[Survey of the management of moderate to severe active Graves' orbitopathy at 28 metropolitan centers of excellence in France]. / État des lieux de la prise en charge de l'orbitopathie dysthyroidïenne modérée à sévère active en France à partir de 28 centres experts métropolitains.

INTRODUCTION: This study investigates the management of Graves'orbitopathy (GO) in France, at 26 university medical centers (CHU) as well as the Rothschild Foundation and the Quinze-Vingts national eye hospital in Paris. METHODS: The 28 metropolitan university medical centers were contacted by telephone or e-mail. The authors devised a 5-item questionnaire that explored the scheduling of multidisciplinary meetings, the existence or lack of a dedicated Graves' consultation service, the place of hospitalization, and first- and second-line treatments. RESULTS: Eighty-nine percent of hospital departments had a dedicated service for patients with GO, with 36% organizing multidisciplinary meetings. Intravenous corticosteroid therapy is still used as first-line treatment, while mycophenolate is used much less (14.3%), despite the new EUGOGO (European Group on Graves' orbitopathy) 2021 recommendations. For second-line treatment, tocilizumab is most commonly used (64%). Teprotumumab is available in France only on a compassionate basis, and its use is limited (18%). CONCLUSION: This study highlights the variability in practices and the importance of a multidisciplinary approach, while calling for national standardization of practices. Despite disparities in the application of recommendations, the emergence of second-line treatments such as tocilizumab and teprotumumab indicates a steady evolution in therapeutic options, although obstacles in terms of accessibility and cost remain.

[Bilateral sub-acute upper limb ischemia revealing Horton's disease, a rare presentation : A case report and literature review]. / Ischémie subaiguë bilatérale des membres supérieurs révélant une Maladie de Horton, une présentation assez rare : à propos d'un cas et revue de la littérature.

STUDY'S GOAL: Our goal is to enrich the medical literature by sharing our experience in managing a case of sub-acute upper limb ischemia that revealed Horton's disease. This is particularly relevant given the current lack of well-established guidelines. INTRODUCTION: Acute upper limb ischemia is rarely seen in Horton's disease. CASE REPORT: We present a case of a 63-year-old female patient with sub-acute ischemia in both upper limbs, accompanied by jaw claudication and absence of bilateral temporal pulses. The CT Angiography scan revealed bilateral occlusions and stenosis of the axillary and sub-clavier arteries, as well as involvement of the external carotid arteries and its branches. The Arteriography confirmed abnormalities in the medium-sized arteries of the supra-aortic trunks. The histopathology examination of the temporal artery biopsy was negative. The patient received emergency corticosteroid therapy followed by an immunosuppressant. Due to the improved clinical condition of the upper limbs, surgical intervention was not performed.

[Local treatments for bullous pemphigoid]. / Les soins locaux dans la pemphigoïde bulleuse.

Bullous pemphigoid affects people who are usually very frail, because they are very old and frequently have neurological co-morbidities. Over the past twenty years, therapeutic management has improved thanks to the application of highly potent corticosteroid-based creams, following a well-established regimen, for several months. Well-conducted nursing care is therefore essential not only to cover wounds, but also to treat the disease. Dedicated nursing time is also an opportunity to provide support to patients, who are often very uncomfortable.

[A trilogy to understand: adrenalin, allergy and anaphylaxis]. / Une trilogie à bien appréhender : adrénaline, allergie et anaphylaxie.

Allergy, anaphylaxis and adrenaline are three closely related terms in medicine. Upon identification or suspicion of anaphylaxis, adrenaline should be administered as soon as possible. An allergy can be characterised by anything from a simple skin rash to severe vital parameter abnormalities. The role of the reception nurse will be first to identify clinically relevant features, to assign the right level of triage to the patient and adjust the care pathway.

Cerebellar syndrome associated with legionellosis: A case report and literature review.

