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Resume Introduction Les hematomes surrenaliens (HS) non traumatiques sont rares et de prise en charge non consensuelle. Nous proposons d’en etudier les caracteristiques a partir d’une serie retrospective recente. Patients et methodes Les patients ont ete identifies dans la base de donnees du Programme de medicalisation des systemes d’information et apres appel a observations au sein des hospices civils de Lyon. Les patients adultes dont le dossier medical etait complet et les examens d’imagerie confirmaient le diagnostic etaient inclus. Resultats Entre 2000 et 2017, 20 patients dont 15 femmes ont ete identifies. La presentation clinique etait aspecifique. Il s’agissait dans cinq cas d’HS survenant en cours de grossesse, dont quatre unilateraux droits. Les causes des 15 autres cas (11 atteintes bilaterales) se repartissaient ainsi : syndrome des antiphospholipides (n = 8), thrombopenie induite par l’heparine (n = 4), thrombocytemie essentielle (n = 3), accident hemorragique sous traitement anticoagulant (n = 1), complication chirurgicale (n = 3) et sepsis (n = 3). Sept cas combinaient 2 causes potentielles. Le diagnostic etait retenu du fait d’un aspect compatible a l’imagerie des surrenales. Seize patients recevaient un traitement anticoagulant. Dix des 11 patients ayant presente un HS bilateral etaient traites par opotherapie substitutive au long cours. Un deces est survenu dans le cadre d’un syndrome catastrophique des antiphospholipides. Conclusion La presentation clinique des HS est heterogene et aspecifique. Le diagnostic repose sur l’imagerie des surrenales. La recherche d’une thrombophilie acquise est essentielle et nous retrouvons dans ce travail des donnees etiologiques comparables aux principales series de la litterature. L’insuffisance surrenalienne est le plus souvent definitive en cas d’atteinte bilaterale.

To determine the ophthalmological and extra-ophthalmological clinical characteristics and visual prognosis of patients with sarcoid uveitis in different ethnic groups. We retrospectively analysed the data from patients with sarcoid uveitis seen at two departments of Ophthalmology between December 2003 and December 2017. Patients presented biopsy-proven sarcoidosis and/or presumed sarcoid uveitis based on the following criteria: compatible thoracic imaging, associated with elevated angiotensin-conversion enzyme (ACE) and/or lymphocytic alveolitis on bronchoalveolar lavage fluid analysis (> 15% lymphocytes and CD4/CD8 > 3.5). Ophthalmological and general characteristics, as well as visual and global prognoses, were compared in three pre-defined ethnic groups: White Europeans, North Africans and Afro-Caribbeans. A total of 194 patients were included: 145 with biopsy-proven and 49 with presumed sarcoid uveitis. Overall, 68% were White Europeans while 20.6% were North Africans and 11.3% were Afro-Caribbeans. Sixty-nine per cent were women and the median age at presentation was 52.1 years. Median ages at first ocular manifestation of the disease in Afro-Caribbeans and North Africans were respectively 34.3 and 43.1 years, while it was 57.8 years in White Europeans (p

AimTo assess the usefulness of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET/CT) and the predictive factors for the diagnosis of sarcoidosis in patients with uveitis who have normal thoracic tomography.MethodsWe retrospectively reviewed 67 consecutive patients with uveitis of unknown aetiology or a suspected sarcoidosis. All patients with normal thoracic tomography underwent an 18F-FDG PET/CT, which was blindly reinterpreted. We then assessed the proportion of positive 18F-FDG PET/CT and the impact on the final aetiology, using Abad’s criteria for the diagnosis of intraocular sarcoidosis.Results19 of the 67 patients (28.4%) had mediastinal hypermetabolic foci on their 18F-FDG PET/CT consistent with sarcoidosis. It identified a biopsy site in two cases, which were consistent with sarcoidosis. At the end of the study, six patients (10%) had a proven sarcoidosis, six patients (9%) were considered as having a presumed sarcoidosis and 18 patients (26.9%) as having indeterminate sarcoidosis. 18F-FDG PET/CT enabled the diagnosis of presumed sarcoidosis in these six patients. An older age at diagnosis (p=0.004) and the presence of synechiae (p=0.02) were significantly related to an abnormal 18F-FDG PET/CT, with a trend for an elevated ACE (p=0.0993). We established a nomogram to estimate the probability of having positive findings on the 18F-FDG PET/CT according to different predictive factors.Conclusion18F-FDG PET/CT enabled the diagnosis of intraocular sarcoidosis even in patients with a normal CT scan. Older age at diagnosis, presence of synechiae and elevated ACE are associated with positive findings on 18F-FDG PET/CT consistent with sarcoidosis.

Purpose: Despite the huge advance in diagnostic technics, about one-third of uveitis is still considered of unknown etiology. In this study, we aimed to report their clinical features and to descri...

Interleukin-1 is a major cytokine of innate immunity and inflammation. It exerts various systemic effects during the inflammatory response, such as fever induction, thrombopoiesis and granulopoiesis, or leukocyte recruitment. Its involvement has been demonstrated in many inflammatory-mediated diseases, such as diabetes or gout. Moreover, interleukin-1 plays a pivotal role in some autoinflammatory diseases, such as cryopyrinopathies or familial Mediterranean fever. In these diseases, a constitutional defect of the inflammasome, a protein complex responsible for the activation of interleukin-1, explains the hypersecretion of interleukin-1. Other autoinflammatory diseases have a more complex pathophysiology involving deregulation of the interleukin-1 pathway, upstream or downstream of the inflammasome, or through more complex mechanisms. In this review, we are detailing the synthesis, the activation, the signalling, and the regulation of interleukin-1. We then describe the autoinflammatory diseases or related-diseases where the pathological role of interleukin-1 has been demonstrated.

International audience; [u]PURPOSE:[/u] ULISSE is the only study that prospectively assessed the efficiency of a standardized strategy, compared to an open strategy for the etiologic diagnosis of uveitis. Our aim was to evaluate the diagnostic yield of the tests prescribed in the ULISSE study to clarify their relevance. [u]METHODS:[/u] ULISSE is a non-inferiority, prospective, multicenter and cluster randomized study. The standardized strategy is a two-steps strategy: in the first step, common standard tests were performed, and in the second step, tests were guided by the clinical and anatomic type of uveitis. We reported the relevance of the diagnostic tests used in the standardized strategy, as well as the profitability of the tests that were prescribed to more than twenty patients in each group. Based on diagnostic criteria, either an ophthalmologist, or an internist, established the profitability of a test by considering whether the test lead to a diagnosis or not. [u]RESULTS:[/u] Among the 676 patients included (standardized 303; open 373), a diagnosis was made for 152 (50.4%) in the standardized group and 203 (54.4%) in the open group. The most common entities were HLA-B27 associated uveitis (22%), spondyloarthritis (11%), sarcoidosis (18%), tuberculosis (10.7%) and herpes virus infections (8.5%). Among the first step's systematic tests, tuberculin skin test was the most contributive investigation (17.1%), followed by chest X-ray (8.4%), C reactive protein and ESR (6.6% and 5.1%), complete blood count (2.2%) and VDRL (2.0%). The second step's most often contributive tests were: HLA B27 (56.3%), chest-CT (30.3%) and angiotensin converting enzyme (ACE) (16.5%). HLA B27 and ACE were significantly more contributive in the standardized group than in the open group. Immunological tests were never contributive. Among the free investigations, or among the investigations guided by clinical or paraclinical findings, the most often contributive tests were: Quantiferon(R) (24%), electrophoresis of serum protein (7.8%) and sacroiliac imagery (46.4%). Intracellular serologies (1.7%), serum calcium (2.1%) and hepatic tests (3.3%) were exceptionally contributive. Among the third intention tests, labial salivary gland biopsies were contributive in 17.9% of cases, but the profitability of other invasive investigations (anterior chamber tap, vitrectomy, bronchoscopy and lumbar puncture) or specialized imagery (18F-FDG PET, Brain MRI) could not be determined since these test were rarely performed. [u]CONCLUSION:[/u] Only a few diagnostic tests are useful for the etiological assessment of uveitis. They are often cheap, simple, more often guided by the clinical findings, and lead to an etiological diagnosis in most patients. On the other hand, some tests are never or exceptionally contributive, such as immunological tests or intracellular serologies. Further studies are required to evaluate the profitability of third intention imagery and invasive investigations.

The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cytopenias, autoimmune hepatitis, and bullous skin diseases (pemphigus, lichen) have been reported. Autoimmune diseases occur most often before thymectomy, but they can be discovered at the time of surgery or later. Two situations require the systematic investigation of a thymoma: the occurrence of myasthenia gravis or autoimmune erythroblastopenia. Nevertheless, the late onset of systemic lupus erythematosus or the association of several autoimmune manifestations should lead to look for a thymoma. Neither the characteristics of the patients nor the pathological data can predict the occurrence of an autoimmune disease after thymectomy. Thus, thymectomy usefulness in the course of the autoimmune disease, except myasthenia gravis, has not been demonstrated. This seems to indicate the preponderant role of self-reactive T lymphocytes distributed in the peripheral immune system prior to surgery. Given the high infectious morbidity in patients with thymoma, immunoglobulin replacement therapy should be considered in patients with hypogammaglobulinemia who receive immunosuppressive therapy, even in the absence of prior infection.

