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Cardiac involvement in systemic lupus (SL) and antiphospholipid syndrome (APS) can be due to variables and involve different presentations. Pericarditis is the most common lupus manifestation and occurs in 16% to 25% of patients. While corticosteroids are usually very effective, colchicine may avoid steroids and prevent relapse. Myocarditis during SL is rare and often inaugural. They may manifest as chest pain, acute heart failure, arrhythmias or conduction disturbances, and may progress to dilated cardiomyopathy and/or permanent heart failure. Their prognosis is however generally good, even in the absence of treatment with cyclophosphamide for the less serious forms. Finally, coronary involvement in SL is most often due to atherosclerotic, thrombotic origin (generally in the context of associated APS), and exceptionally explained by coronary vasculitis. During APS, valve disease is frequent and usually asymptomatic. Thrombotic damage can be (1) coronary, typically manifesting as a myocardial infarction in a young subject with healthy coronary arteries, (2) much more rarely intracardiac, or (3) microcirculatory, generally as part of a catastrophic antiphospholipid syndrome (CAPS) leading to a multiorgan failure. Finally, iatrogenic cardiac manifestations can exceptionally be seen during treatment with cyclophosphamide or antimalarials characterized by conduction disorders and/or heart failure.

During the past 50 years, the mortality due to childhood cancers decreased dramatically thanks to improvements in supportive care and the use of multimodal approaches. In this context, the long-term follow up after childhood cancer has become a main concern for pediatric oncologists. The SALTO programme was developed in 2012 at the CHR Citadelle in Liège in order to organize a multidisciplinary long-term follow-up for the patients previously treated in our department for a childhood cancer. The aim of the present study was to review, for the most frequent childhood cancers, the long-term sequellae and the second cancers developed by the patients participating to the SALTO programme in order to define the follow-up needed. Our data confirm the importance of a multidisciplinary long-term follow-up, based on the treatments received and following international guidelines.Au cours des cinquante dernières années, la mortalité liée aux cancers pédiatriques a fortement diminué grâce à une amélioration des soins de support et à l’utilisation d’approches multimodales. Dans ce contexte, le devenir à long terme des patients guéris d’un cancer pédiatrique est devenu une des préoccupations majeures pour les oncologues pédiatres. Dans cette optique, la consultation SALTO («Suivi À Long Terme en Oncologie») a été mise en place en 2012 au CHR de la Citadelle pour assurer le suivi multidisciplinaire des adultes ayant été traités dans notre secteur d’hémato-oncologie pédiatrique. L’objectif de cette étude a été de revoir, pour les cancers pédiatriques les plus fréquents, les séquelles et les tumeurs secondaires présentées par les patients suivis en consultation SALTO afin de préciser les modalités du suivi au long cours après cancer pédiatrique. Nos résultats confirment l’importance d’un suivi multidisciplinaire à long terme adapté aux traitements reçus, sur base de recommandations internationales.

Cancers are rare pathologies in children. Improvement in survival rates has been obtained thanks to new therapeutic strategies based on the identification of risk factors. Targeted therapies in paediatric oncology are new treatments providing hope that cure is achievable without long-term sequelae.Les cancers pédiatriques sont des pathologies rares. L’amélioration du taux de survie a été obtenue par de nouvelles stratégies de traitement basées sur l’identification de facteurs de risque. Les thérapies ciblées en oncologie pédiatrique, nouvelle arme thérapeutique, sont porteuses d’espoir de guérison, sans séquelles à long terme.

The article describes a clinical case of catatonic syndrome. We describe the manifestations of the syndrome, its diagnostic criteria and associated scales. The modalities of the challenge test, constituting a diagnostic test, and first line treatment are detailed. Clinical and paraclinical investigations are proposed to determine the etiology. The benzodiazepine withdrawal as an etiology of catatonic syndrome is detailed.L’article expose un cas clinique de syndrome catatonique. Nous y décrivons les manifestations du syndrome, ses critères diagnostiques et échelles associées. Les modalités du «challenge test», constituant une épreuve diagnostique, et le traitement de première ligne sont détaillés. Des investigations cliniques et paracliniques sont proposées afin d’en déterminer l’étiologie. Le sevrage en benzodiazépines comme étiologie du syndrome catatonique est discuté.

