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Takayasu arteritis (TAK) is a large vessel vasculitis affecting young women of childbearing age. The outcome of pregnancies in TAK patients, factors associated with maternal and foetal complications and adverse outcomes were analysed.All pregnancies in women with a TAK diagnosis were retrospectively included from 20 French hospitals providing care for TAK, until August 2015.The study consisted of 43 pregnancies in 33 women, including 29 with a pre-existing TAK diagnosis and 4 diagnosed during pregnancy. Complications were observed in 20 pregnancies (47%), including 35% with arterial hypertension (n = 15), 9% with pre-eclampsia (n = 4), 2% with HELLP syndrome (n = 1) and 14% with intrauterine growth restriction (IUGR, n = 6, leading in one case to a medically indicated termination of pregnancy). There were 42 live births (98%) at a median term of 38 [27-42] weeks gestation including 9 before 37 weeks (21%). The median birth weight was 2940 [610-4310] grams. Five children (12%) required transfer to a neonatal intensive care unit. One premature boy (27 weeks gestation) died after 2 days. Treatment during pregnancy included steroids (n = 25/43; 58%), azathioprine (n = 9/43; 21%) and infliximab (n = 1/43; 2%). The risk of developing arterial hypertension during pregnancy was associated with previous chronic arterial hypertension and with an infra-diaphragmatic vasculitis injury (P = 0.01 and P = 0.04, respectively). No correlation was reported between TAK activity and any of the obstetrical complications described in the study.This study showed a high rate of adverse obstetrical complications without significant impact on live birth rates. Pregnancy did not appear to influence TAK disease activity. Key Points • We observed a high rate of adverse obstetrical complications in women with Takayasu arteritis; however, the rate of live births was high. Pregnancy did not appear to influence TA disease activity.

Objective: To investigate the prevalence, clinical features and outcomes of coronavirus disease 2019 (COVID-19) among sarcoidosis patients. Methods: We retrospectively collected features of COVID-19 in a cohort of patients with sarcoidosis followed in a single tertiary university hospital. Results: Among 199 sarcoidosis patients [mean age 58.8 (±14) years, 86 (43.2%) men], 26 (13%) were diagnosed with COVID-19 [definite (n=7), probable (n=12) and possible (n=7)]. Twenty-four out of 26 patients (92%) had at least one comorbidity, and 11/26 (42%) had two or more comorbidities. Demographic and clinical features of COVID-19 positive patients were similar to those of COVID-19 negative patients. The administration of hydroxychloroquine or immunosuppressant was not associated with the occurrence or the severity of COVID-19. Four out of 26 (15.4%) COVID-19 positive patients required admission to hospital and two of them died. Hospitalized patients [mean age of 61 (±11.5) years] were receiving higher doses of long term treatment with corticosteroids than non-hospitalized patients; 4/4 had pulmonary and 2/4 cardiac involvement of sarcoidosis, and all one or more comorbidity. Conclusion: The prevalence of COVID-19 in sarcoidosis is slightly higher to that of the general population. Almost half of the COVID-19 positive patients have two or more comorbidities and about 15% present a severe course.

Sarcoidosis is a systemic granulomatous disease characterized by pulmonary involvement in most patients and more rarely by extrapulmonary involvement such as ocular, skin, salivary, lymph nodes and joints damages. Neurological and cardiac involvements are uncommon but are associated with increased morbidity and mortality. Cardiac sarcoidosis affects 5 to 20% of patients depending on the studies and autopsy studies even report cardiac involvement in 25% of sarcoidosis patients. This review aims to summarise main data on the diagnostic value of the different imaging techniques in cardiac sarcoidosis and to also detail the management of these patients who require a multidisciplinary approach.

