, poor adherence to therapeutic regimens is difficult to evaluate. Two studies have shown that undetectable blood hydroxychloroquine (HCQ) concentration may be a simple, objective and reliable marker of non-adherence in SLE patients. The accurate diagnosis of non-adherence may prevent one from incorrectly interpreting disease manifestations as a lack of response. It may then avoid an unnecessary or even dangerous treatment escalation

Pathologies maternelles chroniques et pertes de grossesse. Recommandations françaises

International audienceAim.-To review the available data on maternal chronic diseases and pregnancy losses.Material and Methods-We searched PubMed and the Cochrane library with pregnancy loss,stillbirth, intrauterine fetal demise, intrauterine fetal death, miscarriage and each maternaldiseases of this paper.Results-Antiphospholipid antibodies (anticardiolipin, anti-beta-2-glycoprotein, lupus anti-coagulant) should be measured in case of miscarriage after 10 WG confirmed by ultrasound(grade B) and an antiphospholipid syndrome should be treated by a combination of aspi-rin and low-molecular-weight heparin during a subsequent pregnancy (grade A). We donot recommend testing for genetic thrombophilia in case of first trimester miscarriage(grade B) or stillbirth (grade C). Glycemic control should be a goal before pregnancyfor women with pregestational diabetes to limit the risks of pregnancy loss (grade A)with a goal of prepregnancy HbA1c < 7%. Overt and subclinical hypothyroidisms should betreated by L-thyroxin during pregnancy to reduce the risks of pregnancy loss (grade A).Women who are positive for TPOAb should have TSH concentrations follow-up during pre-gnancy and subsequently treated by L-thyroxin if they develop subclinical hypothyroidism(grade B).Conclusions.-Prepregnancy management of most chronic maternal diseases, ideally throughprepregnancy multidisciplinary counseling, reduces the risks of pregnancy loss

Intrauterine fetal deaths related to antiphospholipid syndrome: a descriptive study of 65 women

International audienceObjectiveAlthough one of the three obstetric manifestations of antiphospholipid syndrome (APS) is intrauterine fetal death (IUFD), little is known about it in this context. We report the first large series of patients with APS and IUFD.MethodsWe retrospectively analyzed the history and clinical data of women at four French hospitals. All had (1) APS diagnosis (Sydney criteria) and (2) IUFD at or after 10 weeks of gestation (weeks) between 2000 and 2016.ResultsThe study included 65 women. Their median age at the index IUFD was 29 years (IQR 26–33); 38 (58%) were primigravidas. The index IUFD was the first APS clinical manifestation in 48 women (74%). Overall, 35% had a triple-positive antibody profile.IUFD occurred at a median gestational age of 24 weeks (IQR 18–27) and was associated with maternal obstetric complications in 16 women (25%), namely, preeclampsia (n = 12), hemolysis, elevated liver enzymes, and low platelet syndrome (HELLP) (n = 6), and/or placental abruption (n = 5). Half of the 50 women with available data had a small-for-gestational-age fetus.Overall, including during the follow-up period of 4 years (IQR 2–9), 28 women (43%) had at least one thrombosis, and 29% were diagnosed with systemic lupus erythematosus (SLE). Ultimately, 54 women (83%) had at least one live birth. Only one woman had three consecutive early miscarriages.ConclusionIUFD was most often the inaugural sign of APS. Of the APS classification criteria, IUFD, preeclampsia, and thromboses were common in this cohort, while the “3 consecutive early miscarriages” criterion was met only once. With treatment, most of the women successfully had at least one live birth

Intrauterine fetal deaths related to antiphospholipid syndrome: a descriptive study of 65 women

International audienceObjectiveAlthough one of the three obstetric manifestations of antiphospholipid syndrome (APS) is intrauterine fetal death (IUFD), little is known about it in this context. We report the first large series of patients with APS and IUFD.MethodsWe retrospectively analyzed the history and clinical data of women at four French hospitals. All had (1) APS diagnosis (Sydney criteria) and (2) IUFD at or after 10 weeks of gestation (weeks) between 2000 and 2016.ResultsThe study included 65 women. Their median age at the index IUFD was 29 years (IQR 26–33); 38 (58%) were primigravidas. The index IUFD was the first APS clinical manifestation in 48 women (74%). Overall, 35% had a triple-positive antibody profile.IUFD occurred at a median gestational age of 24 weeks (IQR 18–27) and was associated with maternal obstetric complications in 16 women (25%), namely, preeclampsia (n = 12), hemolysis, elevated liver enzymes, and low platelet syndrome (HELLP) (n = 6), and/or placental abruption (n = 5). Half of the 50 women with available data had a small-for-gestational-age fetus.Overall, including during the follow-up period of 4 years (IQR 2–9), 28 women (43%) had at least one thrombosis, and 29% were diagnosed with systemic lupus erythematosus (SLE). Ultimately, 54 women (83%) had at least one live birth. Only one woman had three consecutive early miscarriages.ConclusionIUFD was most often the inaugural sign of APS. Of the APS classification criteria, IUFD, preeclampsia, and thromboses were common in this cohort, while the “3 consecutive early miscarriages” criterion was met only once. With treatment, most of the women successfully had at least one live birth