AimsTo determine the frequency and clinical presentation of Lyme disease in patients with uveitis and to assess the value ofBorrelia burgdorferiserological testing.MethodsRetrospective study on all patients with uveitis who were referred to our tertiary hospital were serologically tested for Lyme in our laboratory between 2003 and 2016. Screening consisted of determiningB. burgdorferiserum IgG and IgM by ELISA method. The patient’s serology was considered as positive if the ELISA-positive result in IgM and/or IgG was confirmed by an immunoblot positive in IgM and/or IgG. Lyme-associated uveitis was diagnosed based on serological results as well as response to antibiotics and exclusion of other diagnosis.ResultsOf the 430 patients with uveitis (60% women, mean age 49 years) fulfilling inclusion criteria, 63 (14.7%) had an ELISA-positive serology, confirmed by immunoblot for 34 patients (7.9%). The diagnosis of Lyme-associated uveitis was finally retained in seven patients (1.6%). These patients reported either a previous exposure including tick bite or forest walks (n=5), symptoms suggestive of Lyme disease (n=5) and resistance to local and/or systemic steroids (n=7). Among the remaining 27 positive patients, 22 had other established aetiologies and 5 other were unclassified.ConclusionThe seroprevalence ofB. burgdorferiamong our patients with uveitis was 7.9% compared with 6 to 8.5% in the general French population which leads to a low predictive value of serological testing. Its use should be reserved for patients with unexplained uveitis, an exposure history, systemic findings suggestive of Lyme disease and steroids resistance.

Resume Les infections opportunistes (IO) sont rares en cas de sarcoidose (1 a 10 %) et surviennent le plus souvent au cours de sarcoidose connue ou, plus rarement, la revelent. Les IO les plus frequentes sont par ordre decroissant : l’aspergillose pulmonaire, la cryptococcose et les infections mycobacteriennes. La corticotherapie systemique represente le facteur de risque le plus frequent d’IO au cours de la sarcoidose mais les immunosuppresseurs et les anti-TNFα peuvent aussi etre en cause. Le plus souvent, la presentation clinique, le traitement et le devenir des patients ne different pas des autres conditions favorisant la survenue d’une IO. Neanmoins, certaines presentations atypiques d’IO peuvent mimer une exacerbation de la sarcoidose et la confusion risque de conduire le clinicien a une intensification de l’immunosuppression, entrainant l’aggravation de l’IO. Le recueil minutieux des antecedents ainsi que la connaissance des indices permettant de differencier une IO d’une exacerbation de la sarcoidose sont donc des facteurs cles dans la prise en charge de ces patients.

Resume Introduction Plusieurs cas cliniques ont rapporte le benefice des immunoglobulines intraveineuses (IgIV) au cours du lupus. Observations Nous decrivons ici deux cas de myocardite lupique traites par IgIV a dose immunomodulatrice. Le premier concerne une patiente de 42 ans qui presentait une myocardite lupique resistante au traitement par corticoides et cyclophosphamide. Les IgIV a la dose de 2 g/kg ont permis une amelioration clinique en quelques jours. Le second cas concerne une patiente de 43 ans qui etait traitee par IgIV a la dose de 2 g/kg suite au diagnostic de myocardite et chez qui les immunosuppresseurs etaient contre-indiques du fait du recent contexte d’endocardite infectieuse. Une amelioration clinique etait observee avec une augmentation de la fraction d’ejection du ventricule gauche de 20 % a 60 % en quelques jours. Nous rapportons egalement neuf observations similaires issues de la litterature. Conclusion L’utilisation des IgIV dans la myocardite lupique n’est pas reconnue officiellement mais pourrait etre une alternative lorsque les therapies conventionnelles ont echoue ou sont contre-indiquees.

Background: Clinical presentation of Lyme-associated uveitis is poorly described. We reported here a case series of seven patients with uveitis related to Lyme disease and a review of the literature. Methods: A retrospective study in our university hospital between 1 May 2003 and 31 July 2016 on 1006 uveitis patients and review of Pubmed library. Results: Seven patients (71.4% male, mean age = 53 (38–70)) were diagnosed with a Lyme-associated uveitis. All anatomical types of uveitis were found (four intermediate, three anterior, and three posterior uveitis); most were unilateral (n = 6; 85.7%), one granulomatous and two with synechiae. Peripheral retinal vasculitis was present in four patients. They all had a risk of exposure or extra-ophthalmological symptoms. Antibiotic and steroid treatment was rapidly effective in all patients. Four patients presented recurrences of uveitis, of whom two received a second antibiotic treatment, which is quite common in literature. Persistent or recurrence of symptoms can be explained by three hypotheses: (1) reinfection, (2) relapse of original infection, and (3) autoimmune reaction. Conclusion: Lyme-associated uveitis appears varied. Hyalitis and involvement of the posterior segment and retinal vasculitis seem to be rather frequent. Its prognosis is mainly good, even if inflammation can be resistant or recurring.

Resume Introduction La microangiopathie thrombotique induite par la gemcitabine est un evenement rare dont le traitement n’est pas consensuel. L’eculizumab pourrait avoir un interet therapeutique. Observation Une femme de 68 ans recevait une chimiotherapie adjuvante par gemcitabine pour un adenocarcinome pancreatique. A 2 mois de traitement, elle developpait une anemie hemolytique mecanique, une thrombopenie et une hypertension arterielle menant au diagnostic de microangiopathie thrombotique. La gemcitabine etait arretee. Des echanges plasmatiques etaient inities secondairement du fait de l’aggravation hematologique et nephrologique. L’efficacite etant insuffisante, l’eculizumab etait debute a la dose de 900 mg/semaine 4 fois, puis 1200 mg tous les 14 jours. Apres 7 perfusions, la pression arterielle, le syndrome œdemateux, l’anemie et l’insuffisance renale se corrigeaient. Conclusion L’eculizumab peut etre propose pour le traitement de la microangiopathie thrombotique induite par la gemcitabine, en cas d’atteinte hematologique ou renale grave et de reponse insuffisante aux echanges plasmatiques.

International audience; PURPOSE: To report the outcomes of initial methylprednisolone pulse then oral prednisone in the treatment of birdshot chorioretinopathy (BSCR). METHODS: We retrospectively reviewed medical records of 21 BSCR patients, treated with initial methylprednisolone, prednisone, or immunosuppressive therapy. Main outcome measures were changes in visual acuity, intraocular inflammation, concomitant immunosuppressive drug or intravitreal steroid, and adverse events. RESULTS: In total, 14 patients were initially treated with methylprednisolone; four patients with prednisone; and three with immunosuppressive drug. Intraocular inflammation was controlled in 10 of 14 patients (71%) at 1 year, with a mean dose of 6.2 mg/day prednisone. Patients treated initially by methylprednisolone had improved visual acuity at 1 year, but it was not significantly different from patients treated initially by prednisone. CONCLUSIONS: Corticosteroid therapy, including initial methylprednisolone pulse then prednisone, was effective in stabilizing vision and decreasing inflammation in most patients with BSCR

BackgroundUveitis is a frequent and early feature of sarcoidosis. As BTNL2 (butyrophilin-like 2) gene polymorphism was found linked with the susceptibility to sarcoidosis, we investigated whether a specific genotype of BTNL2 gene G16071A (or rs2076530) single-nucleotide polymorphism (SNP) would be associated with the risk of sarcoid uveitis in all patient subgroups.MethodsThe study compared the genotype frequencies of SNP G16071A of 135 patients with sarcoid uveitis (Sa+Uv+) with those of 196 patients with sarcoidosis without uveitis (Sa+Uv−), 81 patients with uveitis without sarcoidosis (Sa−Uv+), and 271 controls with no sarcoidosis nor uveitis (Sa−Uv−). Three hypothetical subgroups of patients with sarcoid uveitis (Sa+Uv+ cases) were considered: (1) subgroup I: patients aged 45 years; and (3) subgroup III: all other patients.ResultsA statistically significant difference in genotype frequencies was found between the groups Sa+Uv− and Sa−Uv− (p=3.2×10−6) and between the groups Sa+Uv+ and Sa+Uv− (p=7.1×10−3). There was no difference between the three subgroups of Sa+Uv+ patients. There was a statistically significant difference in genotype frequencies between Sa+Uv− and Sa+Uv+ subgroup II (p=0.005) but no difference between Sa+Uv− and Sa+Uv+ subgroup I.ConclusionNo association was found between G16071A and the susceptibility to sarcoid uveitis. BTNL2 gene G16071A SNP seems to be a predisposing factor for sarcoidosis except in Caucasian postmenopausal women with sarcoid uveitis in whom the GG genotype prevails. These and future results will help in understanding differences between particular subgroups of patients with sarcoid uveitis.

Purpose. To describe patients with new onset sarcoid uveitis occurring after an ophthalmic procedure and compare them with patients with sarcoid uveitis without ocular procedure. Methods. Retrospective analysis of case records from patients with postophthalmic procedure sarcoid uveitis seen at our institution between April 2004 and October 2016. Patients with a previous history of uveitis were not included. Each patient was randomly matched with four controls from our incident cohort of new onset sarcoid uveitis without ophthalmic procedure. Results. We identified 11 patients (8.5%) from our incident cohort of sarcoid uveitis (n=130), who were all women, with a postophthalmic procedure uveitis (mostly after cataract surgery (36%)). These patients were older (69.7 vs 52.7 years) and presented more synechiae than controls. After a mean follow-up of 30 (3–60) months, there was no significant difference between the postprocedure and the control group with regard to demography, clinical presentation, disease course, treatment, and outcome. Conclusions. Sarcoid uveitis has similar characteristics in patients with new onset sarcoid uveitis after or without ophthalmic procedure. As a consequence, ophthalmic intervention should be seen as a potential trigger of latent sarcoidosis.