INTRODUCTION: Legionnaire's disease is a community-acquired pneumonia caused by the Gram-negative bacterium Legionella pneumophila. This disease is often associated with neurological symptoms, the clinical presentation of which can be very varied. CASE REPORT: We report a 47-year-old female patient who developed Legionnaires' disease with cerebellar symptoms (ataxia, dysarthria and hypermetria). Laboratory tests revealed a biological inflammatory syndrome. The cerebrospinal fluid was sterile. Urinary antigen test and serology were positive for L. pneumophila. An interstitial syndrome of the right upper lobe was detected on chest computed tomography (CT) scan. Brain imaging (magnetic resonance imaging and CT angiography) showed no abnormalities. The outcome was favourable after treatment with spiramycin, levofloxacin and corticosteroids. DISCUSSION: Few cases only (n=110) of Legionnaires' disease with cerebellar symptoms have been reported in the literature. The pathogenic mechanism behind neurological dysfunction in patients with Legionnaires' disease is unknown. Neurological symptoms improve with antibiotic therapy and corticosteroids. Extra-pulmonary forms of Legionnaires' disease are frequent, with neurological symptoms being the most common symptoms. Cerebellar dysfunction may be underestimated and requires appropriate management with antibiotic therapy and corticosteroid therapy. Recommendations for the management of Legionnaire's disease with severe extra-pulmonary symptoms are needed.

[Which adjuvant measures should be associated with long-term oral corticosteroid therapy in inflammatory diseases? A summary of existing French recommendations]. / Quelles mesures adjuvantes associer à une corticothérapie orale prolongée dans le cadre des maladies inflammatoires ? Synthèse des recommandations françaises disponibles.

It is common to initiate a long-term corticosteroid therapy for inflammatory diseases. Various specialists are involved in this prescription, and associated measures to prevent side effects are not consensual, with the exception of osteoporosis. The specialty of the prescriber has indeed a significant impact on the attention paid to the adjuvant associated measures. The aim of this review was to draw a summary of the side effects of long-term corticosteroid therapy and of the existing recommendations related to associated measures to prevent them. Unfortunately, it is difficult to give clear recommendations because of the lack of evidence in some fields, especially as they should be adapted to patient's age and comorbidities. We propose a summary table of associated measures to long-term steroid therapy prescription and suggest a monitoring frequency.

Treatment of moderate to severe orbitopathy: Current modalities and perspectives.

Graves' orbitopathy (GO) is the primary cause of exophthalmos in adults. It appears in 30 to 50% of patients with Graves' disease. About 5% are moderate-to-severe cases that might be see-threatening or lead to long term disabling sequelae. Recommendations have been established in 2016 by the European thyroid association (ETA) and the European group on Grave's orbitopathy (EUGOGO), suggesting a wide use of corticosteroids in moderate to severe forms. However, disappointing results have been reported in 20 to 30% of cases. Improved understanding of pathophysiological mechanisms has allowed the use of non-specific immunomodulatory agents, currently under evaluation, and which place in the therapeutic strategy remains to be determined. Very recently, new promising therapeutic advances have emerged with the identification of new therapeutic targets, such as the TSH receptor and IGF-1 receptor complex.

[A rare case of Horton's disease without inflammatory syndrome in the Department of Neurology of the Teaching Hospital of Point G, Bamako, Mali]. / Un cas rare de maladie de Horton sans syndrome inflammatoire dans le Service de Neurologie du CHU du Point G, Bamako, Mali.

Horton's disease is the most common inflammatory disease of the vessels after the age of 50 years. We report here the caseof a 64-year-old male, who presented a chronic frontal headache resistant to usual analgesics. Physical examination found pain on bitemporal pressure, a slight induration of temporal arteries and a bilateral decreased temporal pulse. Biological inflammatory testing was normal. However, Doppler ultrasound revealed bilateral intimal infiltration of the temporal artery, supporting the diagnosis of Horton's disease. Put on corticoids, headaches were completely resolved.

La maladie de Horton (MH) est la plus fréquente des maladies inflammatoires des vaisseaux après l'âge de 50 ans.Nous rapportons le cas d'un patient de 64 ans, ayant présenté une céphalée frontale, d'aggravation progressive etrebelle aux antalgiques usuels. L'examen physique a retrouvé une douleur à la pression bitemporale, une légère induration et une diminution des pouls temporaux de façon bilatérale. Le bilan biologique inflammatoire est revenu normal. Par contre, l'échographie doppler a mis en évidence une infiltration intimale bilatérale de l'artère temporale en faveur de la MH. Mis sous corticothérapie, le patient a rapporté une disparition des céphalées.