Background Takayasu arteritis (TA) is a large-vessel vasculitis that affects young women of childbearing age. Several small case-series described the pregnancy outcome in TA patients with lack of study determining risk factors associated with adverse pregnancy outcome. Objectives We performed a French retrospective study, to determine the risk factors associated with obstetrical adverse outcome; and the relation between pregnancy outcome and TA disease activity. Methods French nationwide retrospective study of pregnancies that lasted at least 12 weeks of gestation (WG) in TA patients. Results Forty-three pregnancies occurred in 33 patients. The diagnosis of TA was preexisting in 29 patients, and done during pregnancy in 4. For the 39 pregnancies in the 29 patients with a preexisting diagnosis of TA: steroids were maintained throughout pregnancy in 23/39 (59%) with a median dose of 5 mg/day2–40 immunosuppressive treatment during pregnancy included azathioprine (n=9, 21%) or infliximab (TNF-α antagonist) (n=1, 2%). For the 4 pregnancies with TA diagnosis during, only one was treated by steroids. Aspirin (100 mg/day) was used in 27/43 pregnancies (63%) and antihypertensive drugs were used in 10 pregnancies (23%). Before pregnancy, immunosuppressive treatment had been used in 16 patients: azathioprine (n=10, 30%), methotrexate (n=7, 21%), TNF-α antagonist (infliximab in 3 and adalimumab in 1; n=4, 12%) and cyclophosphamide (n=2, 6%). Maternal adverse events were noted in 20 pregnancies (47%). The most frequent adverse event was arterial hypertension (n=12; 28%): 10 worsening of previous arterial hypertension and 2 de novo arterial hypertension. Other adverse events included pre-eclampsia (n=3; 7%), HELLP syndrome (n=1; 2%) and post-partum haemorrhage (n=2; 5%). No maternal death was observed. There were 42 live births (98%) delivered at a median term of 3827–42 WG with 9 (21%) before 37 WG and one medical termination of pregnancy for major IUGR at 21WG. IUGR was observed in 6 pregnancies (14%) associated with hypertension and pre-eclampsia or HELLP syndrome in 2 cases. The median birth weight was 2940 [610–4310] grams. Five children (12%) required intensive care units hospitalisation. One premature boy (27 WG) died after 2 days. Treatment during pregnancy included steroids (n=25/43, 58%), azathioprine (n=9/43; 21%) and infliximab (n=1/43; 2%). Pre-eclampsia were less frequent in patients treated with steroids during pregnancy (p=0.02). The risk of developing arterial hypertension was associated with previous chronic arterial hypertension, and an infra-diaphragmatic vasculitis injury (p=0.01 and p=0.04). Activity of TA was observed in the course of 12/43 pregnancies (28%). Conclusions We observed both a high rate of obstetrical complications and of live birth. A preexisting chronic arterial hypertension, the infra-diaphragmatic location of vasculitis and/or an active disease in the 6 months preceding the pregnancy were associated with an impaired pregnancy outcome. TA disease activity did not seem to be influenced by pregnancy. Disclosure of Interest None declared

International audience; Sarcoidosis is associated with cell-mediated immunodeficiency and treatment of symptomatic sarcoidosis usually includes systemic immunosuppressants. Data relative to incidence, prognosis factors, and outcome of infections are scarce.Retrospective cohort study of 585 patients with biopsy proven sarcoidosis in a tertiary referral specialist clinic, with a nested case-control analysis. Twenty nine patients (4.9%) with severe infections were compared to 116 controls subjects with sarcoidosis, matched according to their gender, ethnicity, age at diagnosis, and treatment with corticosteroids.After a median follow-up of 8 years [range; 1–46], 38 severe infections [mycobacterial infections (n = 14), fungal infections (n = 10), bacterial (n = 8), viral (n = 3) and parasitic (n = 1)] were observed in 30 patients. The incidence of severe infections was 0.71% persons-year (CI 95% 0.5–0.98) and 0.43% persons-year (CI 95% 0.27–0.66). Patients with severe infection were more frequently of male gender (60% vs 46%) and were more likely treated by ≥ 3 immunosuppressive agents (OR = 3.8, IC 95% [1.5–9.64], P = .005) and by cyclophosphamide (OR = 5.55, IC 95% [1.9–16.1], P = .002), and with neurological (OR = 3.36 CI 95% [1.37–8.25], P = .008), or cardiac (OR = 2.65 CI 95% [1.09–6.43], P = .031) involvement of the sarcoidosis, compared to the controls. Two patients died within the 6 months following infection, due to progressive multifocal leucoencephalopathy (n = 1), and of peritonitis (n = 1).Severe infections are observed in 5.1% of our patients with sarcoidosis after a median follow-up of 8 years. Risk factors for severe infections included neurological or cardiac involvement of sarcoidosis, the use of immunosuppressive agents and mainly cyclophosphamide.

Resume Introduction La sarcoidose cardiaque (SC) est l’une des principales causes de morbi-mortalite de la sarcoidose. Son diagnostic reste aujourd’hui difficile. La TEP au 82-Rubidium est utilisee comme imagerie de perfusion myocardique dans la SC. Cependant, la quantification du debit sanguin myocardique (DSM) n’a pas encore ete etudiee dans cette indication. Le but de cette etude etait d’evaluer son apport pour le diagnostic de SC. Patients et methodes Trente-sept patients ont ete inclus (47,7 ± 12,2 ans ; fraction d’ejection = 50,6 ± 7,8 %), avec un diagnostic histologique de sarcoidose et une suspicion d’atteinte cardiaque. Le diagnostic de SC etait retenu ou non par une reunion pluridisciplinaire (RCP). Tous les patients passaient une TEP au 82-Rubidum avec acquisitions de repos et de stress. Le resultat de la RCP etait compare a l’analyse visuelle, a la quantification du DSM et a une analyse combinee visuelle puis quantitative dans les cas douteux de la TEP. Resultats Trente-deux examens TEP ont ete analyses (21 patients sans atteinte cardiaque, 11 malades). La sensibilite etait de 90,9 % et la specificite de 90,5 % avec l’analyse visuelle. Il n’existait pas de difference significative entre le DSM des patients sains ou malades pour l’ensemble du ventricule gauche ou pour chaque territoire vasculaire. La sensibilite etait alors de 100 % et la specificite de 81 % avec l’analyse combinee. Conclusion La TEP au 82-Rubidium est un examen performant pour le diagnostic de SC. La quantification du DSM dans les cas douteux permet d’augmenter la sensibilite en maintenant une bonne specificite.