Intrauterine fetal deaths related to antiphospholipid syndrome: a descriptive study of 65 women

International audienceObjectiveAlthough one of the three obstetric manifestations of antiphospholipid syndrome (APS) is intrauterine fetal death (IUFD), little is known about it in this context. We report the first large series of patients with APS and IUFD.MethodsWe retrospectively analyzed the history and clinical data of women at four French hospitals. All had (1) APS diagnosis (Sydney criteria) and (2) IUFD at or after 10 weeks of gestation (weeks) between 2000 and 2016.ResultsThe study included 65 women. Their median age at the index IUFD was 29 years (IQR 26–33); 38 (58%) were primigravidas. The index IUFD was the first APS clinical manifestation in 48 women (74%). Overall, 35% had a triple-positive antibody profile.IUFD occurred at a median gestational age of 24 weeks (IQR 18–27) and was associated with maternal obstetric complications in 16 women (25%), namely, preeclampsia (n = 12), hemolysis, elevated liver enzymes, and low platelet syndrome (HELLP) (n = 6), and/or placental abruption (n = 5). Half of the 50 women with available data had a small-for-gestational-age fetus.Overall, including during the follow-up period of 4 years (IQR 2–9), 28 women (43%) had at least one thrombosis, and 29% were diagnosed with systemic lupus erythematosus (SLE). Ultimately, 54 women (83%) had at least one live birth. Only one woman had three consecutive early miscarriages.ConclusionIUFD was most often the inaugural sign of APS. Of the APS classification criteria, IUFD, preeclampsia, and thromboses were common in this cohort, while the “3 consecutive early miscarriages” criterion was met only once. With treatment, most of the women successfully had at least one live birth

Pregnancy and neonatal outcomes in women with axial spondyloarthritis: pooled data analysis from the European Network of Pregnancy Registries in Rheumatology (EuNeP).

OBJECTIVE To investigate outcome and course of pregnancies in women with axial spondyloarthritis (axSpA) in a pooled data analysis of pregnancy registries in rheumatology. METHODS Prospectively followed women with axSpA, fulfilling ASAS classification criteria and for whom a pregnancy outcome was reported, were eligible for the analysis. Anonymised data of four registries was pooled. Rates of adverse pregnancy outcomes were calculated. Systemic inflammation, disease activity and treatment patterns with tumour necrosis factor inhibitor (TNFi) before, during and after pregnancy were analysed. RESULTS In a total of 332 pregnancies from 304 axSpA women, 98.8% of the pregnancies resulted in live birth. Mean maternal age was 31 years and disease duration 5 years. Most of these patients received pre-conception counselling (78.4%). Before pregnancy, 53% received TNFi treatment, 27.5% in first and 21.4% in third trimester. Pregnancy and neonatal outcomes were favourable with rates of 2.2% for pre-eclampsia, 4.9% for preterm birth, 3.1% for low birth weight and 9.5% for small for gestational age. Neonates were delivered by caesarean section in 27.7% of pregnancies, of which 47.4% were emergencies. Pooled mean CRP was 4 mg/L before conception peaking in the second trimester at 9.4 mg/L. Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) was below 4 at all time-points. CONCLUSIONS Pooled rates of most outcomes were better than what had been reported in the literature and within expected rates of those reported for the general population. Pre-conception counselling, planned pregnancies and a tight management in expert centres applying a tailored treatment approach may have contributed to the favourable pregnancy outcomes

Health Outcome of 215 Mothers of Children with Autoimmune Congenital Heart Block: Analysis of the French Neonatal Lupus Syndrome Registry.

International audienceObjective Transplacental passage of maternal anti-SSA and anti-SSB antibodies, potentially associated with maternal autoimmune diseases, can cause neonatal lupus syndrome. Given the paucity of data in this setting, we report short- and long-term outcomes of mothers of offspring with congenital heart block (CHB).Methods This retrospective study included anti-SSA/SSB antibody–positive mothers of fetuses with high-degree CHB and focused on their health status before pregnancy, at CHB diagnosis, and thereafter.Results We analyzed 215 women with at least 1 pregnancy with CHB. Prior to this diagnosis, only 52 (24%) mothers had been diagnosed with an autoimmune disease, mainly systemic lupus erythematosus (SLE; n = 26, 12%) and Sjögren syndrome (SS; n = 16, 7%). Six more were diagnosed with an autoimmune disease during the index pregnancy. Of the 157 mothers (73%) with no such diagnosis at childbirth, 77 (49%) developed one after a median follow-up of 11 years (range: 21 days to 54 years). By the end of follow-up, 135 women (63%) had an autoimmune disease diagnosis, mainly SLE (n = 54, 25%) and SS (n = 72, 33%). Three patients with SLE had renal involvement, and only 6 (3%) had required an immunosuppressive drug at any point. The symptoms best predicting autoimmune disease development were arthralgia and myalgia (P &lt; 0.001), dry syndrome (P = 0.01), and parotid swelling (P = 0.05).Conclusion One-quarter of the patients had an autoimmune disease diagnosis at the time of the fetal CHB diagnosis. Nearly half of those without an initial diagnosis progressed during follow-up, most without severe manifestations. Severe diseases such as lupus nephritis were rarely seen, and immunosuppressive drugs were rarely required

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