Introduction Cette etude avait pour objectif d’etudier les facteurs associes a la fatigue chez des patients atteints de lupus erythemateux systemique (LES) francais a l’aide de 3 questionnaires de fatigue differents. Patients et methodes Nous avons conduit une etude de cohorte prospective multicentrique et recrute des patients dans 10 hopitaux francais entre 2010 et 2014. A partir des donnees recueillies lors du suivi de 336 patients nous avons analyse les donnees de 3 questionnaires auto-administres de fatigue : le domaine fatigue du questionnaire specifique LupusQoL, le domaine vitalite du questionnaire generique SF-36 et le questionnaire generique de fatigue MFI-20. Les donnees demographiques, socioprofessionnelles et cliniques en rapport avec le LES etaient recueillies a l’inclusion. La precarite etait mesuree par le questionnaire EPICES et la depression par le questionnaire CES-D. Les poussees du lupus etaient mesurees par le SELENA-SLEDAI Flare Index-Revised (SFI-R). Les analyses multivariees, sans et avec ajout du facteur depression, ont porte sur les variables significatives en analyse bivariee. Les scores de fatigue utilises variaient de 100 (pas de fatigue) a 0 (plus forte fatigue imaginable) pour les 2 premiers questionnaires et inversement pour le questionnaire MFI-20. Resultats Trois-cent trente-six patients ont ete inclus dont 302 (90 %) femmes. L’âge moyen ± DS (deviation standard) etait de 41 ± 12 ans, la duree d’evolution du LES de 12 ± 9 ans, l’IMC etait de 24,4 ± 5,1 kg/m2. 114 (34 %) patients avaient un niveau d’etude inferieur au baccalaureat, 103 (31 %) etaient sans emploi et 64 (19 %) etaient en situation de precarite selon le score EPICES. Selon le score CES-D, 99 (29 %) patients presentaient un etat depressif avere. Le score de sequelles SLICC/ACR-DI (SDI) etait de 1 ± 1. Les scores de fatigue moyens etaient a 66 ± 25 pour le LupusQoL et 46 ± 21 pour le SF-36. Concernant les domaines du MFI-20, la fatigue etait mesuree a 50 ± 26 pour le domaine fatigue generale/physique (GPF), 35 ± 26 pour le domaine fatigue mentale (MF), 31 ± 28 pour le domaine reduction des activites (RA) et 27 ± 27 pour le domaine reduction de la motivation (RM) du MFI-20. Les facteurs associes a une fatigue plus importante en analyse bivariee et dans les 6 domaines des questionnaires utilises etaient : la precarite, le niveau d’etude bas, le statut sans emploi et un etat depressif avere. Les autres variables significativement associees a des scores de fatigue eleves selon le questionnaire utilise etaient : le sexe feminin, l’âge ≥ 40 ans, le tabagisme actif, l’obesite, une poussee clinique osteoarticulaire, neurologique ou avec des signes generaux, le SDI ≥ 1 et la prise de corticoides ≥ 10 mg/j. L’analyse multivariee, sans inclusion de la depression identifiait la precarite comme facteur significativement associe a la fatigue dans les 6 questionnaires utilises. Les autres facteurs significativement associes aux scores eleves de fatigue etaient le sexe feminin, l’absence d’emploi, l’obesite et les poussees dans les systemes osteoarticulaire et neuropsychiatrique du SFI-R. L’analyse multivariee, en incluant la depression retrouvait celle-ci comme facteur significativement associe a la fatigue dans tous les questionnaires. La precarite n’etait plus significativement associee a la fatigue que dans 2 questionnaires. L’absence d’emploi, l’obesite, les poussees dans les systemes osteoarticulaire et neuropsychiatrique du SFI-R restaient significativement associes a la fatigue selon le questionnaire utilise mais il existait des differences entre les 2 analyses en nombre de questionnaires significativement associes a la fatigue. Conclusion Les facteurs associes a la fatigue dans le LES sont multiples et sujets a de grandes variations selon les questionnaires et la methodologie employee, comme en temoigne la litterature. L’utilisation de questionnaires de fatigue multidimensionnels semble permettre une analyse plus fine de ces facteurs dans le cadre d’etudes epidemiologiques, mais le choix du questionnaire doit etre guide par les objectifs de l’etude. Dans le cadre de notre travail les facteurs associes a la fatigue dans le LES sont sociodemographiques : le sexe feminin ; socioprofessionnels : la precarite et l’absence d’emploi ; certaines comorbidites : la depression et l’obesite ; des facteurs specifiques du LES : les poussees osteoarticulaire et neuropsychiatrique selon le SFI-R. L’effet de la precarite sur la fatigue pourrait etre due a la plus forte intensite des symptomes depressifs chez les patients precaires. La mise en place de strategies interventionnelles visant a corriger les facteurs identifies comme etant associee a la fatigue chez les patients atteints de LES et l’evaluation de leur efficacite devrait etre l’objectif de prochaines etudes.

Introduction Les infarctus des surrenales sont une pathologie rare dont le diagnostic est souvent retarde en raison d’une presentation clinique trompeuse. L’objectif de cette etude est de decrire les aspects epidemiologiques, cliniques, radiologiques, la distribution etiologique et l’evolution de cette pathologie. Patients et methodes Etude observationnelle, descriptive, retrospective realisee au centre hospitalo-universitaire de Lyon (Hospices Civils de Lyon). Les observations ont ete identifiees a partir des donnees du PMSI (mots-cles : infarctus surrenal, infarcissement, necrose, hemorragie des surrenales, maladies thromboemboliques profondes) entre janvier 2000 et decembre 2017 et par un appel a observations aupres des medecins des services de medecine interne et d’obstetrique. Les donnees cliniques, etiologiques, paracliniques (biologie, imagerie) et l’evolution de chaque patient ont ete analysees. Resultats Parmi 357 dossiers analyses, 20 patients ont ete identifies comme ayant eu un infarctus surrenalien. L’âge median des patients etait de 47 ans avec une predominance feminine (75 %). Les cadres etiologiques etaient les suivants : syndrome catastrophique des antiphospholipides (SCAPL, n = 6, 30 %), grossesse (n = 5, 25 %), sepsis (n = 4, 20 %), thrombopenie immunoallergique induite par l’heparine (TIH, n = 4, 20 %), thrombocytemie essentielle (TE, n = 3, 15 %) ou un contexte chirurgical (n = 3, 15 %). Neuf patients combinaient plusieurs facteurs etiologiques. L’atteinte etait unilaterale dans 9 cas (45 %). Dans les 11 cas d’atteinte bilaterale, une hemorragie surrenalienne etait associee. Sur le plan clinique, 12 patients presentaient une douleur abdominale de survenue brutale, 1 douleur pelvienne et 4 un choc hemodynamique ; dans 3 cas, la presentation clinique n’est pas renseignee. Biologiquement, un syndrome inflammatoire etait observe chez 17 patients (85 %), associe a une hyponatremie chez 4 patients dont l’atteinte etait bilaterale. Le diagnostic etait etabli grâce a la tomodensitometrie abdominopelvienne chez 16 patients (80 %) ou l’echographie abdominale (n = 6). Dans les 5 cas survenant au cours d’une grossesse, l’infarctus etait unilateral, survenait au cours du 3eme trimestre dans 4 cas et interessait la surrenale droite dans 4 cas. Le SCAPL etait revelateur du syndrome des antiphospholipides dans 2 cas et associe a une maladie lupique dans 2 cas. Quatorze patients ont recu un traitement anticoagulant a dose curative, 1 patient atteint de TE recevait un traitement antiagregant plaquettaire ; pour 5 patients, les donnees n’etaient pas precisees. Neuf patients ont recu une opotherapie substitutive par hydrocortisone. Sur le plan evolutif, aucune recidive n’a ete rapportee chez 19 patients ; 1 deces est survenu dans le cadre d’un SCAPL. Dix patients ont conserve une insuffisance surrenalienne chronique sequellaire. Une patiente a mene une grossesse ulterieure sans recidive. Conclusion Les infarctus surrenaliens sont des pathologies rares. Le diagnostic et la frequence de l’infarctus ou des hemorragies des surrenales restent difficilement evaluables car, en dehors du syndrome des antiphospholipides [1] , leur description provient le plus souvent de cas cliniques isoles. Notre etude rapportent 4 etiologies principales : le SCAPL, la TIH, la TE et la grossesse. Le tableau clinique et biologique est trompeur et polymorphe. Il doit etre evoque face a tout patient a risque thromboembolique, en particulier au cours du SAPL et chez la femme enceinte, presentant des douleurs du flanc d’apparition brutale, apres avoir ecarte une lithiase urinaire ou une pyelonephrite. La tomodensitometrie avec injection de produit de contraste permet d’etablir un diagnostic precoce. Le traitement de reference reste l’anticoagulation curative, sans consensus sur la duree. Le pronostic fonctionnel et vital est engage, notamment en cas d’atteinte bilaterale. La majorite des patients presentant un SAPL developpe une atrophie et une insuffisance des surrenales irreversibles [1] .