Cancer is the second leading cause of death among children aged 5 to 14, after accidents. We conducted a study on the epidemiology of childhood cancer in the university pediatric oncology department of the CHU-CHR in Liège, Belgium. We studied a cohort of 662 patients between the ages of 0 and 17 whose malignancy diagnosis was made between 1985 and 2016. The analyzes were performed retrospectively using medical files. The number of new cases, the proportion of different cancers, sex ratio, age at diagnosis and survival at 5 and 10 years were the epidemiological factors studied.We have been able to show an increase in the number of new diagnoses per year. More than 40 % of childhood cancers occur before the age of five. The most common neoplasias are leukemias, tumors of the central nervous system and lymphomas. This distribution is influenced by age. All malignant tumours combined, we observed a slightly larger proportion of affected boys than girls. Overall survival at 5 years reaches 80.2 %. However, it varies according to the type of tumour from 59.3 % for malignant soft tissue tumors up to 100 % for hepatoblastomas.Le cancer est la deuxième cause de décès chez les enfants de 5 à 14 ans, après les accidents. Nous avons réalisé une étude sur l’épidémiologie des cancers de l’enfant au sein du service universitaire d’oncologie pédiatrique du CHU-CHR de Liège. Nous avons étudié une cohorte de 662 patients, âgés de 0 à 17 ans, dont le diagnostic de tumeur maligne a été posé entre 1985 et 2016. Le nombre de nouveaux cas, la proportion des différents cancers, le sex ratio, l’âge au diagnostic et la survie à 5 et 10 ans ont été les facteurs épidémiologiques étudiés. Nous avons pu démontrer une augmentation du nombre de nouveaux diagnostics par an. Plus de 40 % des cancers de l’enfant surviennent avant l’âge de 5 ans. Les néoplasies les plus fréquentes sont les leucémies, les tumeurs du système nerveux central et les lymphomes. Cette répartition est néanmoins influencée par l’âge. Toutes tumeurs malignes confondues, nous avons observé une proportion légèrement plus grande de garçons atteints que de filles. La survie globale à 5 ans s’élève à 80,2 %. Elle varie cependant selon le type de tumeur de 59,3 % pour les tumeurs malignes des tissus mous jusqu’à 100 % pour les hépatoblastomes.

Objectives Pericardial involvement is a frequent manifestation of systemic lupus erythematosus (SLE). Growing evidence suggests that colchicine may be useful for acute or recurrent pericarditis. We report for the first time a series of 10 consecutive cases of SLE with pericarditis treated with colchicine. Methods Inclusion criteria in this retrospective study were diagnosis of SLE, pericarditis and receiving colchicine. Results We included 10 consecutive cases of SLE with pericarditis treated with colchicine (nine women, mean age at the index pericarditis 35 ± 12 years). Pericarditis was the initial manifestation of SLE for two patients, whereas eight patients had SLE lasting for a median of 2.5 years (15 days to 13 years) and had received prednisone ( n = 7, 2–30 mg/d), hydroxychloroquine ( n = 7), azathioprine ( n = 3), methotrexate ( n = 2), and mycophenolate mofetil ( n = 1). For six patients, pericarditis was associated with other SLE manifestations. Altogether, colchicine avoided the use ( n = 2) or increase in dosage ( n = 5) of steroids in seven cases; the increase in steroids dosage was minimal for two patients. Colchicine 1 mg was given for a median of 39 days (10 days to 54 months). Symptoms completely resolved after a median of 2.5 days (1–30 days) after initiation of colchicine. Colchicine was maintained or resumed in six patients to prevent recurrence, with no further relapse. Conclusions Colchicine may be safe and effective in treating SLE pericarditis and used as a steroids-sparing agent. These preliminary results need to be confirmed in a larger study with longer follow-up.

The Acquired ImmunoDeficiency Syndrome (AIDS) is a chronic infectious disease. By its complexity and the importance of psychiatric syndromes observed among HIV patients, the common contribution of somatic and psychiatric health specialists is often mandatory. Conversely, more or less 20 % of psychiatric patients are infected by the AIDS' virus. Moreover, these troubles would be their second reason of hospitalization. These psychiatric symptoms, so caused by HIV, may occur before its serological discovery. Thereby, it will be interesting for the psychiatrist to acquire enough knowledge about this disease to establish preventing actions and strategies of psychoeducation.Le Syndrome de l’ImmunoDéficience Acquise (SIDA) est une pathologie infectieuse chronique. Par sa complexité et l’importance des syndromes psychiatriques observés chez les patients séropositifs, l’intervention conjointe des professionnels de la santé psychique et somatique peut s’avérer souvent nécessaire. Jusqu’à 20 % des patients psychiatriques seraient également infectés par le virus du SIDA. De plus, ces troubles seraient leur 2ème cause d’hospitalisation. Cette symptomatologie psychiatrique, alors induite par le VIH, pourrait même en précéder la découverte sérologique. Ainsi, il serait intéressant pour le psychiatre d’acquérir suffisamment de connaissances sur cette affection afin d’établir des actions de prévention et des stratégies de psychoéducation.

Relapsing polychondritis (RP) is a rare condition characterized by recurrent inflammation of cartilaginous tissue and systemic manifestations. Data on pathophysiology are scarce and suggest an autoimmune mechanism. Recently, the possibility of dividing patients with RP into three distinct clinical phenotypes has been suggested: the hematological form representing less than 10% of patients, essentially older men with associated myelodysplasia and poor prognosis, the respiratory form representing about 25% of patients with predominant tracheobronchial involvement, and the mild and most frequent form, representing 65% of patients, with a good prognosis. Recent data on survival shows an improvement of overall prognosis compared to historical series. Reported poor prognosis factors are male gender, associated haemopathies and cardiac involvement. Few recent series suggest an interest for positron emission tomography for the diagnosis and the follow-up of treatment. Due to the lack of randomized therapeutic trial, treatment remains empirical and is mainly based on oral corticosteroids sometimes associated with immunosuppressive agents. The use of biologic agents has recently been reported in small retrospective series with different outcome. Finally, some selected patients with mild and occasional peripheral chondritis might justify a treatment with colchicine or a therapeutic abstention with occasional short-term corticosteroids therapy.