[Cerebral infarction and tuberculosis: case report and literature review]. / Infarctus cérébral et tuberculose : rapport d'un cas et analyse de la littérature.

INTRODUCTION: Although tuberculous meningitis is an uncommon presentation of tuberculosis, it still remains one of the deadliest forms of this disease. In this context, the occurrence of a cerebral infarct is an aggravating factor. OBSERVATION: A 48-year-old Asian man presented himself in the emergency room for dysarthria and dysphagia of progressive onset. Cerebral CT showed a recent ischemic defect of the right internal capsule. Lumbar puncture showed meningitis with low sugar levels. Pulmonary micronodules on the thoracic CT suggested tuberculosis, which was confirmed by a broncho-alveolar lavage. Anti-tuberculosis treatment and early corticosteroid resulted in an improvement of the patient's state. CONCLUSION: Cerebral infarctions in patients with tuberculous meningitis are events that cannot be underestimated in terms of frequency or severity. Their poor prognosis is partly the result of insufficiently defined management, which combines anti-tuberculosis treatment and early corticosteroid therapy.

[Treatment of noninfectious intermediate uveitis, posterior uveitis, or panuveitis]. / Traitement des uvéites intermédiaires, postérieures et panuvéites non infectieuses.

Controlling long-term inflammation during non-infectious intermediate, posterior or panuveitis while limiting side effects remains challenging. There is no standardized pre-therapeutic evaluation providing diagnostic certainty, but some simple tests allow us to identifiy the main etiologies. The ophthalmologist identifies the type of uveitis, and the internist completes the investigations according to the ophthalmologist's findings. Fundus photographs, optical coherence tomography, and fluorescein and indocyanine green angiography should be considered during diagnosis and follow-up. Ocular complications of uveitis are numerous. They require close monitoring and specific medical and sometimes surgical management. The growing number of available drugs makes it possible to optimize the management of these conditions with varied etiologies and presentations. Currently, systemic corticosteroids remain the mainstay of therapy, and other alternatives are considered in the case of poor tolerance, steroid resistance or dependence. The choice of a systemic, periocular or intravitreal treatment depends on several factors: chronicity or recurrence of uveitis, duration, bilaterality, association with a systemic inflammatory disease, the presence of contraindications to certain treatments, and also socioeconomic constraints. It is of the utmost importance to find the best compromise allowing tight control of ocular inflammation by means of adapted systemic and/or local treatment while avoiding the main complications.

[Strokes and Giant cell arteritis: imaging characteristics]. / AVC et Horton : caractéristiques de l'imagerie.

Giant cell arteritis (GCA) is associated with an increased risk of cardio- or cerebro-vascular death. The stroke rate in patients with GCA varies between 2.7 and 7.4%. The etiological diagnosis may be challenging between atherosclerotic stroke and stroke related to GCA. Case of an old woman who had ischemic stroke and concomitant diagnosis of GCA and brain imaging characteristics.

[Cataract surgery in children with non-infectious uveitis: Review of current practices in France]. / Chirurgie de la cataracte uvéitique non infectieuse de l'enfant : bilan des pratiques actuelles en France.