Introduction L’atteinte hypothalamo-hypophysaire (HH) au cours de la sarcoidose est rare et peu decrite. Elle peut entrainer des insuffisances antehypophysaires, une hyperprolactinemie de deconnexion et un diabete insipide. Patients et methodes Etude descriptive multicentrique retrospective sur 32 patients avec sarcoidose HH. Resultats Trente-deux patients (18 femmes) ont ete inclus. Les principaux signes cliniques endocriniens etaient : asthenie (n = 19), amenorrhee (n = 12), dysfonction erectile (n = 10). Trente et un patients avaient des anomalies antehypophysaires : deficit gonadotrope (n = 25), thyreotrope (n = 20), somatotrope (n = 11), corticotrope (n = 2), hyperprolactinemie (n = 24). Quinze avaient un diabete insipide. Un syndrome hypothalamique caracterise par une prise de poids massive (mediane 22 kg) etait retrouve chez 6 patients. L’IRM etait anormale chez 31 patients : tige epaissie (n = 20), infiltration de l’hypothalamus (n = 17), anomalies hypophysaires (n = 11). Tous avaient une sarcoidose multisystemique, dont 26 confirmes par histologie. Parmi les patients recontroles apres traitement de la sarcoidose : 11 avaient une prolactine normalisee (58 %) et 8 avaient recupere une fonction gonadotrope (42 %). Aucune guerison de diabete insipide n’a ete rapportee. Discussion Les deficits gonadotrope, thyreotrope et l’hyperprolactinemie sont les atteintes les plus frequentes dans notre etude realisee sur la plus grande cohorte de sarcoidose HH publiee. Une recuperation de la fonction gonadotrope est observee dans 42 % des cas apres traitement, et une normalisation de la prolatine dans 58 %. La moitie des patients presente un diabete insipide definitif.

Sarcoidosis may affect functional outcome and sometimes may be a life-threatening condition. Functional outcome is particularly compromised by cutaneous, ocular, nasal, osteoarticular manifestations and some neurological expression. Central nervous system involvement and cardiac sarcoidosis could be life-threatening, as laryngeal and renal involvement. Diagnosis is sometimes very difficult to obtain. Several tissue biopsies are usually necessary to rule out all differential diagnosis. Extrathoracic manifestations of severe sarcoidosis require a corticosteroid therapy, initiated, if life-threatening at a daily dose of 1 mg/kg, usually associated with an other immunosuppressive drug (methotrexate, cyclophosphamide, anti TNFα).

Resume Les formes chroniques de sarcoidose posent en pratique de difficiles problemes therapeutiques. Le but de cette etude observationnelle etait d’analyser l’efficacite et la tolerance de l’infliximab dans la sarcoidose chronique. Cette etude retrospective multicentrique portait sur 31 cas de sarcoidose chronique, pulmonaire et/ou systemique, traites par infliximab. La maladie evoluait depuis neuf ans et touchait en moyenne quatre organes. Les patients avaient recu plusieurs immunosuppresseurs et 30/31 beneficiaient d’une corticotherapie (19 ± 16 mg/j) associee dans 17 cas a un ou plusieurs immunosuppresseurs. La duree du traitement par infliximab etait de 13 ± 12 mois. L’efficacite de l’infliximab etait observee dans 62 % des cas tous organes confondus : 65 % pour les atteintes pulmonaires, 67 % de succes pour les atteintes cutanees, 50 % de succes pour les atteintes du systeme nerveux central. Pour les autres organes, les reponses etaient disparates. L’effet d’epargne cortisonique etait faible (2,8 ± 9,7 mg/j). L’efficacite etait plus frequente chez les patients sous immunosuppresseurs. Treize des 31 patients ont presente des effets secondaires, dont sept severes necessitant parfois l’arret de l’infliximab. Notre etude confirme l’interet de l’infliximab dans le traitement de la sarcoidose chronique, mais illustre aussi la frequence et la severite des effets secondaires. Les indications restent difficiles a preciser et son utilisation doit actuellement etre reservee aux protocoles d’etude.