Conversion disorder is a psychiatric disorder often encountered in neurology services. This condition without organic lesions was and still is sometimes referred as an imaginary illness or feigning. However, the absence of organic lesions does not exclude the possibility of cerebral dysfunction. The etiologic mechanisms underlying this disorder remain uncertain even today.The advent of cognitive and functional imaging opens up a field of exploration for psychiatry in understanding the neurobiological mechanisms underlying mental disorders and especially the conversion disorder. This article reports several neuroimaging studies of conversion disorder and attempts to generate hypotheses about neurobiological mechanisms.Le trouble de conversion est une pathologie psychiatrique fréquemment rencontrée dans les services de neurologie. Cette pathologie «sans substrat» a été et est encore parfois qualifiée de maladie imaginaire ou de simulation. Cependant, l’absence de substrat «organique» n’exclut pas la possibilité d’un dysfonctionnement cérébral. Les mécanismes étiopathogéniques qui sous-tendent ce trouble sont longtemps restés incertains, encore aujourd’hui. L’avènement des sciences cognitives et de l’imagerie fonctionnelle ouvre un champ d’exploration pour la psychiatrie dans la compréhension des mécanismes neurobiologiques qui sous-tendent les troubles mentaux et, en particulier, le trouble de conversion. Cet article reprend plusieurs études de neuroimagerie sur le trouble de conversion et tente d’en dégager des hypothèses sur ses mécanismes neurobiologiques.

Anorexia nervosa is a complex pathology that regularly leads clinicians to a therapeutic impasse. The origin of this disorder is multifactorial. It occurs most often in adolescence and affects mainly women. Paradoxically, hunger is present but dissociated from adequate food function. Somatic and psychological impact can be particularly heavy. The treatment is difficult and requires a multidisciplinary care. An eclectic and integrative approach, moving away from rigid therapeutic methods and integrating the plurality of techniques, seems to be particularly recommended to increase the chances of therapeutic success. This article summarizes the various theoretical models of anorexia nervosa and its modalities of treatment.L’anorexie mentale est une pathologie complexe qui entraîne régulièrement les cliniciens dans une impasse thérapeutique. L’origine de ce trouble est plurifactorielle. Il survient le plus souvent à l’adolescence et touche principalement les jeunes femmes. Paradoxalement, la sensation de faim est présente, mais elle n’est plus au service d’une fonction nutritive adéquate. Les répercussions somatiques et psychiques peuvent être particulièrement lourdes. Le traitement de l’anorexie mentale reste difficile et nécessite une prise en charge pluridisciplinaire. Une approche éclectique et intégrative, s’écartant de méthodes thérapeutiques rigides et intégrant la pluralité des techniques, semble être particulièrement recommandée pour accroître les chances de succès thérapeutique. Cet article résume les différents modèles théoriques de l’anorexie mentale et ses modalités de prise en charge.

Studies in pediatric oncology describe a relative good quality of life in child cancer survivor. However, few studies were interested in the parents of a child cancer survivor. 61 parents are recruited in the Belgian hospitals. Three groups of parents are constituted : the parents whose child is in 4 years of survivorship (group 1), in 5 years of survivorship (group 2) and in 6 years of survivorship (group 3). Clinical scales and a Stroop emotion task are administered. Parents (of the 3 groups) present a low tolerance of uncertainty, have excessive worries about the evolution of the health of their child, and suffer from anxious symptoms. The Stroop emotion tasks reveals a cognitive bias of the attention in favour of threatening stimuli. The study highlights the importance to detect parents who are intolerant of uncertainty at the cancer diagnosis stage and their continuous psychological follow-up once the treatments are ended.Les études en oncologie pédiatrique décrivent une relativement bonne qualité de vie chez les enfants survivants de cancer. À ce jour, peu d’études se sont intéressées aux parents d’un enfant survivant de cancer. Soixante-et-un parents sont recrutés dans les hôpitaux belges. Trois groupes de parents sont constitués : les parents dont l’enfant est à 4 ans de rémission (groupe 1), à 5 ans de rémission (groupe 2) et à 6 ans de rémission (groupe 3). Des échelles cliniques et une tâche de Stroop émotion sont administrées. Les parents (des 3 groupes) présentent une faible tolérance à l’incertitude, ont des inquiétudes excessives quant à l’évolution de la santé de leur enfant et souffrent de symptômes anxieux. Le Stroop émotion révèle un biais cognitif de l’attention en faveur des stimuli de nature menaçante. L’étude met en exergue l’importance de détecter les parents intolérants à l’incertitude lors du diagnostic d’annonce du cancer et leur suivi psychologique continu une fois les traitements terminés.

Objective To study the outcome of patients with antiphospholipid syndrome (APS) after oral anticoagulant treatment cessation. Methods We performed a retrospective study of patients with APS experiencing cessation of oral anticoagulant and enrolled in a French multicentre observational cohort between January 2014 and January 2016. The main outcome was the occurrence of recurrent thrombotic event after oral anticoagulation cessation. Results Forty four APS patients interrupted oral anticoagulation. The median age was 43 (27–56) years. The median duration of anticoagulation was 21 (9–118) months. Main causes of oral anticoagulant treatment cessation were switch from vitamin K antagonists to aspirin in 15 patients, prolonged disappearance of antiphospholipid antibodies in ten, bleeding complications in nine and a poor therapeutic adherence in six. Eleven (25%) patients developed a recurrent thrombotic event after oral anticoagulation cessation, including three catastrophic APS and one death due to lower limb ischemia. Antihypertensive treatment required at time of oral anticoagulants cessation seems to be an important factor associated with recurrent thrombosis after oral anticoagulant cessation (15.2% in patients with no relapse versus 45.5% in patients with recurrent thrombosis, p = 0.038). Oral anticoagulant treatment was re-started in 18 (40.9%) patients. Conclusion The risk of a new thrombotic event in APS patients who stopped their anticoagulation is high, even in those who showed a long lasting disappearance of antiphospholipid antibodies. Except for the presence of treated hypertension, this study did not find a particular clinical or biological phenotype for APS patients who relapsed after anticoagulation cessation. Any stopping of anticoagulant in such patients should be done with caution.