Aim: To assess the clinical features and outcome of lymphoma when associated with sarcoidosis and to determine whether this association gives lymphoma a better prognosis. Design : Multicentre retrospective cohort study. Methods : Retrospective chart review. Results: Twenty-one patients were included (9 males, 12 females). Median age at sarcoidosis diagnosis was 48 years (range: 24–68 years). In 14 cases, lymphoma occurred within a previously known sarcoidosis. Five patients received a concomitant diagnosis of sarcoidosis and lymphoma, whereas lymphoma preceded sarcoidosis in two patients. Three patients were diagnosed with Hodgkin’s lymphoma and 18 patients with non-Hodgkin’s lymphoma (diffuse large B-cell lymphoma (DLBCL) ( n = 11), follicular lymphoma ( n = 2), chronic lymphocytic leukemia/small lymphocytic lymphoma ( n = 2), anaplastic large cell lymphoma ALK + ( n = 1), angioimmunoblastic T-cell lymphoma ( n = 1) and T-cell prolymphocytic leukemia ( n = 1)). Thirteen patients were alive and in complete remission. Median age at the time of diagnosis of sarcoidosis was lower in patients with concomitant lymphoma compared with patients with sarcoidosis preceding lymphoma (34 years vs. 51 years, P = 0.01). Patients presenting with DLBCL associated with sarcoidosis were compared with DLBCL without sarcoidosis. No statistical difference was found in the risk of death or progression between the two groups ( P = 0.685). Conclusions: We report here the largest series of lymphoma associated sarcoidosis patients. As opposed to previous studies, we observed a predominance of patients with DLBCL. Our study confirms the concept of the sarcoidosis-lymphoma syndrome. Large B-cell lymphoma does not have a better prognosis when associated with sarcoidosis.

Sarcoidosis is an inflammatory disease marked by a paradoxical immune status. The anergic state, which results from various immune defects, contrasts with the inflammatory formation of granulomas. Sarcoidosis patients may be at risk for opportunistic infections (OIs) and a substantial number of cases have been reported, even in untreated sarcoidosis. It is not clear how OIs in patients with sarcoidosis are different from other groups at risk. In this review, we discuss the most common OIs: mycobacterial infection (including tuberculosis), cryptococcosis, progressive multifocal leukoencephalopathy, and aspergillosis. Unlike peripheral lymphocytopenia, corticosteroids are a major risk factor for OIs but the occurrence of Ols in untreated patients suggests more complex predisposing mechanisms. Opportunistic infections presenting with extrapulmonary features are often misdiagnosed as new localizations of sarcoidosis. Aspergillomas mostly develop on fibrocystic lungs. Overall, physicians should be aware of the possible occurrence of OIs during sarcoidosis, even in untreated patients.

Resume Propos L’incidence des anemies hemolytiques immunologiques medicamenteuses est estimee a un cas par million d’habitants. Elles peuvent etre fatales. L’objectif de ce travail est de decrire leurs caracteristiques et les medicaments actuellement en cause. Methodes Etude retrospective a partir des donnees du centre de pharmacovigilance et du laboratoire d’immuno-hematologie de l’etablissement francais du sang de Lyon entre 2000 et 2012. Les criteres d’inclusion etaient : (1) une anemie (hemoglobine inferieure a 100 g/L) ; (2) hemolytique ; (3) immunologique (test de Coombs direct positif) ; (4) avec une imputabilite au moins plausible selon la methode francaise d’imputabilite ou avec la mise en evidence d’anticorps anti-medicaments ; (5) l’exclusion des autres causes d’hemolyse. Resultats Dix observations (5 hommes et 5 femmes, d’âge median 54,4 ans) ont ete identifiees. Les medicaments incrimines etaient les suivants : ambroxol, cefotetan, ceftriaxone, interferon-beta, loratadine, oxacilline, oxaliplatine, piperacilline-tazobactam, pristinamycine et quinine. Le delai moyen d’apparition de l’anemie etait de 6 jours (2 heures a 16 jours). Le nadir moyen de l’hemoglobine etait de 57,9 g/L (34–78). Le test de Coombs direct etait positif chez 8 patients a IgG seul ( n = 4), IgG et complement ( n = 3) et IgA ( n = 1). L’anemie hemolytique immunologique medicamenteuse etait averee (presence d’anticorps immuno-allergiques) chez 5 patients, probable par mecanisme immuno-allergique (imputabilite intrinseque plausible) chez 4 et probable par mecanisme auto-immun chez 1. Conclusion Le diagnostic d’anemie hemolytique immunologique medicamenteuse est difficile en raison d’une similitude avec les anemies hemolytiques auto-immunes et de la sensibilite variable des tests immunologiques qu’ils convient de repeter.

Summary Purpose Despite recent diagnostic advances, a fever of unknown origin (FUO) remains a clinical challenge. Methods This study reports the clinical courses and outcomes of 103 cases of FUO and evaluates the places of 18F-Fluorodeoxyglucose positrons emission tomography (18FDG-PET) and molecular biology in the diagnostic approach. Results This retrospective study was carried out from 2002 to 2012 in two departments of internal medicine. The diagnosis of FUO was based on the updated criteria of Durack and Street. It included 54 men and 49 women (mean age: 57 years) in 52 of whom the final diagnosis could not be established. Among the 51 patients with final diagnosis, non-infectious inflammatory disorders were the most prevalent (61%). The other diagnoses were infectious diseases (23.5%), miscellaneous causes (10%) and malignancies (6%). 18FDG-PET was performed in 48 patients and was contributory in 10. Molecular biology techniques were performed in 28 patients and were contributory in only one case: detection of a cytomegalovirus infection by polymerase-chain-reaction assay. At study closure, eleven patients had died, of whom five died from the disease that caused the FUO. Only two deaths among the 52 patients without diagnosis could be attributed to the feverish illness. Conclusion As observed in the most recent case series, the rate of undiagnosed patients is increasing. The prognosis was good for undiagnosed FUO. Here, the yield of 18FDG-PET was 21% but that of molecular biology negligible. The recourse to molecular biology seems useless unless directed by a high degree of clinical suspicion.

Resume Propos Rapporter et analyser les differentes presentations cliniques, les diagnostics etiologiques, le pronostic et le traitement des patients atteints de sclerites. Methodes Etude retrospective, monocentrique a partir d’une serie de 32 patients adresses pour le diagnostic etiologique d’une sclerite dans un centre tertiaire. Resultats L’âge moyen au diagnostic de sclerite etait de 46,8 ans (extreme : 22 a 77 ans). Il s’agissait de 19 femmes et 13 hommes. Vingt-six patients (81 %) avaient une atteinte anterieure (15 nodulaires, 8 diffuses et 3 necrosantes), six (19 %) avaient une atteinte posterieure. La sclerite etait unilaterale chez 24 patients (75 %). Le bilan revelait une maladie systemique chez 12 patients (37,5 %) : granulomatose avec polyangeite ( n = 3), maladie de Behcet probable ( n = 2), rhumatisme inflammatoire indetermine ( n = 2), rhumatisme psoriasique ( n = 1), spondylarthrite ankylosante ( n = 1), sarcoidose ( n = 1), syndrome de Cogan ( n = 1) et rectocolite hemorragique ( n = 1). Une origine herpetique etait retenue chez 6 patients (18,8 %) devant un contexte clinique evocateur et l’efficacite du traitement d’epreuve par valaciclovir. Vingt-huit patients (87,5 %) ont recu un traitement systemique associe a un traitement topique. La premiere ligne therapeutique a comporte des anti-inflammatoires non steroidiens chez 15 patients (47 %) et des corticoides chez 8 patients (25 %). Six patients ont recu un traitement immunosuppresseur. Apres une duree moyenne de suivi de 16,3 mois, l’acuite visuelle finale etait abaissee chez 6 patients (19 %). Conclusion La proportion de diagnostic dans notre serie est proche des principales series de la litterature. Un traitement par valaciclovir peut etre efficace chez des patients suspects de sclerite d’origine herpetique.

Resume Introduction Les anevrismes infectieux de l’aorte sont rares, representant environ 2 % des anevrismes aortiques. La majorite des cas decrits dans la litterature proviennent de series chirurgicales et concernent l’aorte abdominale. Dans ce travail retrospectif, nous avons analyse les caracteristiques cliniques, paracliniques et evolutives de patients presentant un anevrisme infectieux de l’aorte thoracique. Patients et methodes Le diagnostic a ete porte sur l’association d’une imagerie thoracique evocatrice et d’une documentation microbiologique ou de l’evolution favorable sous traitement anti-infectieux. Etude retrospective de cas. Resultats Il s’agissait de 6 hommes et une femme, d’âge moyen 66 ans. Tous presentaient au moins un facteur de risque cardiovasculaire ou de l’atherome. La fievre (71 %) et les douleurs thoraciques (42 %) etaient les signes cliniques les plus frequents. Les germes identifies etaient : Salmonella enteritidis (n = 3), Staphylococcus aureus (n = 1), et Candida albicans (n = 1). L’angioscanner montrait un aspect inflammatoire de l’aorte (n = 4), un anevrisme superieur a 50 mm (n = 5) ou augmentant rapidement de diametre (n = 5). La prise en charge etait medicale et interventionnelle : chirurgie (n = 3) ou prise en charge endovasculaire (n = 4). L’evolution etait favorable pour 6 patients ; le dernier decedait de complications liees a l’anevrisme. Conclusion La presentation clinique d’un anevrisme infectieux de l’aorte thoracique est peu specifique. Il convient d’evoquer ce diagnostic devant un anevrisme volumineux ou augmentant rapidement de diametre, associe a un syndrome inflammatoire. Le traitement endovasculaire semble une bonne alternative a la chirurgie. La place du TEP-scanner dans le diagnostic et le suivi reste a definir.