Introduction L’atteinte hypothalamo-hypophysaire (HH) au cours de la sarcoidose est rare, inferieure a 1 %, et peu decrite. L’infiltration granulomateuse peut entrainer des insuffisances antehypophysaires, une hyperprolactinemie de deconnection et un diabete insipide. L’objectif de notre travail etait de mieux decrire les atteintes HH au cours de la sarcoidose, afin d’ameliorer le diagnostic et la prise en charge. Patients et methodes Une etude multicentrique retrospective a permis d’inclure 32 patients avec une sarcoidose systemique avec atteinte HH, diagnostiques entre 1991 et 2016. Nous avons realise une analyse descriptive des donnees cliniques, biologiques, hormonales et radiologiques. Resultats Parmi les 32 patients inclus, il y avait 18 femmes et 14 hommes. L’âge moyen au diagnostic de sarcoidose HH etait de 40,5 ans (± 13,4). La majorite des patients (n = 29) presentaient des symptomes compatibles avec un dysfonctionnement hormonal : asthenie (n = 19), amenorrhee (n = 12/14 femmes en âge de procreer), dysfonction erectile et/ou diminution de la libido (n = 10/14 hommes). Trente et un patients avaient une insuffisance antehypophysaire : 25 deficits gonadotropes (78 %), 20 deficits thyreotropes (65 %), 11 deficits somatotropes (46 %) et 2 deficits corticotropes. Presque la moitie des patients (n = 15) avait un diabete insipide, dont 1 seul cas d’hypernatremie a 149 mmol/L. Une hyperprolactiemie a ete mesuree chez 24 patients (77 %). Un syndrome hypothalamique a ete retrouve chez 6 patients (19 %), caracterise par une prise de poids massive en quelques mois (mediane 22 kg). Des anomalies radiologiques etaient quasi constantes sur l’IRM (n = 31) : epaississement focal ou diffus de la tige pituitaire (n = 20), infiltration de l’hypothalamus ou du plancher du 3e ventricule (n = 17), anomalies hypophysaires (n = 11). Tous les patients avaient une sarcoidose multisystemique avec comme localisations principales : ganglionnaire (n = 24), pulmonaire (n = 23) et neurologique (n = 18), dont 5 patients asymptomatiques avec des anomalies IRM isolees. Les atteintes ORL etaient frequentes avec une localisation naso-sinusienne pour 10 patients (31 %) et 9 cas de parotidites (28 %). L’enzyme de conversion etait augmentee dans 68 % des cas (n = 19/28) et 3 patients avaient une hypercalcemie. Le diagnostic histologique de sarcoidose a ete etabli pour 26 patients. La quasi-totalite des patients a ete traitee par corticotherapie (n = 29), le plus souvent en association avec un autre immunosuppresseur (n = 20) : methotrexate (n = 11), infliximab (n = 7) ou mycophenolate mofetil (n = 5). La duree moyenne de suivi etait de 10 ans (± 7,5). Parmi les patients recontroles sur le plan hormonal : 11 avaient une prolactine normalisee (58 %), 8 avaient recupere une fonction gonadotrope (30 %) et 1 seul avait recupere sa fonction thyreotrope. Aucune guerison de diabete insipide n’a ete rapportee. Seuls 10 patients (31 %) etaient consideres en remission de leur sarcoidose a la fin du suivi. Conclusion Les deficits gonadotrope, thyreotrope et l’hyperprolactinemie sont les atteintes les plus frequentes dans notre etude realisee sur la plus grande cohorte de sarcoidose HH. Une recuperation de la fonction gonadotrope a ete observee dans 30 % des cas apres traitement. La moitie des cas presente un diabete insipide definitif. Les patients avec atteinte HH ont tous une sarcoidose multisystemique, et il semblerait que les localisations naso-sinusiennes et parotidiennes soient plus frequentes.

Resume Maladie inflammatoire, granulomateuse multisystemique, la sarcoidose demeure toujours de cause inconnue. Le systeme nerveux represente une des localisations rares et graves de cette maladie benigne. La meningite lymphocytaire, les troubles psychiques, le diabete insipide, la paralysie faciale peripherique en sont les manifestations les plus classiques. L’etude du LCR et l’IRM cerebrale et medullaire cervicale avec injection de gadolinium sont les investigations de choix. Une preuve histologique est parfois obtenue au prix de biopsies neuromusculaires, meningees, cerebrales stereotaxiques. Les risques fonctionnels et vitaux imposent une corticotherapie precoce. En cas d’aggravation, de non-reponse ou d’une corticodependance, d’autres immunosuppresseurs ou exceptionnellement une irradiation encephalique seront associes. Le traitement d’entretien, le plus souvent a vie permet de maintenir le succes therapeutique et evite les rechutes.

Resume Propos. – Revue de la litterature sur l'epidemiologie de la sarcoidose et les differents facteurs susceptibles d'influencer la maladie. Actualites et points forts. – Les etudes epidemiologiques montrent que la sarcoidose est une maladie planetaire, dont l'emergence varie en fonction de multiples elements predisposants. Les plus determinants sont la race, le sexe, l'âge, les facteurs hereditaires, genetiques et/ou infectieux, mais un seul de ces facteurs n'est pas suffisant. Perspectives et projets. – Pour qu'une sarcoidose s'exprime chez un individu, doivent coexister des facteurs environnementaux (agent infectieux, climat, lieu) et des facteurs predisposants personnels (race, sexe, heredite…). Dans ces conditions, chaque patient presentera sa sarcoidose avec ses propres particularites.

