Search

Background Trephine bone marrow biopsy (BMB) in internal medicine has only been studied in fever of unknown origin and inflammation of unknown origin. The aim was to assess BMB diagnostic yield according to main indications and patient characteristics in internal medicine. Quality of BMB and contribution of bone marrow aspiration (BMA) to BMB were also analyzed. Methods BMB performed in the internal medicine department of Poitiers university hospital between January 2000 and December 2015 were retrospectively analyzed. Patient characteristics, BMB indications, quality parameters, and results were collected from medical records. Contributive BMB was BMB allowing accurate final diagnosis. Diagnostic yield was the proportion of contributive BMB among total BMB performed. Results A total of 468 BMBs conducted for primary diagnostic purpose from 468 patients were analyzed. Cytopenia(s) and the indication 'adenopathy and/or splenomegaly and/or hepatomegaly' represented 70% of the indications. Overall BMB diagnostic yield was 32.7%, lymphoma being the main histologic finding (31%). Among indications, cytopenia(s) had the highest diagnostic yield (49.1%). Isolated fever of unknown origin had low diagnostic yield (5.6%). Factors independently associated with contributive BMB were: anemia, neutropenia, circulating immature granulocytes or blasts, monoclonal gammopathy, period of BMB processing, quality of BMB, and immunohistochemestry (IHC) analysis. Concomitant BMA improved diagnostic yield by 5.5%, mostly for myelodysplastic syndromes. Conclusion Cytopenia(s), blood cythemias and monoclonal gammopathy are indications with the highest diagnostic yield. Concomitant BMA and IHC analysis should be systematically performed to increase BMB diagnostic yield in internal medicine.

OBJECTIVE: Temporal arteritis (TA) is a typical manifestation of giant cell arteritis (GCA). Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are rarely revealed by TA manifestations, leading to a risk of misdiagnosis of GCA and inappropriate treatments. This study was undertaken to describe the clinical, biologic, and histologic presentations and outcomes in cases of TA revealing AAV (TA-AAV) compared to controls with classic GCA. METHODS: In this retrospective case-control study, the characteristics of patients with TA-AAV were compared to those of control subjects with classic GCA. Log-rank test, with hazard ratios (HRs) and 95% confidence intervals (95% CIs), was used to assess the risk of treatment failure. RESULTS: Fifty patients with TA-AAV (median age 70 years) were included. Thirty-three patients (66%) presented with atypical symptoms of GCA (ear, nose, and throat involvement in 32% of patients, and renal, pulmonary, and neurologic involvement in 26%, 20%, and 16% of patients, respectively). Blood samples were screened for ANCAs at the time of disease onset in 33 patients, and results were positive in 88%, leading to a diagnosis of early TA-AAV in 20 patients. The diagnosis of AAV was delayed a median interval of 15 months in 30 patients. Compared to controls with GCA, patients with TA-AAV were younger (median age 70 years versus 74 years), were more frequently men (48% versus 30%), and had high frequencies of atypical manifestations and higher C-reactive protein levels (median 10.8 mg/dl versus 7.0 mg/dl). In patients with TA-AAV, temporal artery biopsy (TAB) showed fibrinoid necrosis and small branch vasculitis in 23% of patients each, whereas neither of these characteristics was evident in controls with GCA. Treatment failure-free survival was comparable between early TA-AAV cases and GCA controls, whereas those with delayed TA-AAV had a significantly higher risk of treatment failure compared to controls (HR 3.85, 95% CI 1.97-7.51; P < 0.0001). CONCLUSION: TA-AAV should be considered diagnostically in cases of atypical manifestations of GCA, refractoriness to glucocorticoid treatment, or early relapse. Analysis of TAB specimens for the detection of small branch vasculitis and/or fibrinoid necrosis could be useful. Detection of ANCAs should be performed in cases of suspected GCA with atypical clinical features and/or evidence of temporal artery abnormalities on TAB. ispartof: ARTHRITIS & RHEUMATOLOGY vol:73 issue:2 pages:286-294 ispartof: location:United States status: published

International audience; The relapse rate in antiphospholipid syndrome (APS) remains high, i.e. around 20%–21% at 5 years in thrombotic APS and 20–28% in obstetrical APS [2, 3].Hydroxychloroquine (HCQ) appears as an additional therapy, as it possesses immunomodulatory and anti-thrombotic various effects [4–16].Our group recently obtained the orphan designation of HCQ in antiphospholipid syndrome by the European Medicine Agency.Furthermore, the leaders of the project made the proposal of an international project, HIBISCUS, about the use of Hydroxychloroquine in secondary prevention of obstetrical and thrombotic events in primary APS. This study has been launched in several countries and at now, 53 centers from 16 countries participate to this international trial.This trial consists in two parts: a retrospective and a prospective study.The French part of the trial in thrombosis has been granted by the French Minister of Health in December 2015 (the academic trial independent of the pharmaceutical industry PHRC N PAPIRUS) and is coordinated by one of the members of the leading consortium of HIBISCUS.