Resume Propos Le parvovirus B19 est responsable du megalerytheme epidemique chez l’enfant, de crise d’erythroblastopenie aigue chez le sujet porteur d’une hemoglobinopathie, d’anemie aregenerative chronique chez l’immunodeprime et d’anasarque fœtale pendant la grossesse. Chez l’adulte immunocompetent, le spectre des manifestations cliniques et biologiques ne cesse de s’elargir. Methodes Nous rapportons une serie de 26 observations de primo-infection a parvovirus B19 chez l’adulte immunocompetent. Il s’agit d’une etude retrospective sur la periode 2000 a 2010 realisee dans deux services de medecine interne. Le diagnostic etait clinique, serologique ou moleculaire. Resultats Il existait une predominance feminine (sex-ratio 3,33/1). L’âge moyen au diagnostic etait de 38,8 ans (extremes : 18–68). Les principales manifestations cliniques etaient la fievre (65 %), des polyarthralgies peripheriques et symetriques (62 %) et un exantheme (58 %). Deux patients ont presente une atteinte neurologique (paralysie du VI, paresthesies des extremites) et un patient une myocardite. Les anomalies biologiques observees etaient moderees et transitoires avec un syndrome inflammatoire (73 %), une thrombopenie (43 %), une lymphopenie (38 %) et une cytolyse hepatique (37 %). Une production d’anticorps anti-nucleaires (19 %), d’anticorps anti-ADN natifs (28 %), d’anticorps anti-phospholipides (14 %) associee a une hypocomplementemie (32 %) ont ete observees. Des fausses reactions positives d’IgM anti-CMV et anti-EBV etaient presentes dans 27 % des cas. Deux patients ont presente une infection a parvovirus B19 persistante. Conclusion La diversite des presentations peut etre a l’origine de tableaux trompeurs pour le clinicien. Le diagnostic doit etre evoque chez l’adulte immunocompetent en raison du risque de transmission a des patients susceptibles de developper une infection severe comme la femme enceinte ou les patients immunodeprimes.

We evaluated mortality rates and underlying causes of death among French decedents with sarcoidosis from 2002 to 2011.We used data from the French Epidemiological Centre for the Medical Causes of Death to 1) calculate sarcoidosis-related mortality rates, 2) examine differences by age and gender, 3) determine underlying and nonunderlying causes of death, 4) compare with the general population (observed/expected ratios), and 5) analyse regional differences.1662 death certificates mentioning sarcoidosis were recorded. The age-standardised mortality rate was 3.6 per million population and significantly increased over the study period. The mean age at death was 70.4 years (versus 76.2 years for the general population). The most common underlying cause of death was sarcoidosis. Sarcoidosis decedents were more likely to be males when aged 1 for infectious disease, tuberculosis and chronic respiratory disease, and Despite the limitation of possibly capturing the more severe cases of sarcoidosis, this study may help define and prioritise preventive interventions.

The off-label use of TNF antagonists in refractory sarcoidosis is increasingly reported but data on their efficacy and safety are still insufficient.To report on efficacy and safety of TNF antagonists in severe and refractory sarcoidosis.Examination of retrospective demographic, clinical, therapeutic, and adverse event data on 132 sarcoidosis patients (58% women; mean (min-max) age = 45.5 (14-78) years) given TNF antagonists (mainly infliximab, 91%) and investigation of response-linked factors.The overall clinical response (complete and partial) rate was 64%. TNF-antagonist efficacy (i.e., significant decrease of the ePOST score) was noted in cases with neurologic, heart, skin, and upper respiratory tract involvements. No significant difference in efficacy was found between anti-TNF used alone and TNF with immunosuppressant. The use of anti-TNF allowed reducing prednisone dosage at end of follow-up (p0.001). Adverse events were observed in 52% of the patients; they included infections (36%) and allergic reactions (8%) and required treatment interruption in 31 cases (23%). When TNF antagonists were interrupted, 13 patients experienced relapses within 14 months on average (median follow-up: 20.5 months).TNF antagonists were efficacious in about two-thirds of patients with severe/refractory sarcoidosis but their use led to a high rate of adverse events.

Familial Mediterranean fever is an autosomal-recessive autoinflammatory disorder more commonly observed in Mediterranean populations and characterized by recurrent episodes of fever, serositis, myalgia and arthritis. There is rarely any association with spondyloarthritis. The most important long-term complication is progressive systemic type AA amyloidosis. Treatment with colchicine is effective in reducing the frequency of attacks and prevents the development of amyloidosis. However, 5% of cases are considered resistant to colchicine. We here describe the case of a 39-year-old man, with a history of arthritis, arthralgias, and sacroiliitis in the course of a familial Mediterranean fever. He is homozygous for the M694I mutation in the MEFV gene. He subsequently developed myositis of the right quadriceps muscle confirmed by magnetic resonance imaging, electromyography and histology. He had frequent and severe arthralgias, despite colchicine, then etanercept and adalimumab, impairing his quality of life. The patient was successfully treated with the IL-1 receptor antagonist anakinra with a dramatic improvement of muscular and articular symptoms. To our knowledge, our patient is the first patient with coexisting FMF, spondyloarthritis and myositis responding to anakinra treatment. Moreover this is the second case in the literature of myositis associated with familial Mediterranean fever.

Sarcoidosis is a systemic disorder of unknown cause characterized by its pathological hallmark, the non-caseating granulomas, and by variable clinical course. While most of the cases affect people aged between 25 and 40 years, approximately 30 % of cases occur in older patients. Elderly-onset sarcoidosis (EOS) is defined as the onset of sarcoidosis in people over 65 years of age. Specific studies on the incidence and prevalence of sarcoidosis in this subgroup are scarce. Several studies suggest that the clinical features of EOS differ from those of sarcoidosis in younger patients. Compared with younger patients, fatigue, uveitis and specific skin lesions are more common, while erythema nodosum and chest x-ray abnormalities are less frequent. The diagnosis of EOS is challenging and may be delayed for many months because of its insidious onset, low prevalence and similarity to other more common disorders. When there is a granulomatous reaction in the elderly, clinicians should doubt the diagnosis and first think of tuberculosis, neoplasia or rare settings such as granulomatosis with polyangiitis or granulomatous reaction due to interferon and tumour necrosis factor-α (TNFα) blockers. A minor salivary gland biopsy also has a higher accuracy for diagnosis in the elderly. The current management of EOS remains empiric because of the lack of randomized, controlled studies. However, the approach to treatment is similar, regardless of the age of the patient. The treatment may be complicated by co-morbidities and increased risk of toxicities from usual treatments, particularly steroids. This review discusses the epidemiology, clinical course, prognosis and treatment of EOS.

Introduction La sarcoidose est une granulomatose systemique rare qui atteint avec predilection les adultes d’âge moyen. Les etudes portant sur les patients de plus de 60 ans, et a fortiori plus âges, sont donc rares. L’objectif de cette etude etait d’analyser les caracteristiques et le pronostic des patients de plus de 75 ans au diagnostic de sarcoidose et de les comparer a celles de patients plus jeunes. Patients et methodes Etude cas-temoin retrospective, multicentrique, comparant les caracteristiques des sarcoidoses du sujet âge (SSA) definies par un âge d’au moins 75 ans au moment du diagnostic, a celles des sarcoidoses du sujet jeune (SSJ), a partir des donnees du programme de medicalisation des systemes d’information des Hospices civils de Lyon, entre 2006 et 2017. Chaque patient du groupe SSA etait apparie aleatoirement a 3 temoins. Le diagnostic de sarcoidose reposait sur les criteres consensuels internationaux de l’ATS/ERJ/WASOG [1] . La severite de la sarcoidose etait evaluee selon la « sarcoid clinical activity classification » [2] . Resultats Nous avons inclus 34 patients dans le groupe SSA que nous avons compare a 102 patients plus jeunes. L’âge moyen etait de 79,8 (75–89) ans dans le groupe SSA contre 43,1 (21–74) ans dans le groupe SSJ. Dans le groupe des SSA, les patients etaient plus volontiers de sexe feminin (ratio F/H 2,78 vs 1,43, p = 0,125), d’origine caucasienne (94,1 vs 59,8 % ; p La duree moyenne de suivi etait de 44 (± 41) mois dans le groupe des SSA et de 71(± 67) mois dans le groupe des SSJ. Un traitement systemique initial etait introduit dans 52,9 % des cas de SSA et 37,3 % des cas de SSJ (p = 0,11). Il n’y avait pas de difference entre les groupes en terme de rechutes. Les sujets du groupe SSA presentaient plus d’effets secondaires sous traitement (89,5 % vs 65,7 ; p = 0,04), notamment infectieux (26,5 % vs 12,8 % ; p = 0,003). 5 deces sont survenus dans le groupe SSA contre 3 dans le groupe SSA (p = 0,012), aucun du fait de la sarcoidose. Conclusion La presentation clinique de la sarcoidose du sujet âge est marquee, dans notre etude, par la frequence de l’atteinte ophtalmologique, l’absence d’erytheme noueux et la faible proportion de signes respiratoires, confirmant les donnees anterieures [3] . L’asthenie et la presence de signes cutanes specifiques, donnees comme plus frequentes chez le sujet âge dans les etudes anterieures, n’est pas confirmee [3] . Les patients âges traites par un traitement systemique presentent plus d’effets secondaires des traitements, imposant une prevention et une attention particuliere dans cette population.