Resume Introduction Le cancer du col de l’uterus touche annuellement 150 femmes en Bretagne en l’absence de depistage organise. Materiel et methodes Etude retrospective concernant 191 patientes traitees pour un cancer invasif du col uterin entre 2000 et 2005 centree sur leur passe cytologique. L’âge moyen des patientes etait de 52 ans (22–87 ans). Le niveau socioeconomique des patientes a ete releve. La repartition des sous-types histologiques etait : 73 % de type epidermoide (âge moyen de 54 ans) et 22 % d’adenocarcinomes (âge moyen 47 ans). Tous les stades etaient representes : stade I 46 %, II 32 %, III 9 % au stade IV (13 %). Resultats Le cancer etait symptomatique dans 89 % des cas et 72 % des patientes n’avaient pas beneficie de depistage cytologique selon les recommandations francaises (50 % aucun suivi, 22 % suivi entre trois et dix ans), tandis que 28 % des patientes avaient eu un frottis dans les trois ans. Le niveau socioeconomique des patientes influait fortement sur la participation au depistage. La proportion de patientes ayant un adenocarcinome invasif du col etait de 31 % chez les patientes avec un frottis datant de moins de trois ans (contre 22 % dans notre population globale) et ce sous-type histologique etait surrepresente chez les patientes de moins de 35 ans (35 %). Enfin, 2,6 % des patientes ont ete perdues de vue apres realisation d’un frottis pathologique. Conclusion L’extension du depistage et son organisation restent une priorite dans notre region. La sensibilite moyenne du frottis est illustree par la surrepresentation des adenocarcinomes, en particulier chez les femmes jeunes.

Resume L’arteriopathie obliterante des membres inferieurs est une pathologie frequente, liee le plus souvent aux facteurs de risque cardiovasculaires classiques comme le tabagisme, le diabete, les dyslipidemies, l’hypertension arterielle. Malgre l’existence de ces facteurs de risque, diverses thrombophilies (deficit en inhibiteurs physiologiques de la coagulation, facteur V Leiden et variant 20210A du gene de la prothrombine, anticorps antiphospholipides, hyperhomocysteinemie moderee comprise entre 15 et 30 μmol/l) peuvent etre evoquees devant certaines formes cliniques d’arteriopathie des membres inferieurs. L’article fait la synthese des etudes realisees a ce sujet. Ces thrombophilies sont a evoquer en cas de maladie thromboembolique veineuse associee, en cas de signes evocateurs de syndrome des antiphospholipides mais aussi en cas de debut precoce de l’arteriopathie, de maladie evoluee (ischemie chronique), de maladie evolutive et en cas d’echecs de revascularisation en l’absence d’explication technique. Toutefois, en dehors du syndrome des antiphospholipides, il n’y a pas a l’heure actuelle de consensus pour la recherche de thrombophilie et le traitement en cas d’arteriopathie des membres inferieurs.

Adrenal necrosis, a rare life threatening complication of antiphospholipid syndrome, is difficult to diagnose during pregnancy. We report the case of a 33-year-old woman with bilateral adrenal necrosis which started during the third trimester of her second pregnancy. Antiphospholipid syndrome had been diagnosed few years ago, after a thrombotic event. The pregnancy was uneventful until 36 weeks plus five days, when the patient was admitted for bilateral back ache, initially considered as uterine contractions. Labour was induced because pain persisted and was associated with major thrombocytopenia. A healthy infant was delivered vaginally on the second day, adrenal failure was diagnosed based on intense asthenia, persistent severe lumbar pain, low blood sodium and cortisol. Bilateral adrenal oedema was documented by CT scan and MRI. Symptoms resolved following administration of hydrocortisone and fludrocortisone. This case illustrates the difficulty to diagnose adrenal necrosis in the third trimester of pregnancy.

Resume La detection des autoanticorps et la determination de leurs cibles antigeniques sont utiles au diagnostic et a la classification des maladies auto-immunes. Objectif Le but de cette etude etait de comparer les resultats obtenus en utilisant la technologie Luminex™ pour la detection semi-quantitative des anticorps antiantigenes nucleaires solubles (ENA), anticytoplasme des polynucleaires neutrophiles (ANCA) et antithyroidiens avec ceux obtenus avec les techniques conventionnelles. Methodes Les reactifs QUANTA Plex™ Inova (Menarini, France) ont ete utilises pour analyser 815 serums adresses au laboratoire pour bilan d'auto-immunite, notamment pour la detection d'anticorps anti-ENA (n = 400), ANCA (n = 161) et antithyroidiens (n = 254). Les methodes conventionnelles comprenaient des techniques d'immunofluorescence et immunoenzymatiques (Elisa et immunodot). Resultats Pour les anti-ENA, la concordance globale etait de 95,6 %. Pour les ANCA, la concordance etait de 78,5 % (anti-PR3) et 91,4 % (anti-MPO). Pour les antithyroidiens, la concordance etait de 90 % (anti-TPO) et 88 % (anti-TG). Conclusion L'utilisation des reactifs QUANTA Plex™ est simple et permet une detection semi-quantitative simultanee, et rapide des principaux autoanticorps associes aux maladies auto-immunes. Ces tests sensibles et specifiques tendent a se developper pour la detection, et le suivi des autoanticorps dans le domaine de l'auto-immunite.