Resume L'analyse de l'activite electrique du cœur repose sur une methodologie d'enregistrement et de lecture rigoureuse. La connaissance de l'electrocardiogramme normal, ainsi que ses variations physiologiques sont un prealable indispensable.

Resume L’electrocardiogramme (ECG) enregistre l’activite electrique du cœur. Cette activite electrique de l’ensemble des cellules cardiaques peut etre assimilee a celle d’une seule des cellules du cœur.

Resume Propos Etat des connaissances sur la sarcoidose du systeme nerveux central. Actualites et points forts Une localisation dans le systeme nerveux central est observee dans 5 a 16% des cas de sarcoidose. Les manifestations cliniques neurologiques sont variees: epilepsie, troubles cognitifs ou psychiques, manifestations hypothalamohypophysaires, tableaux focaux d'allure pseudotumorale, hydrocephalie, association tres frequente d'une meningite aseptique lymphocytaire, latente et avec une frequence variable de l'atteinte de nerfs crâniens, en particulier le nerf VII. L'atteinte du systeme nerveux central est le plus souvent precoce et revelatrice et s'integre quasi constamment dans le cadre d'une sarcoidose systemique typique, d'emblee evidente ou plus rarement differee. L'atteinte isolee du systeme nerveux central reste exceptionnelle. Le diagnostic repose principalement sur deux arguments: la confirmation du diagnostic de sarcoidose systemique et la compatibilite clinique et paraclinique (notamment en IRM) des anomalies neurologiques. Une confirmation histopathologique neurologique est rarement necessaire et/ou possible. Un traitement corticoide general est necessaire dans toutes les formes symptomatiques et permet le plus souvent une reponse clinique et une regression des lesions actives identifiees en imagerie, avec une amelioration clinique plus ou moins complete. Le traitement est le plus souvent prolonge sur plusieurs annees, la posologie etant guidee sur l'evolution clinique et en imagerie qui permettent de determiner la dose seuil necessaire. L'atteinte du systeme nerveux central demeure une localisation potentiellement grave de la sarcoidose avec une mortalite et une morbidite non negligeables. La prise en charge evolutive doit etre rigoureuse pour limiter les risques iatrogenes en fonction des enjeux therapeutiques. Perspectives et projets Des etudes multicentriques sont necessaires pour preciser les facteurs intervenant dans l'incidence, la presentation et le pronostic a distance sous traitement de la sarcoidose du systeme nerveux central. La place des traitements epargnant en corticosteroides doit etre precisee.

Resume Propos Rechercher une interaction reciproque entre le deroulement d'une grossesse et le profil evolutif d'une sarcoidose. Methodes Tous les evenements obstetricaux survenus chez 11 femmes suivies par ailleurs pour une sarcoidose prouvee par histologie ont ete notes. Pour chaque grossesse etudiee, le deroulement de la grossesse et l'influence de la grossesse sur l'evolution de la sarcoidose ont ete enregistres. Resultats Sur 33 grossesses etudiees, 23 ont permis la naissance de nouveau-nes en bonne sante (cinq fausses couches, quatre interruptions volontaires, une interruption therapeutique). Le seul fait notable a ete la decouverte de six hypotrophies fœtales, dont trois sont survenues chez des patientes ayant une sarcoidose active traitee par prednisone durant la grossesse. Il n'a pas ete observe de rechute pour les sarcoidoses gueries, et les sarcoidoses inactives en debut de grossesse le sont demeurees. Les sarcoidoses actives en debut de grossesse avaient une evolution variable: amelioration (un cas), stabilisation (deux cas) et aggravation (deux cas). Durant l'annee qui a suivi l'accouchement, quatre rechutes ont ete notees, ainsi que les premiers signes de sarcoidose dans deux cas. Conclusion En dehors d'un risque possible, mais non certain d'hypotrophie, il ne semble pas exister d'effet deletere de la sarcoidose sur le deroulement de la grossesse. La grossesse ne semble pas interferer sur le mode evolutif de la sarcoidose. Compte tenu du risque de rechute durant le post-partum, une surveillance clinique et radiologique doit etre proposee a ces patientes.

Aim: To assess the clinical features, treatment and outcome of oral sarcoidosis and to determine whether oral involvement is associated with a particular clinical phenotype of sarcoidosis. Design: Multicentric retrospective study. Methods: Retrospective chart review. Each patient was matched with four controls. Results: Twelve patients (9 women, 3 men) were identified. Their median age at sarcoidosis diagnosis was 38 years. Oral involvement was the first clinical evidence of sarcoidosis in seven cases and was a relapse symptom in five cases. Clinical presentations were nodules ( n = 7) or ulcers ( n = 5) and were mostly solitary. The tongue was the commonest site affected ( n = 4), followed by lips ( n = 3), oral mucosa ( n = 2), palate ( n = 2) and gingiva ( n = 1). Patients with oral sarcoidosis were significantly younger and had more frequent lacrimal or salivary glands and upper airway tract clinical involvement than the controls; increased angiotensin-converting enzyme was less frequent in oral sarcoidosis. Multiple treatments of oral sarcoidosis were used: no treatment ( n = 3), surgery ( n = 2), corticosteroids ( n = 7), hydroxychloroquine ( n = 3), methotrexate ( n = 2), doxycycline ( n = 1). Methotrexate was efficient in one patient, hydroxychloroquine showed benefit in only 1 out of 3 patients. Three patients presented oral relapses. After a mean follow-up of 6 years, 10 patients experienced a complete ( n = 7) or partial ( n = 3) remission of oral sarcoidosis; stability was observed in the remaining two cases. Conclusion: Although oral manifestations of sarcoidosis are unusual, physicians should be aware that this specific localization is frequently the first manifestation of the disease. Treatment modalities range from observation in asymptomatic patients to immunosuppressants for severe involvement.