PURPOSE: To evaluate the medical-surgical management of cataract surgery in children with chronic uveitis in various French pediatric ophthalmology centers. MATERIALS AND METHODS: Two-part study: first, a descriptive observational segment on the evaluation of French practices. A questionnaire was sent to the various pediatric ophthalmologists in France. A second retrospective chart review, including children with non-infectious chronic uveitis who had cataract surgery in the pediatric ophthalmology department of Bordeaux University Hospital from 2008 to 2017. RESULTS: Twenty-one ophthalmologists responded to the questionnaire. Only 23.8% systematically initiated immunosuppressive drugs (aside from corticosteroids) before surgery. A total of 88.2% prescribed oral corticosteroid treatment preoperatively. Eleven surgeons administered intravenous corticosteroid boluses during the surgery, and primary lens implantation is the most common method used in 95.2%. A total of 76.2% initiated oral steroid therapy after surgery. Postoperatively, all surgeons started local therapy with high-dose corticosteroids. At one year, 100% achieved improvement of visual acuity greater than or equal to 2 lines. On our service, 10 eyes (7 children) underwent cataract surgery. Seven were treated with systemic immunosuppressive drugs (aside from corticosteroids) and 80% of cases received oral corticosteroid therapy a few days before surgery. An intravenous corticosteroid bolus was administered preoperatively in 8 cases, and primary lens implantation was performed in 100% of cases. Postoperatively, 5 children received oral corticosteroid treatment. All were treated with local high dose steroids. At one year, the mean best-corrected visual acuity was 0.18 LogMar (0-0.7, SD: 0.25). CONCLUSION: When performed with an aggressive anti-inflammatory protocol, cataract surgery leads to a good visual outcome in selected children with chronic uveitis.

[Glucocorticoid sensitive bilateral leg swelling in an 85-year-old woman presenting with polymyalgia rheumatica: A case report]. / Œdèmes des membres inférieurs cortico-sensibles chez une patiente âgée de 85 ans présentant une pseudopolyarthrite rhizomélique.

INTRODUCTION: Polymyalgia rheumatica (PMR) can be associated with distal swelling indicating an associated RS3PE syndrome. We report a case of PMR associated with oedema of the lower limbs, which resolved rapidly under glucocorticoid therapy. CASE REPORT: A 85-year-old woman presented with a 4 month history of PMR responding to the 2012 EULAR/ACR classification criteria. Examination of the lower limbs revealed pitting oedema bilaterally up to the knees, with mild erythema and warmth. Hypoalbuminemia (30g/L) was present. There was no cardiac, renal or hepatic cause to explain leg swelling. FDG-PET/CT demonstrated increased metabolism in the periarticular area of shoulders and hips. There was no sign of aortitis or neoplasia. Under treatment with prednisone 10mg/day leg swelling disappeared concomitantly to a weight loss of 8kg within 8days. CONCLUSION: This case, the first to report leg swelling of inflammatory origin in the context of PMR, could indicate an increased vascular permeability caused by inflammation in the elderly.

N° 364 - La Corticothérapie Prénatale Pour Améliorer Les Issues Néonatales.

OBJECTIF: Évaluer les avantages et les risques de la corticothérapie prénatale chez les femmes qui présentent un risque d'accouchement prématuré ou qui subissent une césarienne à terme avant début de travail, et formuler des recommandations visant l'amélioration des issues néonatales et des issues à long terme. OPTIONS: Administrer ou ne pas administrer une corticothérapie prénatale aux femmes qui présentent un risque élevé d'accouchement prématuré ou qui subissent une césarienne avant travail à terme. RéSULTATS: Morbidité périnatale, notamment le syndrome de détresse respiratoire, l'hémorragie intraventriculaire, la dysplasie bronchopulmonaire, l'infection, l'hypoglycémie, ainsi que les troubles de la croissance somatique et cérébrale et du neurodéveloppement; mortalité périnatale; et morbidité maternelle, notamment l'infection et la suppression surrénalienne. UTILISATEURS CIBLES: Fournisseurs de soins de maternité, notamment les sages-femmes, les médecins de famille et les obstétriciens. POPULATION CIBLE: Femmes enceintes. ÉVIDENCE: Nous avons interrogé les bases de données Medline, PubMed et Embase ainsi que la Bibliothèque Cochrane, de leur création au mois de septembre 2017. Nous nous sommes servis de Medical Subjet Headings (MeSH) et de mots clés en lien avec la grossesse, la prématurité, les corticostéroïdes ainsi que la mortalité et la morbidité périnatales et néonatales. Nous avons également consulté les déclarations d'organismes professionnels tels que les National Institutes of Health, l'American College of Obstetricians and Gynecologists, la Society for Maternal-Fetal Medicine, le Royal College of Obstetricians and Gynaecologists et la Société canadienne de pédiatrie pour obtenir des références additionnelles. Les essais cliniques randomisés évaluant la corticothérapie prénatale menés sur des femmes enceintes et les revues systématiques antérieures sur le sujet étaient admissibles, tout comme les données venant de revues systématiques d'études non expérimentales (études de cohorte). VALEURS: La présente opinion de comité a été révisée et approuvée par le Comité de médecine fœto-maternelle de la SOGC, et approuvée par le Conseil de la SOGC. AVANTAGES, INCONVéNIENTS ET COûTS: L'administration d'une corticothérapie prénatale dans les sept jours précédant l'accouchement réduit significativement la morbidité et la mortalité périnatales associées à la naissance prématurée survenant entre 24+0 et 34+6 semaines de grossesse. Si la corticothérapie prénatale est administrée plus de sept jours avant l'accouchement ou après 34+6 semaines de grossesse, les effets indésirables peuvent surpasser les avantages. Les données probantes sur l'impact à long terme de la corticothérapie prénatale sont rares. Par ailleurs, les effets neurodéveloppementaux néfastes potentiels de l'exposition répétée à la corticothérapie prénatale ou de l'administration de corticostéroïdes en période préterme tardive ou à terme n'ont pas été quantifiés. MIS-à-JOUR à LA DIRECTIVE: Une revue des données probantes sera menée cinq ans après la publication de la présente directive clinique afin d'évaluer si une mise à jour complète ou partielle s'impose. Si de nouvelles données probantes importantes sont publiées avant la fin de ces cinq ans, une mise à jour tenant compte des nouvelles connaissances et recommandations sera publiée. COMMANDITAIRES: La présente directive clinique a été élaborée à l'aide de ressources fournies par la Société des obstétriciens et gynécologues du Canada et avec l'appui des Instituts de recherche en santé du Canada (APR-126338). MOTS CLéS: Corticothérapie prénatale, maturation fœtale, prématurité, période préterme tardive, césarienne avant travail DÉCLARATION SOMMAIRES: RECOMMANDATIONS: Considérations relatives à l'âge gestationnel.