Rectal prolapse is rare in children and usually benign. However, there are various diseases that can be associated with it, such as cystic fibrosis or other causes of increased abdominal pressure. Here, we review the various underlying conditions that pediatricians or pediatric gastroenterologists should consider in the case of rectal prolapse. We report on three cases of children with a rectal prolapse and intra-abdominal tumors. Current recommendations and practice do not include a systematic check via abdominal imaging in cases of rectal prolapse. However, in some situations, imaging is indicated to detect a possible expansive process. Thus, in the presence of recurrent prolapse or of associated urinary or neurological signs, imaging is justified so as to allow for an early diagnosis and treatment of these neoplasms. Given its lack of radiation exposure and good sensitivity in children, ultrasound imaging is the first choice.

To assess the relationship between Takayasu arteritis (TAK) and pregnancy outcome.This study included 240 pregnancies in 96 patients fulfilling the American College of Rheumatology 1990 criteria for the classification of TAK and/or the 1994 Chapel Hill Consensus Conference nomenclature/criteria for vasculitis. We analyzed obstetric and maternal outcomes in women who were pregnant before and/or at the same time as or after TAK diagnosis. We assessed factors associated with complicated pregnancy.One hundred forty-two pregnancies occurred in 52 patients before TAK diagnosis (median age at pregnancy 26 years [interquartile range 23-30 years]), and 98 pregnancies occurred in 52 patients concomitant with or after TAK diagnosis (median age at pregnancy 28 years [interquartile range 26-31 years]). Pregnancies concomitant with or after TAK diagnosis had a 13-fold higher rate of obstetric complications compared to pregnancies before TAK diagnosis (odds ratio 13 [95% confidence interval 5-33], P0.0001). TAK was associated with a 40% frequency of obstetric complications, including preeclampsia/eclampsia (24 pregnancies [24%]), premature delivery (8 pregnancies [8%]), and intrauterine fetal growth restriction or death (5 pregnancies [5%]). Maternal complications of TAK occurred during 39% of pregnancies and included mainly new-onset or worsening hypertension (26 pregnancies [27%]). In multivariate analysis, smoking (odds ratio 6.15 [95% confidence interval 1.31-28.8]) and disease activity of TAK (a National Institutes of Health score of1) (odds ratio 28.7 [95% confidence interval 7.89-104.7]) were independently associated with obstetric and maternal complications.TAK negatively affects pregnancy outcomes. Disease activity increases the risk of obstetric and maternal complications, mainly due to arterial hypertension.

International audience; Blood concentrations of hydroxychloroquine (HCQ) vary widely among patients with systemic lupus erythematosus (SLE). A pharmacokinetic/pharmacodynamic relationship has been found in different situations, and a very low blood concentration of HCQ is a simple marker of nonadherence to treatment. Therefore, interest in blood HCQ concentration measurement has increased, but little is known about factors that influence blood HCQ concentration variability. This study was undertaken to analyze determinants of blood HCQ concentrations. We conducted a retrospective analysis of patient data, including data from the Plaquenil Lupus Systemic (PLUS) study, to determine the association of epidemiologic, clinical, and biologic factors with blood HCQ concentrations. Data for nonadherent patients (blood HCQ concentration

Recent advances in the neurobiology of depression have underlined the importance of markers of inflammation, neurotrophins, and hypothalamo-pituitary adrenal (HPA) axis dysfunction in the development of this pathology. These disorders could have some impact on other systems such as the glucose metabolism regulation with an increased risk of insulin resistance and type 2 diabetes. Type 2 diabetes is also frequently associated with a pro-inflammatory state that could favour the development of a depressive episode. Inflammatory phenomena and HPA axis dysfunction could be biological links between depression and type 2 diabetes and account not only for the frequent association between those two disorders, but also for the treatment-resistance to classical antidepressants.

Resume Les fœtus ou les enfants des femmes porteuses d’un anticorps anti-SSA/Ro et/ou anti-SSB/La peuvent developper un « lupus erythemateux neonatal » comportant une atteinte cutanee, hematologique, hepatique et/ou un bloc auriculo-ventriculaire congenital (BAVc) survenant sur un cœur indemne de cardiopathie malformative majeure. La frequence du BAVc chez les femmes ayant une connectivite associee a un anticorps anti-SSA/Ro est estimee a 1 a 2 % et le risque de recurrence est de 10 a 17 %. Le BAVc complet est definitif et est associe a une morbidite (necessite d’implanter un pace maker dans 2/3 des cas) et une mortalite (16 a 19 %) faisant toute la gravite de ce syndrome. Une atteinte myocardique peut etre associee d’emblee ou apparaitre tardivement. Les autres atteintes sont egalement discutees. Lorsqu’une femme enceinte est porteuse d’un anticorps anti-SSA/Ro, la surveillance echographique obstetricale doit etre realisee tous les 15 jours entre 16 et 24 SA, voire toutes les semaines si elle a un antecedent de BAVc lors d’une grossesse precedente. Un electrocardiogramme est pratique de principe chez tous les enfants en periode neo-natale pour depister des BAVc incomplets. Le traitement curatif in utero du BAVc fait appel aux corticoides fluores, en privilegiant la betamethasone. Leur efficacite est inconstante.