Although primary immunodeficiencies (PID) are typically marked by increased susceptibility to infections, autoimmune manifestations have increasingly been recognized as an important component of several forms of PID. Here, we discuss two forms of PID in which autoimmune cytopenias are particularly common and may be the first manifestation of the disease in adults: autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency (CVID). Approximately one fifth of patients with CVID develop autoimmune diseases, and immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AHA) are the most common. Since autoimmune cytopenias frequently precede the diagnosis of CVID, testing for immunoglobulin levels should be performed in patients diagnosed with AITP and AHA. Patients with CVID in association with autoimmune cytopenias have a "particular phenotype" with lower susceptibility to infection and higher susceptibility to autoimmune manifestations and, for patients with AHA, a more frequent development of splenomegaly and lymphoma. Corticosteroids and high doses of intravenous immunoglobulins (IVIg) seem to have the same efficacy as in idiopathic AITP and AHA. Splenectomy and rituximab are as effective as in idiopathic autoimmune cytopenias but are associated with an increased risk of severe infection and should, in our opinion, be considered only for those rare patients with "refractory diseases". The course and outcome of autoimmune cytopenias is not affected by supportive IVIg therapy. Autoimmune destruction of blood cells affects over 70% of ALPS patients. The median age of first presentation is 24 months of age, but with increasing awareness of this condition, adults with autoimmune cytopenias are now being diagnosed more frequently. Testing for ALPS should therefore be considered in young adults with unexplained Evan's syndrome. Patients usually respond to immunosuppressive medications, including corticosteroids. Unlike many patients with idiopathic autoimmune cytopenias, the cytopenias in patients with ALPS typically do not respond to IVIg. After corticosteroids, the immunosuppressive drug that is the most studied in ALPS patients is mycophenolate mofetyl. Rituximab and splenectomy are relatively contraindicated in ALPS because of an increase risk of severe infection and should be reserved for patients who fail all other therapies.

International audience; BACKGROUND AND OBJECTIVES: Progressive multifocal leukoencephalopathy (PML) is a rare, JC-virus-mediated, demyelinating disease with a high mortality rate. As no recommended treatment exists, mirtazapine, a potential blocker of virus entry into cells, has been empirically used. METHODS: We analysed existing data on mirtazapine's efficacy to treat PML by systematically reviewing the literature since 2005, when it was first used. RESULTS: Searches in PubMed, EBSCO, SCOPUS and Google Scholar between January 2005 and December 2015, identified five cohort studies and 74 case reports. No statistically significant effect of mirtazapine on PML outcome was observed in the cohort studies. From studying the case reports, mortality rate for PML was associated with the underlying circumstances, such as an older age, the use of an immunosuppressant, or PML occurring in patients with a haematological malignancy or a transplant. CONCLUSIONS: Except for natalizumab-associated PML, we did not highlight any potential benefit of mirtazapine on disease outcomes. Further interventional studies are needed to confirm that 5-HT2AR inhibition is relevant to treat PML

International audience; Parenchymal lung involvement (PLI) in adult-onset Still's disease (AOSD) has seldom, if ever, been studied. We examine here retrospective cohort AOSD cases and present a review of the literature (1971-2014) on AOSD-related PLI cases.Patients with PLI were identified in 57 AOSD cases. For inclusion, the patients had to fulfill Yamaguchi or Fautrel classification criteria, show respiratory symptoms, and have imaging evidence of pulmonary involvement, and data allowing exclusion of infectious, cardiogenic, toxic, or iatrogenic cause of PLI should be available. This AOSD + PLI group was compared with a control group (non-PLI-complicated AOSD cases from the same cohort).AOSD + PLI was found in 3 out of the 57 patients with AOSD (5.3%) and the literature mentioned 27 patients. Among these 30 AOSD + PLI cases, 12 presented an acute respiratory distress syndrome (ARDS) and the remaining 18 another PLI. In the latter, a nonspecific interstitial pneumonia computed tomography pattern prevailed in the lower lobes, pulmonary function tests showed a restrictive lung function, the alveolar differential cell count was neutrophilic in half of the cases, and the histological findings were consistent with bronchiolitis and nonspecific interstitial pneumonia. Corticosteroids were fully efficient in all but 3 patients. Ten out of 12 ARDS cases occurred during the first year of the disease course. All ARDS-complicated AOSD cases received corticosteroids with favorable outcomes in 10 (2 deceased). Most PLIs occurred during the systemic onset of AOSD.PLI may occur in 5% of AOSDs, of which ARDS is the most severe. Very often, corticosteroids are efficient in controlling this complication

Backgroung: This study investigated the efficacy and safety of TNF antagonists in sarcoid uveitis in unselected cases. Design: This is a multicentre study on patients with sarcoidosis who received TNF antagonists in pneumology and internal medicine departments in France. We present here the subgroup of patients with biopsy-proven sarcoid uveitis included in the nationwide registry STAT (Sarcoidosis treated with TNF AnTagonists). Results: Among the 132 patients included in this multicenter study, 18 patients with refractory uveitis were treated as a first-line TNF antagonist with infliximab (n=14), adalimumab (n=3) and certolizumab (n=1). Before anti-TNF initiation, the median duration of sarcoidosis was 42 months and 83% of the patients have been treated with at least one immunosuppressive drug. Six patients switched for a second-line TNF antagonist. After a mean time under treatment of 29 months, the treatment resulted in a significant decrease of the ophthalmic extrapulmonary Physician Organ Severity Tool (ePOST) (mean score: 4.2 vs. 2.6) scores and a steroid sparing effect (29.4±20.7 vs. 6.2±5.2 mg/d). Overall, the ophthalmic response, either complete or partial, was 67%. Nine patients (50%) presented adverse events, including severe infectious complications in 5 patients, which required anti-TNF treatment interruption in 6 cases (33%). Among the 7 responder patients who discontinued anti-TNF therapy, 71% relapsed. Finally, 12 patients (67%) could continue TNF antagonist treatment. Conclusions: TNF antagonists were efficient in 67% of biopsy-proven refractory sarcoid uveitis. Severe adverse events, mainly infectious complications, were frequent. The high frequency of relapses after anti-TNF-α discontinuation requires a close patient follow-up thereafter. (Sarcoidosis Vasc Diffuse Lung Dis 2017; 34: 74-80)

Objectives To describe the clinical features, treatment, and outcome of autoimmune diseases (AD) in a cohort of patients with thymoma. Design Pathological records from three university hospitals, between 2005 and 2011, were reviewed to identify patients with thymoma. Patients with thymoma and AD were compared with patients with thymoma without AD. Results 47/85 (55%) cases of thymoma had AD, including myasthenia gravis (MG) (n = 33), Hashimoto's thyroiditis (n = 4), Isaac's syndrome (n = 3), Morvan syndrome (n = 2), pure red cell aplasia (n = 2), systemic lupus (n = 2), lichen planus (n = 2), and one case of each following conditions: aplastic anemia, autoimmune hemolytic anemia, Good's syndrome, pemphigus, autoimmune hepatitis, Graves' disease, limbic encephalitis, and inflammatory myopathy. Six patients (7%) presented at least 2 ADs. The median duration of follow-up after surgery was 60 months (40–78 months). In 32 patients, the diagnosis of AD preceded the diagnosis of thymoma, in 9 patients, thymoma was diagnosed at the same time as the AD and 7 patients had been operated on when they developed an AD. We found a significative difference on the Masaoka stage between the MG patients and the patients who present another AD (p = 0.028). No risk factor for developing an AD after thymectomy was identified. Conclusions We describe here the long-term follow-up of a large series of AD related to thymoma. Our results confirm previous data concerning AD occurrence in patients with thymoma and suggest that preexisting autoimmunity is not a risk factor for developing autoimmune manifestations after thymectomy.

International audience; Hemophagocytic lymphohistiocytosis (HLH) is a critical condition that may lead to organ failure and early death. The aim of this retrospective observational study was to describe a cohort of HLH patients admitted to intensive care unit (ICU) and investigate the risk factors of early death. A positive HLH diagnosis was defined by an HScore \textgreater= 169. Univariate and multivariate analyses were carried out to investigate hospital and 28-day mortality risk factors. Between January 2002 and July 2014, 71 HLH cases were seen at our institution. The overall 28-day mortality (start at ICU admission) and hospital mortality were 38% and 68%, respectively. The factors associated with increased 28-day mortality were the sequential organ failure assessment score at ICU admission (P \textless .001) and advance in age (P = 0.03). The factors associated with increased hospital mortality were a high sequential organ failure assessment score at ICU admission (P \textless 0.01), advance in age (P = 0.04), and the presence of lymphoma-related HLH or HLH of unknown origin (P \textless 0.01). Organ failure overtops the classical early-death risk factors in adult ICU-admitted HLH patients. This failure and the subsequent early death may be prevented by timely specific cytotoxic therapies and the control of the underlying disease.

International audience; PURPOSE: To describe uveitis clinical characteristics in the elderly. METHODS: Retrospective review of 91 patients at the age of 60 or more years at the authors' uveitis tertiary center over a 7-year period. RESULTS: Uveitis in the elderly accounted for 30.1% of this population. Uveitis localization was anterior in 22.0% of patients, intermediate in 8.8%, posterior in 20.9%, while 41.7% patients presented with panuveitis. Sarcoidosis (37.4%) and idiopathic uveitis (36.3%) accounted for the majority of cases, whereas other diagnostic entities accounted for 26.3%. Panuveitis (41.7%) and sarcoidosis (37.4%) were detected at a significantly higher frequency than in the younger population. Contrarily, ankylosing spondylitis and established ophthalmological entities (pars planitis, Birdshot chorioretinopathy, Fuchs heterochromic cyclitis) were more common in patients younger than 60 years old. CONCLUSION: In the authors' experience, sarcoidosis is the leading cause of uveitis in the elderly. Idiopathic uveitis and other specific entities account for less than two-thirds of cases.