Resume Cinq cas de pancreatite survenant au cours de l'evolution d'un lupus erythemateux dissemine sont rapportes. L'evolution de trois d'entre eux a ete fatale, de facon directement liee a la pancreatite dans deux cas. Il est generalement difficile de faire la part respective des differentes causes possibles: vascularite lupique, thrombose dans le cadre d'un syndrome des antiphospholipides, lithiase biliaire, infection, anomalies metaboliques ou traitements. La revue de la litterature permet de retrouver pres de 70 cas de pancreatite au cours de lupus. Elle etait inaugurale de la maladie dans six cas et est survenue dans 12 cas en l'absence de tout autre facteur susceptible d'etre implique dans la pathogenie de la pancreatite. Cependant, la demonstration d'une vascularite ou de thromboses pancreatiques dans le cadre d'un syndrome des antiphospholipides n'a pu etre apportee que dans de tres rares cas, meme a l'autopsie. L'evolution des pancreatites lupiques est favorable sous corticoides dans la majorite des cas. Dix-huit cas ont ete fatals. Le role de la corticotherapie dans la genese des pancreatites lupiques reste donc douteux. A notre avis, la survenue d'une pancreatite au cours du lupus ne doit pas faire arreter ou diminuer la corticotherapie, a moins que sa responsabilite dans la survenue de la pancreatite ne soit formellement etablie.

Resume Nous rapportons 2 observations associant polychrondrite atrophiante et panniculite mesenterique. Observation 1: Une femme nee en 1949 presente en fevrier 1989 une fievre a 40°C associee a de violentes douleurs abdomino-pelviennes. Huit jours plus tard apparaissent des lesions cutanees nodulaires des deux membres inferieurs. L'abdomen est meteorise et non depressible. Le scanner abdominal montre des collections peritoneales cloisonnees dont la ponction guidee ramene un liquide chyleux. La laparotomie revele une masse mesenterique de 20 cm de diametre. Les recherches bacteriologiques directes et indirectes sont negatives. L'histologie confirme l'existence d'une panniculite mesenterique en montrant une intense reaction inflammatoire avec presence de cellules geantes et de lipophages. Six mois plus tard, le diagnostic de polychondrite est pose devant des chondrites touchant le nez puis le pavillon de l'oreille. Une corticotherapie est administree pendant quelques mois. En janvier 1993, la patiente est durablement asymptomatique. Observation 2: Une femme nee en 1937 presente en octobre 1977 une polychondrite associant fievre prolongee, syndrome inflammatoire, chondrites du nez, du larynx et du pavillon de l'oreille. En decembre 1978 survient un syndrome occlusif. La laparotomie revele une infiltration de toute la racine du mesentere avec des formations nodulaires multiples. Les biopsies entrainent l'ecoulement d'un liquide puriforme. L'histologie montre un tissu adipeux partiellement necrose comportant la presence de cellules geantes et de lipophages. Un traitement corticoide a fortes doses controlera partiellement les manifestations douloureuses chondritiques et abdominales. La survenue de douleurs abdominales au cours d'une polychondrite peut relever de mecanismes multiples: complications du traitement, vascularite digestive, colite ulcereuse mais aussi panniculite mesenterique associee.

Sarcoidosis may affect functional outcome and sometimes may be a life-threatening condition. Functional outcome is particularly compromised by cutaneous, ocular, nasal, osteoarticular manifestations and some neurological expression. Central nervous system involvement and cardiac sarcoidosis could be life-threatening, as laryngeal and renal involvement. Diagnosis is sometimes very difficult to obtain. Several tissue biopsies are usually necessary to rule out all differential diagnosis. Extrathoracic manifestations of severe sarcoidosis require a corticosteroid therapy, initiated, if life-threatening at a daily dose of 1 mg/kg, usually associated with an other immunosuppressive drug (methotrexate, cyclophosphamide, anti TNFα).