International audience; ObjectivesTo assess the efficacy of tocilizumab in patients with Takayasu arteritis (TA).MethodsWe conducted a retrospective multicenter study in 46 TA patients treated with tocilizumab. We analyzed factors associated with response to tocilizumab (assessed using NIH score).ResultsForty-six patients with TA were included, with a median age of 43 years [29–54], and 35 (76%) females. We observed a decrease in the median NIH scale (from 3 [2–3] at baseline to 0 [0–1] and 0 at 3 and 6 months, respectively; p

Background Objectives We report the long term outcome of 46 TA patients treated with tocilizumab, describe the factors of response and compare the vascular event free survival to DMARDs. Methods retrospective nationwide french study Results We observed a decrease in the NIH scale; in C-reactive protein levels and in daily prednisone dose at 3 and 6 months, respectively; p Conclusions This nationwide study shows that tocilizumab may reduce the incidence of vascular complications in TA. Disclosure of Interest None declared

Introduction L’arterite a cellules geantes (ACG) s’accompagne dans 40 a 65 % des cas d’une atteinte aortique au diagnostic ; qui peut entrainer des complications structurales (anevrysme, dissection) engageant le pronostic vital. La survenue de ces complications a ete peu etudiee et rien ne permet de les predire avec certitude. Le but de cette etude etait d’evaluer si des symptomes d’atteinte inflammatoire de l’aorte presents au diagnostic pouvaient etre associes a un risque plus important de complication aortique. Patients et methodes Les patients inclus retrospectivement etaient issus de 5 centres, ils presentaient une ACG (diagnostiquee de mai 1998 a avril 2019) associee a une aortite presente au diagnostic. L’aortite etait definie par un epaississement ≥ 2,2 mm a la TDM et/ou un hypermetabolisme de grade 2 ou 3 de la paroi aortique a la TEP. Nous avons compare les caracteristiques cliniques des patients selon la presence de symptomes d’aortite et effectue des analyses univariees et multivariees a la recherche de facteurs predictifs associes a la survenue ou a l’aggravation d’anomalies structurales aortiques au cours du suivi. Resultats Cent soixante et onze patients atteints d’une aortite au diagnostic d’ACG ont ete inclus. Cinquante-cinq (32 %) presentaient des symptomes d’aortite (douleurs, souffle d’insuffisance aortique, dyspnee). La duree mediane de suivi pour tous les patients etait de 38 mois (0–237 mois). Les complications aortiques survenaient apres un delai median de 32 mois (1–138 mois). Au total, il s’agissait de 19 nouveaux anevrysmes ou complications d’un anevrysme, et 5 dissections. Quinze complications aortiques etaient survenues chez les patients symptomatiques (27 %) et 9 (8 %) chez les patients asymptomatiques (p En analyses uni- et multivariees, la presence de symptomes d’aortite etait le seul facteur pronostic significativement associe a la survenue d’une complication aortique a distance (OR 5,00 IC95 % [1,79 ; 14] p = 0,002). Conclusion Dans cette etude, la presence de symptomes d’aortite au diagnostic de l’arterite a cellules geantes etait le seul facteur predictif independant de survenue de complications aortiques au cours du suivi. Cette donnee, si elle est confirmee lors d’etudes prospectives, etaye les differences entre les cadres nosologiques de l’ACG entre les formes « craniales » et « extra-craniales » dont les pronostics et les complications au cours de l’evolution sont distinctes.

La Presse Medicale - In Press.Proof corrected by the author Available online since mardi 3 mars 2015

Background Cogan syndrome is mainly treated with steroids. We aimed to determine the place of DMARDs and biologic-targeted treatments. Patients and methods We conducted a French nationwide retrospective study of patients with Cogan syndrome (n = 40) and a literature review of cases (n = 22) and analyzed the efficacy of disease-modifying anti-rheumatic drugs (DMARDs) and tumor necrosis factor α (TNF-α) antagonists. Results We included 62 patients (31 females) (median age 37 years [range 2–76]. At diagnosis, 61 patients (98%) had vestibulo-auditory symptoms, particularly bilateral hearing loss in 41% and deafness in 31%. Ocular signs were present in 57 patients (92%), with interstitial keratitis in 31 (51%). The first-line treatment consisted of steroids alone (n = 43; 70%) or associated with other immunosuppressive drugs (n = 18; 30%). Overall, 13/43 (30%) and 4/18 (22%) patients with steroids alone and with associated immunosuppressive drugs, respectively (p = 0.8), showed vestibulo-auditory response; 32/39 (82%) and 15/19 (79%) ocular response; and 23/28 (82%) and 10/14 (71%) general response. Overall 61 patients had used a total of 126 lines of treatment, consisting of steroids alone (n = 51 lines), steroids with DMARDs (n = 65) and infliximab (n = 10). Vestibulo-auditory response was significantly more frequent with infliximab than DMARDs or steroids alone (80% vs 39% and 35%, respectively), whereas ocular, systemic and acute-phase reactant response rates were similar. Infliximab was the only significant predictor of vestibulo-auditory improvement (odds ratio 20.7 [95% confidence interval 1.65; 260], p = 0.019). Conclusion Infliximab could lead to vestibulo-auditory response in DMARDS and steroid-refractory Cogan syndrome, but prospective studies are necessary.