[Bilateral pseudotumoral proptosis as presenting sign of orbital hyper-IgG4-related disease]. / Exophtalmie bilatérale pseudo-tumorale révélant un syndrome hyper-IgG4 orbitaire.

INTRODUCTION: Hyper-IgG4 syndrome is a rare cause of bilateral proptosis. It must always be considered after ruling out orbital lymphoma. CASE REPORT: We report a case of progressive bilateral proptosis for 4 years in a 34-year-old man. Orbital MRI showed an infiltrative process extending to the orbital fat, extraocular muscles and lacrimal glands. Lacrimal gland biopsy with immunohistochemical study showed a lymphoplasmocytic infiltrate rich in IgG4 and fibrosis. The diagnosis of orbital hyper-IgG4 syndrome was suggested. The patient responded well to systemic steroid treatment. DISCUSSION: Orbital hyper-IgG4 syndrome manifests most often as pseudo-tumoral bilateral proptosis. Elevated IgG4 levels are neither sensitive nor specific. Biopsy with immunohistochemical study is the key to diagnosis. Systemic steroid treatment is the gold standard, but recurrences may occur.

[Intra-cardiac manifestation during adult-onset Still's disease's, a tricuspid vegetation as a rare expression of systemic disease]. / Végétation tricuspidienne au cours d'une maladie de Still, une manifestation non infectieuse rare de lésion intracardiaque.

INTRODUCTION: Adult-onset Still's Disease is a rare multisystemic inflammatory disease characterized by fever, maculo-papular erythematous rash and arthralgia. Adult-onset Still's disease is a diagnosis of exclusion. CASE REPORT: We report the case of a 33 years old man, hospitalized for fever, arthralgia and throat manifestations, leading to Adult-onset Stills's Disease diagnosis. Cardiac ultrasound revealed tricuspid vegetation. Once infectious causes were ruled out, the vegetation was related to Adult-onset Still's Disease according to Fautrel and Yamaguchi criteria. The patient was treated with systemic high doses corticosteroid and cardiac surgery. Histological examination excluded infection and neoplasia, and showed cruoric and fibrinous vegetation. CONCLUSION: Non-infectious endocarditis, with a vegetation made of cruoric and fibrinous material, is a rare complication of Adult-onset Still's disease.