Resume Au cours de la derniere decennie, le developpement de biotherapies est venu enrichir l’arsenal therapeutique utilise au cours des maladies auto-immunes systemiques (MAIS). Ces immuno-interventions peuvent etre dirigees contre tous les « acteurs » de la reponse immunitaire et comprennent schematiquement les inhibiteurs du lymphocyte B (LB). Du fait du role majeur des LB dans la pathogenie des maladies auto-immunes, son inactivation par des inhibiteurs specifiques a ete envisagee. Trois voies sont a l’etude : depletion des LB par anticorps anti-CD20, inhibition du systeme BLyS et tolerogenes specifiques du LB ;les inhibiteurs de la co-stimulation. Les cellules presentatrices de l’antigene expriment differentes molecules de co-stimulation qui interagissent avec des molecules a la surface des lymphocytes T (LT). Ces co-stimuli sont necessaires a l’activation des LT et constituent de ce fait des cibles privilegiees. Plusieurs therapeutiques sont en cours d’etude : anti-CD40 ligand ou CTLA-4 Ig (abatecept) ;les inhibiteurs des LT qui permettent de bloquer les lymphocytes autoreactifs, cellules cles dans l’emergence des MAIS ;les immunomodulateurs cytokiniques ;les inhibiteurs des chemokines et des adhesines, molecules impliquees dans le « routage » de cellules immunocompetentes vers les organes. Ces nouvelles approches, fondees sur une reflexion immunologique, sont liees a l’identification de cibles impliquees dans l’immunopathologie des MAIS. Ces nouveaux traitements sont en cours de developpement chez l’homme.

Resume Introduction. – Le syndrome catastrophique des antiphospholipides (CAPS) est un mode de revelation rare du syndrome des antiphospholipides (SAPL) dont les facteurs favorisants ne sont pas tous identifies. Le SAPL peut etre une cause de sterilite amenant les patientes a recourir a l'induction d'ovulation (IO). Exegese. – Apres une IO, une patiente de 32 ans est hospitalisee pour une hypertension arterielle severe avec insuffisance renale, defaillance respiratoire et crises convulsives subintrantes. Les antecedents et le bilan immunologique orientent vers un SAPL. La biopsie renale met en evidence une microangiopathie thrombotique. Le diagnostic de CAPS est retenu. Grâce a une assistance respiratoire, un traitement antihypertenseur et anticoagulant, la patiente s'ameliore. Conclusion. – L'IO semble etre le facteur declenchant du CAPS dans cette observation. Afin d'eviter de telles complications, il est important de connaitre l'histoire et le statut immunologique des patientes consultant pour ce traitement afin de mieux prendre en charge leurs grossesses.

Resume Les arteriopathies iatrogenes et toxiques, bien que non exceptionnelles, restent mal connues des medecins. Les troubles vasomoteurs realises peuvent etre tres variables : phenomene de Raynaud, acrorhigose, livedo reticulaire, ergotisme, syndrome amantadinique ou syndrome acrodynique. Les explorations complementaires n'aident pas a l'enquete etiologique. Les diagnostics positif et etiologique reposent sur l'anamnese. Les auteurs precisent les principales caracteristiques des arteriopathies induites par les derives de l'ergot de seigle, les betabloqueurs, les chimiotherapies antineoplasiques, les inhibiteurs calciques, les anorexigenes, le sumatriptan et d'autres molecules pour lesquelles les preuves d'imputabilite sont moins bien etablies. Sont egalement abordees les arteriopathies iatrogenes non medicamenteuses, toxiques professionnelles, et celles induites par la cocaine ou les amphetamines.

Pregnancy in systemic lupus erythematosus patients is a common situation that remains associated with higher maternal and fetal mortality/morbidity than in the general population. Complications include lupus flares, obstetrical complications (fetal loss, in utero growth retardation, prematurity) and neonatal lupus syndrome. The association with antiphospholipid antibodies or antiphospholipid syndrome increases the risk of obstetrical complications. Improving the care of these pregnancies depends upon a systematic pregnancy planning, ideally during a preconception counseling visit and a multidisciplinary approach (internist/rheumatologist, obstetrician and anesthetist). The absence of lupus activity, the use of appropriate medications during pregnancy adjusted to the patient's medical history and risk factors, and a regular monitoring are the best tools for a favorable outcome for these high-risk pregnancies. The aim of this review article is to perform an update on the medical care of pregnancy in systemic lupus erythematosus or antiphospholipid syndrome to reduce the risk of complications and to ensure the best maternal and fetal prognosis.