Resume Propos L’uveite regroupe un large ensemble de maladies inflammatoires de l’uvee. Les presentations anatomocliniques aussi bien que les etiologies sont nombreuses, ce qui constitue un enjeu diagnostique et therapeutique pour les cliniciens qui prennent en charge ces patients. L’objectif de notre travail est de decrire les differents aspects anatomocliniques et etiologiques des uveites, au moyen d’une nouvelle classification, ainsi que des nouveaux moyens d’investigations paracliniques utilises pour le diagnostic des uveites. Methodes Il s’agit d’une etude retrospective, realisee de janvier 2002 a decembre 2006, concernant les patients consultant pour une uveite dans un centre d’ophtalmologie tertiaire. Les uveites associees au virus de l’immunodeficience humaine, ainsi que celles secondaires a une endophtalmie post-traumatique ou post-chirurgicale ont ete exclues. Tous les patients ont beneficie d’un examen ophtalmologique complet suivi d’un bilan clinique et paraclinique adapte. Les diagnostics ont ete etablis selon les criteres internationaux recents. Resultats L’analyse a porte sur 121 patients. Les etiologies les plus frequentes sont l’infection par le toxoplasme (14 %), la sarcoidose (11,6 %), les spondylarthropathies et les uveites associees a l’antigene d’histocompatibilite HLA B27 (13,2 %), et les infections a Herpes virus (9,1 %). Ces quatre entites rendent compte de pres de la moitie des etiologies (47,9 %). Pres d’un tiers des uveites (29,7 %) reste de cause indeterminee. Les autres etiologies representent au total 20,6 % de la serie. Le groupe des maladies systemiques represente dans cette serie 35,5 % des diagnostics (43 patients), les infections 26,4 % (32 patients) et les entites ophtalmologiques pures 8,3 % (12 patients). Conclusion Cette etude, bien que portant sur un nombre limite de patients, montre des resultats etiologiques comparables a ceux des series les plus importantes de la litterature. Elle se distingue, neanmoins, par un taux eleve de sarcoidose et de maladies systemiques, soulevant l’interet d’une prise en charge tenant compte de criteres cliniques et paracliniques modernes utilises de facon standardisee en collaboration avec le medecin interniste.

Introduction Les uveites designent un large ensemble de maladies inflammatoires de l’uvee. La grande diversite des manifestations anatomocliniques et des etiologies constitue un interet diagnostic et therapeutique majeur. L’objectif de ce travail est une description des caracteristiques anatomocliniques de ces uveites, ainsi que du diagnostic et du traitement qui leur sont associes. Patients et methodes Il s’agit d’une etude retrospective realisee entre juillet 2002 et aout 2016, concernant les patients referes dans le service de medecine interne de la Croix-Rousse pour le diagnostic etiologique et/ou le traitement d’une uveite. Tous les patients ont beneficie d’un examen ophtalmologique complet suivi d’un bilan clinique et paraclinique oriente. Les diagnostics ont ete etablis selon les criteres internationaux en vigueur. Les uveites post-chirurgicales, secondaires a une endophtalmie post-traumatique ou au virus de l’immunodeficience acquise, ont ete exclues. Resultats La population se compose de 957 patients, comprenant 588 femmes (59,3 %), avec un âge moyen au diagnostic de l’uveite de 47,3 ans. La repartition ethnique est la suivante : caucasienne (n = 782, 81 %), Afrique du Nord (n = 124, 12 %), Afrique subsaharienne et Antilles (n = 26, 2,7 %) et Asie (n = 12, 1,3 %). La repartition anatomique des uveites est la suivante : anterieure (UA) (n = 312, 32,6 %), intermediaire (UI) (n = 96, 10 %), posterieure (UP) (n = 162, 16,9 %), panuveites (PU) (n = 260, 26,5 %), ainsi que des formes combinees comprenant 68 UA plus UI soit 7,1 %, 51 UI plus UP soit 5,3 % et enfin 10 UA plus UP soit 1 %. Il s’agit d’uveites unilaterales chez 410 patients (42,8 %), chroniques pour 633 patients (66 %), avec un aspect granulomateux chez 263 patients (27,5 %). Sur le plan diagnostic, la repartition etiologique est la suivante : 360 uveites (37,6 %) sont d’origine indeterminee, 345 (36,05 %) d’origine inflammatoire, 121 uveites (12,64 %) associees a une entite purement ophtalmologique, 106 uveites (11 %) d’origine infectieuse et 20 uveites (2,08 %) liees a un lymphome. Parmi les causes inflammatoires les plus frequentes, la sarcoidose rend compte de la pres de la moitie des cas (n = 175, dont 114 sarcoidoses histologiquement prouvees), suivie des uveites associees au groupage HLA-B27 (n = 62), des spondylarthrites (n = 43), de la maladie d’Harada (n = 27) et de la maladie de Behcet (n = 21). Les entites ophtalmologiques les plus frequentes sont la maladie de Birdshot (n = 38), les pars planites (n = 22) et la cyclite de Fuchs (n = 20). Les etiologies infectieuses comprennent par ordre decroissant : la tuberculose (n = 44), les virus du groupe Herpes (n = 22), la toxoplasmose (n = 12), la maladie de Lyme (n = 11) et la syphilis (n = 7). La repartition des traitements administres est la suivante : corticotherapie systemiques (n = 349, 36,46 %) ; seule (n = 170) ou en association avec un autre traitement (n = 179), traitement local seul (n = 346, 36 %), immunosuppresseurs (n = 169, 17,5 %). Cinquante et un patients ont recu un anti-TNF ; il s’agissait de l’adalimumab dans 35 cas. Cent un patients (10,5 %) ont recu un traitement anti-infectieux, incluant 47 antibiotherapies antituberculeuses. Conclusion Cette serie, provenant d’un centre tertiaire offre une photographie recente de la repartition etiologique et de la prise en charge des uveites en France. La sarcoidose, les spondyloarthrites et les uveites associees au groupage HLA-B27, les maladies infectieuses (toxoplasmose, herpes virus et tuberculose), les entites ophtalmologiques (pars planite, cyclite heterochromique de Fuchs et maladie de Birdshot) rendent compte de pres de ¾ des etiologies identifiees. Meme si plus rarement en cause, la syphilis, les bacteries intracellulaires, la maladie de Behcet, le lymphome primitif oculo-cerebral et la maladie de Vogt-Koyanagi-Harada doivent etre evoques en raison des consequences therapeutiques et pronostiques. La part importante des maladies inflammatoires, la place des immunosuppresseurs et le developpement des anticorps monoclonaux dans l’arsenal therapeutique renforcent l’interet de la collaboration ophtalmologiste-medecin interniste pour la prise en charge des patients.

UNLABELLED BASIC: To describe the main characteristics and treatment of sarcoidosis in patients with chronic hepatitis C virus (HCV) infection. METHODS Retrospective cohort study of patients with chronic HCV infection and sarcoidosis at our tertiary institution. RESULTS Eleven cases (eight women, three men) fulfilled the criteria for sarcoidosis. Four cases belong to our population of 3194 (0.12%) HCV patients seen in our department between 2001 and 2008. In five cases, sarcoidosis was triggered by antiviral therapy (consisted of interferon-alpha monotherapy in one case and combined therapy with interferon-alpha and ribavirin in four cases) and developed from 23 to 82 months after completion of therapy in three cases. For these patients, pulmonary adenopathies were found in three patients while two presented cutaneous involvement, one had uveitis and one presented both arthritis and extrapulmonary lymphadenopathies. Two patients received systemic corticosteroids with a favourable outcome. Four treatment-naive patients developed sarcoidosis. Two had pulmonary disease, one had medullar involvement, one had superficial lymphadenopathy and one had arthralgia. Three patients received systemic corticosteroids with chronic outcome in all cases. One of the two patients with an earlier history of sarcoidosis experienced a benign relapse that resolved spontaneously. CONCLUSION Clinical manifestations of sarcoidosis may occur in HCV patients, especially during or after treatment with immunotherapy. In our experience, sarcoidosis triggered by antiviral therapy was more frequent after completion of therapy, but concording with literature, presented a benign outcome. In sarcoidosis, seen in treatment-naive HCV patients, systemic corticosteroids had to be used more often and outcome was less favourable.

Introduction Les etiologies des uveites sont multiples et un tiers des cas demeure sans cause identifiee [1] . Elles peuvent survenir a tout âge mais leur etiologie et leur type anatomoclinique peuvent varier en fonction de l’âge de decouverte. Cette etude a pour but de decrire les caracteristiques cliniques et etiologiques des uveites du sujet âge. Patients et methodes Etude retrospective monocentrique dans un centre tertiaire de 1044 patients consecutifs nouvellement diagnostiques d’une uveite sur une periode de 14 ans. Les caracteristiques anatomocliniques etaient recueillies au diagnostique. Le diagnostique etiologique etait etabli par un interniste apres un bilan etiologique cible. La population âgee de 60 ans et plus etait comparee a la population plus jeune concernant le type anatomoclinique de l’uveite et le diagnostic etiologique retenu. Les patients de 60 a 70 ans etaient egalement compares aux patients de 71 ans et plus. Resultats Entre mars 2003 et novembre 2017, 1044 patients ont ete inclus comprenant 283 patients (27,1 %) âges de 60 ans ou plus, dont 141 de plus de 71 ans (13,5 %). Apres 60 ans, l’uveite est le plus souvent une panuveite (31,8 %), puis une uveite anterieure (23,7 %), posterieure (20,5 %), intermediaire (8,8 %), ou mixte (15,2 %). Il existe significativement plus de panuveite (31,8 % vs. 23 %), d’uveite posterieure (20,6 % vs. 16,3 %), et moins d’uveite anterieure (23,7 % vs. 36,7 %) ou intermediaire (8,8 % vs. 11,6 %) (p = 0,0005), chez les patients de 60 ans et plus. Il y a plus d’uveite chronique (80,6 % vs. 61,2 %, p Il n’y a pas de difference pour le type anatomique entre 60–70 ans et apres 71 ans, en dehors du caractere granulomateux plus frequent apres 71 ans (41,1 % vs. 23,2 %, p = 0,001). Il existe une tendance pour plus d’uveite idiopathique apres 71 ans (41,1 % vs. 31,7 %, p = 0,004). Dans ce groupe, les sarcoidose histologiquement prouvee sont moins frequentes qu’entre 60 et 70 ans (28,3 % vs. 62,8 %, p = 0,003) a l’inverse des sarcoidoses presumees (25,6 vs. 56,5 %, p = 0,003). Il existe une tendance a moins de lymphome (3,6 % vs. 6,3 %), plus d’uveite herpetique (7,1 % vs 2,8 %) et idiopathiques (41,1 % vs. 31,7 %) apres 71 ans. Conclusion Les uveites du sujet âge sont le plus souvent des uveites chroniques. Il s’agit dans environ 1/3 des cas d’une uveite idiopathique, dans 1/3 des cas d’une sarcoidose et dans 1/3 des cas d’une cause autre. Les entites ophtalmologiques sont exceptionnelles apres 60 ans. Peu d’etudes ont evalue les caracteristiques cliniques et epidemiologiques des uveites du sujet âge [2] . Notre etude retrospective realisee a partir d’un effectif important montre des donnees comparables a celles de la litterature avec notamment une nette predominance de sarcoidose a cet âge, a l’inverse des uveites associee a HLA B27 ou spondyloarthrite. Ce travail montre les specificites des uveites survenant apres 70 ans.