The management of chronic forms of sarcoidosis can be a difficult therapeutic problem. The purpose of this observational study was to analyze the effectiveness and tolerance of infliximab in chronic sarcoidosis. This multicentre retrospective study involved 31 cases of chronic, systemic, and/or pulmonary sarcoidosis treated by infliximab. Disease had been present for 9 years and involved a mean of four organs. Patients had received several immunosuppressive drugs and 30/31 were treated with corticosteroids (19 ± 16 mg prednisone/day) with the addition in 17 cases, of one or more other immunosuppressive agents. The duration of infliximab therapy was 13 ± 12 months. A beneficial response to infliximab was observed in 62% of the cases across all organs involved: 65% for lung involvement, 67% for skin lesions and 50% for central nervous system lesions. For other organs, responses were disparate. The corticosteroid sparing effect was small (2.8 ± 9.7 mg/day). Effectiveness was more frequent in patients who were treated with additional immunosuppressive agents. Thirteen (41.9%) patients developed side effects; in seven out of 13, side effects were severe, sometimes requiring infliximab to be stopped. Our study supports the continuing interest in the use of infliximab for the treatment of chronic sarcoidosis, but also highlights the frequency and severity of side effects. Indications are difficult to specify, and currently, its use should be restricted to clinical trials.

The origins of neurosarcoidosis, a multisystemic granulomatous disease, remain unknown. Nervous system localizations remain rare, but severe. Lymphocytic meningitis, psychiatric disorders, diabetes insipidus and cranial nerve palsy are the most frequent signs. Cerebral fluid test and cervical medullar and cerebral MRI with gadolinium have to be performed first. In some cases, histological evidence of granuloma have to be obtained with neuromuscular, meningeal or cerebral biopsies. Functional impairment and life-threatening conditions require early corticosteroid therapy. In worsening cases or in the event of no therapeutic response or poor tolerance to corticosteroids, other immunosuppressive agents should be associated. Maintenance therapy and most often life long maintenance therapy allow a continuous success while avoiding relapse.

Although ocular involvement concerns about 25 to 60% of patients with systemic sarcoidosis, papilledema is a rare event.A 43-year-old man presented with left visual loss related to papillitis. Despite high-dose corticosteroids, visual loss became rapidly bilateral. Thoracic CT-scan showed mediastinal and parenchymal involvement. Histological study of lung, bronchial and lymphadenopathy biopsies displayed giant-cells granuloma without necrosis. Diagnosis of sarcoidosis complicated with optic neuropathy was made. Therapy combining prednisone, methotrexate and infliximab was started without significant improvement of visual acuity.This report underlines the potential severity of optic neuropathy in sarcoidosis.

CT is a noninvasive technique that has a major role at each stage of the management of patients with suspected or known lung cancer: in diagnosis; in staging the extension of cancer before surgery or medical treatment; in evaluating the response to the treatment, and in detecting complications [1,2].

Background Etiologies of non infectious inflammatory uveitis are multiple. Their prognosis can be severe and may lead to blindness. Anti-TNF alpha are used for several years in refractory inflammatory uveitis but few data on large studies have been reported. Objectives The aim of this study is to report on the efficacy and the safety of anti-TNF alpha in inflammatory uveitis. Methods We performed a retrospective multicenter study of main characteristics and outcomes of 162 patients with inflammatory uveitis treated with anti-TNF alpha. Results The median of age was 30 years [19-40] and with 59% of women. Uveitis were mainly bilateral (82%) and chronic (87%). Panuveitis, macular oedema and retinal vasculitis were observed in 62, 54 and 35% of cases, respectively. Main etiologies of uveitis included juvenile idiopathic arthritis (25%), Behcet9s disease (24%), spondylarthropathies (12%), and sarcoidosis (6%). Patients were treated by infliximab in 58%, adalimumab in 39.5% or etanercept in 2.5%. The median of follow up was 27 months [8-55]. Ninety three percent of patients were complete or partial responders to anti-TNF alpha therapy. No significant difference was found relative to efficacy between infliximab and adalimumab. Anti-TNF alpha had a significant corticosteroid sparing effect (daily prednisolone dose of 20mg at time of introduction of anti-TNF alpha vs 10mg and 5mg at 6 and 12 months, respectively; p Conclusions These preliminary results showed that TNF alpha inhibitors are highly efficient in severe and refractory non infectious inflammatory uveitis. Infliximab and adalimumab had the same efficacy profile but infliximab was associated with more frequent and more serious side effects. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.6013

Neurosarcoidosis is an uncommon but severe, sometimes life threatening, manifestation of sarcoidosis. Signs of neurological involvement usually are seen in patients known to have active disease. Strictly neurological forms are seen in less than 10% of cases.Neuropsychic manifestations are the most common clinical signs, independent of corticosteroid therapy or neuroendocrine involvement, epileptic seizures, and signs related to hypocephalia.Diagnosis of systemic sarcoidosis is confirmed on the basis of clinical arguments and laboratory findings favoring neurosarcoidosis. Key investigations include angiotensin converting enzyme assay in cerebrospinal fluid, and brain stem magnetic resonance imaging with gadolinium injection. Nerve biopsies may be needed in certain cases.Corticosteroid therapy is given as first line treatment with a satisfactory effect in most cases. Immunosuppressors may be added in case of failure.