Fertility is not impaired in systemic lupus erythematosus or antiphospholipid syndrome, but, similarly to the general population, these patients may undergo in vitro fertilization. This type of treatment increases the risk of lupus flare, thrombosis, and ovarian hyperstimulation syndrome. This review will focus on in vitro fertilization in systemic lupus erythematosus or antiphospholipid syndrome. Literature data are relatively scant with only 3 reported studies. The first one included 17 patients and 63 cycles of induction ovulation/in vitro fertilization leading to 25 % of lupus flare, no thrombosis, and 3 % of ovarian hyperstimulation syndrome. The second study included 10 patients and 40 cycles of in vitro fertilization showing 31 % of lupus flare, no thrombosis and no ovarian hyperstimulation syndrome. The last one included 34 patients and 83 procedures of in vitro fertilization leading to 8 % of flares, 5 % of thrombosis and no ovarian hyperstimulation syndrome. Interestingly, in this last study, half of the complications were explained by poor adherence to treatment. These data are reassuring but it is important to remember that in vitro fertilization should be scheduled and carefully supervised in the same way as the high-risk pregnancies occurring in these patients.

Idiopathic Chronic Eosinophilic Pneumonia (ICEP) or Carrington's disease is a rare disease, exclusively pulmonary, and of an unknown origin. Connective tissues of the lungs are infiltrated by eosinophilic cell elements. This illness is progressive, consisting of dyspnea, cough and thoracic pain. In addition, the general condition is impaired. The average delay between onset of symptoms and discovery of chest radiographic opacities is often longer than 3-4 months. Symptoms and chest X-ray quickly improve under corticosteroid treatment. In the future, new research could lead to alternative treatments. We report the case of a woman with ICEP. We shall discuss the diagnostic approach, envisage the potential complications and describe the treatment of the disease.

Background Antineutrophil cytoplasmic antibodies (ANCA) of the IgA isotype have, for the most part, been detected in patients with Henoch-Schonlein purpura (HSP) or inflammatory bowel disease. Objectives We have evaluated the prevalence of IgA ANCA in a series of patients with different causes of cutaneous vasculitis. Methods Forty consecutive patients with histologically proven leucocytoclastic vasculitis were included in the study: 18 had systemic vasculitis as well as cutaneous lesions, 10 of whom were diagnosed as having HSP, and 22 had only cutaneous vasculitis (with no identified cause in 10 cases). IgA ANCA were sought by indirect immunofluorescence using ethanol-fixed human neutrophil preparations as the substrate. Results IgA ANCA were detected in six of 40 patients (15%) (one each with HSP, ulcerative colitis, Sjogren's syndrome, hypergammaglobulinaemia associated with Castelman's disease, erythema elevatum diutinum and bacterial endocarditis). Three of these patients also had IgG ANCA whose target antigen remained unidentified. Conclusions IgA ANCA are rarely observed in HSP (10%) and can be detected in a wide variety of other cutaneous vasculitides.

Vascular involvement is a common complication of Behçet's disease (BD) and affects up to 40% of BD patients. These complications worsen the prognosis of BD. The concept of vasculo-Behçet has been adopted for cases in which vascular complications dominate the clinical features. Vascular manifestations affect particularly young men, during the first years following onset of the disease. Venous complications are the most frequent vascular complications, affecting 14 to 40% of BD patients. Superficial and deep lower limb thrombosis is the most frequent venous complications but one third of venous thrombosis concern large vessels (such as cerebral venous thrombosis, pulmonary embolism, and inferior or superior vena cava, etc.). Budd-Chiari syndrome is the worst prognostic factor increasing mortality by 9 times. Arterial complications (2 to 17% of BD patients) include aneurysms and occlusions/stenosis. Main locations of arterial lesions are aortic (abdominal and thoracic), femoral, pulmonary and iliac arteries. Aneurysms are the most severe arterial complications, particularly pulmonary aneurysms associated with a high risk of massive bleeding. Cardiac complications (up to 6% of BD patients) include pericarditis, endocardial lesions (aortic regurgitation and less often mitral insufficiency), myocardial lesions (myocardial infarction, myocarditis and endomyocardial fibrosis) and intracardiac thrombosis (right ventricle and atrium). Coronary lesions complicated to myocardial infarction are the most severe cardiac complications. Treatment is based on corticosteroids and immunosuppressive drugs. The use of anticoagulation in venous thrombosis is still controversial.

Neurological manifestations of Behçet's disease (BD) occur in 5.3 to more than 50% of patients. They are divided into two major forms: "parenchymal" lesions, which include mainly meningoencephalitis as opposed to "extra-parenchymal" lesions (i.e. cerebral venous thrombosis and arterial aneurysms). Myelitis or peripheral neuropathy is exceptional. The neuro-Behçet syndrome (NBS) should be considered in the setting of neurological manifestations, particularly headache and pyramidal signs, in a young man diagnosed with BD. However, its recognition may be difficult when neurological manifestations are the presenting features of BD (one third of cases), and requires a thorough knowledge of clinical manifestations and morphological lesions. Thus, parenchymal NB lesions classically exhibit inflammatory characteristics on MRI and are located at the meso-diencephalic junction and in the brainstem, rarely with a supratentorial extension. Meningitis is not systematically associated, and may be absent in about 30% of cases. The pathogenesis of these lesions is incompletely understood, but inflammatory infiltrates include mainly neutrophils and activated T cells (mainly Th17). Differential diagnoses include infectious diseases (herpes, listeria, tuberculosis), and inflammatory diseases (i.e. multiple sclerosis and sarcoidosis). A prompt recognition of NBS should lead to initiate adequate therapies in order to limit the risk of sequelae, relapses or death.