Kawasaki disease (KD) is an acute multisystemic vasculitis occurring predominantly in children and rarely in adults. Diagnosis is made clinically using diagnostic guidelines; no specific test is available. "Incomplete" KD is a more recent concept, which refers to patients with fever lasting > or =5 days and 2 or 3 clinical criteria (rash, conjunctivitis, oral mucosal changes, changes of extremities, adenopathy), without reasonable explanation for the illness. To describe the clinical and laboratory features of classical (or "complete") KD, and incomplete KD in adults, we report 10 cases of adult KD, including 6 patients who fulfilled the criteria for incomplete KD, diagnosed either at presentation (n = 4) or retrospectively (n = 2). At the time of clinical presentation, complete KD was diagnosed in 4 patients, while 4 patients fulfilled the criteria for incomplete KD. For 3 of the 4 patients with incomplete KD, presence of severe inflammation, laboratory findings (hypoalbuminemia, anemia, elevation of alanine aminotransferase, thrombocytosis after 7 days, white blood cell count > or =15,000/mm, and urine > or =10 white blood cell/high power field), or echocardiogram findings were consistent with the diagnosis. In 2 patients, the diagnosis of KD was made retrospectively in the presence of myocardial infarction due to coronary aneurysms, after an undiagnosed medical history evocative of incomplete KD. Seven patients received intravenous immunoglobulins (IVIG), after a mean delay of 12.5 days, which appeared to shorten the course of the disease. This relatively large series of adult KD highlights the existence of incomplete KD in adults and suggests that the algorithm proposed by a multidisciplinary committee of experts to diagnose incomplete KD in children could be useful in adults. Further studies are needed to determinate whether prompt IVIG may avoid artery sequelae in adult patients with complete or incomplete KD.

Resume Introduction Le syndrome hypereosinophilique (SHE) est rarement responsable de polyserite. L’imatinib est le traitement de reference des variants myeloides du SHE associes au transcrit de fusion FIP1L1-PDGFRA. Une reponse a ete rapportee chez des patients atteints de SHE, chez lesquels le transcrit de fusion n’etait pas detecte. Observation Nous rapportons ici l’observation d’un patient porteur d’un SHE negatif pour le transcrit avec une atteinte pericardique severe et pleurale bilaterale. L’existence d’une corticodependance a dose elevee, conduisait a mettre en place un traitement par imatinib a la dose de 100 mg/jour. Une reponse etait obtenue en 15 jours. La corticotherapie pouvait etre arretee au bout de sept mois et le patient demeurait asymptomatique apres 23 mois de traitement. Conclusion Cette observation souligne l’existence de polyserites associees au SHE et l’interet du traitement par imatinib meme en l’absence de transcrit de fusion FIP1L1-PDGFRA.

Introduction Les atteintes ophtalmologiques sont frequentes au cours de la sarcoidose rapportees chez 25 a 50 % des patients [1] . Les atteintes, les plus frequentes concernent par ordre de frequence decroissante : l’uvee (uveite), la cornee (30 %), et la conjonctive (7,5 %). Plusieurs cas cliniques et une serie [2] ont montre que la tomographie par emission de positons (TEP) marquee au Fluorodeoxyglucose (FDG) pouvait mettre en evidence des fixations mediastinales, salivaires et/ou lacrymales compatibles avec le diagnostic de sarcoidose, chez des patients presentant des uveites inexpliquees ou suspectes de sarcoidose. Un âge superieur a 56 ans, la presence de synechies posterieures et des adenopathies mediastinales sur le scanner sont associees aux anomalies scintigraphiques [2] . L’objectif de cette etude etait d’evaluer l’impact de la TEP au FDG chez les patients pris en charge pour une uveite avec scanner thoracique considere comme normal. Patients et methodes Etude monocentrique a partir d’un centre specialise pour la prise en charge d’une uveite. Soixante-neuf patients presentant une uveite chronique inexpliquee ou suspecte de sarcoidose et ayant eu une TDM thoracique non consideree comme evocateur de sarcoidose, ont beneficie d’une TEP de janvier 2010 a aout 2017. Les TEP–FDG ont ete interpretees en insu par un medecin nucleaire, puis correlees aux caracteristiques cliniques des patients et aux resultats des autres examens complementaires. Les criteres d’Abad [3] ont ete utilises pour le diagnostic d’uveites sarcoidosiques. La regression logistique a ete utilisee pour l’analyse multivariee ainsi que pour la construction d’un nomogramme afin de predire la probabilite d’une TEP positive. Resultats Il s’agit de 47 femmes et 22 hommes, d‘âge median 55,3 ans (6,1–86 ans). La repartition anatomique des uveites est la suivante : anterieure (n = 24), intermediaire (n = 8), posterieures (n = 9) et panuveite (n = 28). L’uveite est chronique (n = 53), bilaterale (n = 53), granulomateuse (n = 39), compliquee de synechies (n = 26) et d’un œdeme maculaire (n = 36). L’enzyme de conversion de l’angiotensine (ECA) etait elevee chez 25 patients et 4 patients presentaient une anergie tuberculinique. La TEP–FDG a mis en evidence des fixations hilaires (n = 22) ou mediastinales (n = 22) et parenchymateuses pulmonaires (n = 2) evocatrices de sarcoidose dans 22 cas. L’analyse multivariee montre une correlation de la positivite de la TEP–FDG avec : l’âge (p = 0,0005), l’existence de synechies (p = 0,0509) ainsi qu’une elevation de l’enzyme de conversion de l’angiotensine (ECA) (p = 0,034). Les 22 patients qui avaient une TEP compatible avec le diagnostic de sarcoidose etaient significativement plus âges (âge moyen de 68,6 ans [min–max, 36–86 ans] versus 46,9 ans [min–max 6–84 ans] ; p Conclusion La FDG–TEP peut montrer des fixations evocatrices de sarcoidose chez des patients presentant une uveite chronique inexpliquee ou idiopathique ayant une TDM thoracique normal. Nous presentons un nomogramme predictif de la positivite de cet examen prenant en compte l’âge, la presence de synechies et l’elevation de l’ECA. Nos resultats doivent etre valides sur une population independante.

Background: Spinal cord involvement in sarcoidosis is rare, occurring in

Autoimmune hemolytic anemia (AIHA) is a relatively uncommon cause of anemia. Classifications of AIHA include warm AIHA, cold AIHA (including mainly chronic cold agglutinin disease and paroxysmal cold hemoglobinuria), mixed-type AIHA and drug-induced AIHA. AIHA may also be further subdivided on the basis of etiology. Management of AIHA is based mainly on empirical data and on small, retrospective, uncontrolled studies. The therapeutic options for treating AIHA are increasing with monoclonal antibodies and, potentially, complement inhibitory drugs. Based on data available in the literature and our experience, we propose algorithms for the treatment of warm AIHA and cold agglutinin disease in adults. Therapeutic trials are needed in order to better stratify treatment, taking into account the promising efficacy of rituximab.

Granulomatous myositis (GM) is a rare condition that has generally been described in association with sarcoidosis. In the absence of sarcoidosis or other underlying disease, a diagnosis of isolated GM is considered. Only one study has focused on the clinical difference between isolated GM and sarcoid myopathy (SM). We report 13 cases of symptomatic GM; 8 had sarcoidosis. All patients with sarcoidosis had predominantly proximal, symmetrical lower-limb weakness, and 3 subsequently developed upper-limb or distal involvement. Three of the five patients with isolated GM had predominantly distal muscle involvement, and two had dysphagia. Corticosteroid treatment was followed by prolonged improvement in only one patient with sarcoidosis. One patient had acute sarcoid myositis and benefited from methotrexate; other immunosuppressants and etanercept proved ineffective in chronic sarcoid myopathy. Three of the five patients with isolated GM responded to corticosteroid treatment. When last examined, three patients with sarcoidosis had severe disability, whereas patients with isolated GM showed milder weakness. Thus, SM was frequently associated with severe disability and rarely improved after corticosteroid treatment, whereas most patients with isolated GM improved.