To analyze the evolutive profile of sarcoidosis together with reciprocal interactions between pregnancy and sarcoidosis.All events that occurred during pregnancy in 11 women presenting with sarcoidosis were analyzed. Histological confirmation was obtained for the 11 cases. For all pregnancies were analyzed the course of both sarcoidosis and pregnancy, and the influence of pregnancy on the disease evolution.Among 33 pregnancies, 23 led to the birth of healthy fetuses (five spontaneous abortions, four voluntary abortions, and one therapeutic abortion). The major event was fetal hypotrophy in six cases. Three of them occurred during pregnancy in prednisone-treated patients with active sarcoidosis. No relapse of cured sarcoidosis or further evolution of sarcoidosis that was inactivated as of the beginning of pregnancy were observed. The course of active sarcoidosis varied, as improvement (one case), worsening (two cases) and stabilization (two cases) were observed. During the first year of follow-up after delivery, four relapses and, in two cases, preliminary signs of the disease were observed.Apart from the hypothetical but not definite risk of hypotrophy, no negative interaction between sarcoidosis and pregnancy could be established. Pregnancy does not seem to interfere with the course of sarcoidosis. Considering the risk of relapse after delivery, pregnant women presenting with sarcoidosis should benefit from clinical and radiological follow-up.

Current knowledge on central nervous system sarcoidosis.Sarcoidosis is localized in the central nervous system in 5 to 16% of the cases. Various neurological manifestations are observed, including: seizures, cognitive or psychic manifestations, hypothalamic and pituitary involvement, local pseudotumors, and hydrocephalus very frequently associated with asymptomatic lymphocytic meningitis and with cranial nerve palsy, particularly palsy of the seventh nerve, occurring less regularly. CNS localization is most often an early manifestation of the disease, unmasking sarcoidosis. It is often part of primary or secondary systemic polyvisceral sarcoidosis. The diagnosis is mainly based on two arguments: confirmation of the existence of systemic sarcoidosis and clinical and paraclinical compatibility of neurological abnormalities (particularly at magnetic resonance imaging [MRI]). Neurological histopathology is rarely necessary to confirm the diagnosis. Corticotherapy is indicated in all symptomatic cases and most often leads to a more or less complete clinical response evidenced by regression of active lesions identified on MRI. The treatment must often be prolonged for several years, and clinical and MRI evolution help guide therapeutical choices for dosages and threshold doses. CNS involvement is potentially severe with mortality and morbidity rates that are not insignificant. Limiting of iatrogenic risks requires adequate follow-up.Multicenter studies are necessary to determine factors influencing the incidence and long-term prognosis of CNS sarcoidosis treated with corticotherapy. The efficacy of treatments other than corticotherapy must be evaluated.

Magnetic resonance angiography (MRA) has become a widely accepted technique with regards to the other available noninvasive techniques in the diagnosis of vascular disease. This paper proposes a review of the different indications of this technique in arterial and venous diseases. Among several MRA pulse sequences, the most frequently used until today consisted of a time-of-flight technique which provided angiograms without any injection of intravascular contrast medium. It required to be performed in a plane perpendicular to the main axis of the vessel to be optimal. New techniques, such as contrast medium bolus-enhanced acquisitions allow examination of vascular segments in a plane parallel to their course (coronal for the aorta and lower limb arteries). An increasing number of clinical applications has raised since the implementation of MRA techniques on MR devices; some of them are widely accepted, whereas some others remain under the scope of extensive validation. With a high level of accuracy in grading carotid artery stenosis, MRA is now routinely used in cerebral arterial occlusive diseases and has in part replaced contrast angiography. MRA of the venous system of the brain plays a major role in the diagnosis and follow up of dural venous thrombosis. Other vascular brain diseases, such as vascular malformations, yet have limited uses. Carotid artery dissections are fairly demonstrated with MRA, which can be used for diagnosis as well as for follow-up. The accuracy of MRA in the diagnosis of venous thrombosis of the cervical/mediastinal veins has been reported as high as 100%. Moreover, MRA allows a precise assessment of collateral vessels in case of complete cervical/mediastinal venous thrombosis.

Primary tumors of the heart are very rare [6], the majority being benign. The least rare forms are myxomas (30%) and lipomas (10%) [21]. For this type of pathology, transthoracic and trans-esophageal ultrasound are the examinations of choice [11] and may be complemented by CT and particularly MRI.

This study illustrated 10 cases of sarcoidosis symptomatic cardiomyopathy. All sarcoidosis were confirmed by histology. One was revealed by cardiological signs. Extra-cardiologic manifestations were non specific, as well cardiological and electrical signs. ECG were abnormal in only 70% of cases. Echocardiography and thallium scintigraphy with dipyramidole test were always abnormal. No sudden death appeared in our series. As opposed to all cases in literature, all patients, but one, responded positively to corticotherapy. These results can be explained by high doses of prednisolone associated, when non effective, to another immunosuppressive agent.