The use of Helical CT significantly improves image quality of examinations in a number of clinical settings. It is particularly suited to the study of the tracheo-bronchial tree as a result of new ways of image processing (developed by GEMS research) which can produce virtual endoscopic images without the use of an endoscope. We present our initial anatamo-radiological findings and their educational value as well as our thoughts on potential future clinical applications.

Mesenteric panniculitis is a nonspecific inflammatory process affecting the fatty tissue at the root of the mesentery. This term is also used to describe the clinical and imaging findings in this disorder. Mesenteric panniculitis can be a misleading term: it is commonly misused to design an increased density of the mesentery without prejudice regarding the etiology. Pain is the main clinical symptom. Half of the patients are asymptomatic. There is a palpable mass in half of cases. Laboratory tests sometimes reveal an acute phase reaction of varying intensity. Mesenteric panniculitis is suspected when CT scan shows increased density of the mesenteric fat. Nevertheless, only histological examination could establish the diagnosis. Histologic examination may reveal various stages: lipodystrophy (the first stage when fat necrosis is predominant), mesenteric panniculitis (a majority of infiltrating lymphocytes), sclerosing mesenteritis (the end stage when fibrosis is predominant). Histopathologic differential diagnoses are lymphomas, lipomas, liposarcomas that can mimic mesenteric panniculitis on CT scan. Mesenteric panniculitis is associated with various diseases, especially with intra-abdominal inflammatory process. It also can be idiopathic. Rare complications can occur with vascular or digestive tract compressions. Empirical treatment is only useful in symptomatic patients. Colchicine, corticosteroids or immunosuppressive agents can be used. The only interest of surgery is the histological confirmation of the diagnosis. A better understanding of the pathophysiology of the immunoregulatory functions of adipose tissue will improve mesenteric panniculitis management.

The prevention of thrombosis in the antiphospholipid syndrome (APS) remains controversial. The purpose of this review is to provide updated recommendations. There is evidence that patients at risk of thrombosis are those with "a pattern of high risk antiphospholipid antibodies (aPL)" (presence of a lupus anticoagulant [LA], association of several aPL, or persistent aCL at a medium or high level), or those with associated systemic lupus erythematosus (SLE). The prescription of aspirin in primary prevention is recommended in SLE patients with positive LA or persistent aCL at a significant level. Secondary prevention is based on a very prolonged anticoagulation. An INR around 2.5 seems to be sufficient in patients with venous APS. In case of arterial events, the attitude is debated. We propose to maintain a target INR between 3 and 3.5. The possible occurrence of relapse despite anticoagulation in the therapeutic target may lead to the addition of aspirin. The development of new anti-thrombotic agents might change the management of APS in the coming years.

Summary In a multi-centre prospective study of systemic lupus erythematosus and pregnancy in France, 117 cases were identified from 1987 to 1992. We report significant morbidity and mortality for mother and fetus from an analysis of 103 cases. Pregnancy outcome was as follows: 28 full-term births, 48 premature births, 18 fetal losses (13 early and two late spontaneous abortions, three stillbirths), five therapeutic abortions and four elective abortions (for unwanted pregnancy). Four preterm neonates died. Lupus was active at pregnancy onset in 28 patients. Of 75 patients with inactive lupus at conception, 27 relapsed during pregnancy, and seven postpartum. Two patients with nephrotic syndrome treated with high-dose corticosteroids died from opportunistic infections. Fetal loss correlated with a history of proteinuria and absence of anti-SSA antibodies. Prematurity was related to a history of fetal loss, active lupus at pregnancy onset, hypertension and ≥ 20 mg/day prednisone during pregnancy. Intrauterine growth retardation correlated with pregnancy of short duration, low serum C3 or C4, hypertension, and absence of SSA antibodies. Three of 22 newborns whose mothers had anti-SSA antibodies developed neonatal lupus: two with cutaneous involvement and one with complete atrioventricular block.

The catastrophic antiphospholipid syndrome (CAPS) is a life-threatening condition resulting from rapidly progressive widespread thromboses mainly affecting the microvasculature in the presence of antiphospholipid antibodies. Within a few days, the patients develop multiorgan failure with pulmonary distress, renal failure with severe hypertension, cerebral, cardiac, digestive or cutaneous involvement. CAPS develops in less than 1% of patients with antiphospholipid syndrome, either primary or associated with systemic lupus erythematosus. CAPS reveals the antiphospholipid syndrome in about 50% of cases. CAPS may be precipitated by infectious diseases, surgical procedures or discontinuation of anticoagulation. CAPS overall mortality rate has decreased in the past decade and is now around 30%. Within our hospital, it has been reduced to 10%. The main differential diagnoses are other thrombotic microangiopathies, and heparin-induced thrombocytopenia. The treatment of CAPS consists of the empirical association of anticoagulation and corticosteroids, plus plasma exchange or intravenous immunoglobulins. Cyclophosphamide is added in patients with systemic lupus erythematosus. The prevention of CAPS is based upon the adequate management of the perioperative period when surgery cannot be avoided, the prompt treatment of infections and the education of patients with antiphospholipid syndrome.