Introduction Pour evaluer l’efficacite du tocilizumab chez les patients atteints d’arterite de Takayasu (TA). Patients et methodes Nous avons realise une etude retrospective multicentrique de 46 patients avec TA traites par tocilizumab. Nous avons analyse les facteurs associes a la reponse au tocilizumab et l’incidence des complications vasculaires dans TA les patients traites avec le tocilizumab en comparaison avec ceux sous DMARDs. Resultats Quarante-six patients avec TA ont ete inclus, avec un âge mediane de 43 ans [29–54], et 35 de sexe feminin (76 %). Nous avons observe une diminution de NIH median (de 3 [2–3] au depart a 0 [0–1] et de 0 a 3 et 6 mois, respectivement ; p Conclusion Cette etude a l’echelle nationale montre que le tocilizumab est une alternative therapeutique interessante et pourrais reduire les complications vasculaires dans TA.

International audience; BACKGROUND: The aims of the present study were to determine both clinical manifestations and outcome of anti-PL7 patients with antisynthetase syndrome (ASS). METHODS: The medical records of 15 consecutive anti-PL7 patients with biopsy proven ASS were retrospectively analyzed without prior selection. RESULTS: Anti-PL7 patients exhibited polymyositis (n=14) and dermatomyositis (n=1); extra-pulmonary manifestations of ASS included: Raynaud's phenomenon (40%), mechanic's hands (33.3%), joint impairment (26.7%), pericardial effusion (20%) and esophageal/gastrointestinal involvement (20%). The outcome of myositis was as follows: remission/improvement (91.7%) and deterioration (8.3%). Fourteen patients (93.3%) experienced interstitial lung disease (ILD). ILD preceded ASS diagnosis (n=5), was identified concomitantly with ASS (n=8) and occurred after ASS diagnosis (n=1). Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n=1), progressive onset of lung signs (n=11) and asymptomatic patients exhibiting abnormalities consistent with ILD on PFT and HRCT-scan (n=2). No patient had resolution of ILD, whereas 64.3% and 35.7% experienced improvement and deterioration of ILD, respectively. ILD resulted in respiratory insufficiency requiring O2 therapy in 14.3% of cases. Two patients died. Predictive parameters of ILD deterioration were: DLCO

Introduction L’arterite a cellules geantes (ACG) est une vascularite non necrosante touchant les vaisseaux de gros calibre, caracterisee par la presence de cellules geantes. A l’inverse, les vascularites associees aux ANCA (VAA) sont des vascularites necrosantes touchant preferentiellement les vaisseaux de petit calibre. Toutefois, des VAA peuvent se reveler par une arterite temporale avec des manifestations evocatrices d’ACG. Dans des series de biopsies d’arteres temporales anormales, certaines se revelaient etre des VAA. La prise en charge therapeutique et le pronostic sont pourtant differents entre ces 2 entites. Materiels et methodes Nous avons realise une etude retrospective, nationale, multicentrique, observationnelle incluant des patients atteints d’une VAA (granulomatose avec polyangeite ou GPA, granulomatose eosinophilique avec polyangeite ou GEPA, polyangeite microscopique ou MPA) revelees par une symptomatologie d’arterite temporale. Les patients ont ete identifies par appel a observations suivie d’une analyse retrospective de dossiers. Resultats Quarante-quatre patients (20 hommes et 24 femmes, âge median 70,5 ans [54–89]) ont ete inclus. Le diagnostic d’arterite temporale et de VAA etait concomitant chez 16 patients (36 %), tandis que le diagnostic d’arterite temporale precedait celui de VAA dans 28 cas (64 %), avec un intervalle median de 15,2 mois (1,1–1354). Tous les patients repondaient aux criteres de classification de l’ACG selon l’American College of Rheumatology. Les manifestations cliniques au moment du diagnostic d’arterite temporale etaient : signes cephaliques dans 39 cas (89 %), dont cephalees (70 %), claudication de la mâchoire (45 %), hyperesthesie du scalp (45 %) et abolition d’un pouls temporal (16 %), signes generaux dans 36 cas (81 %), signes ophtalmologiques dans 7 cas (16 %), dont flou visuel dans 7 %, diplopie dans 5 % et cecite binoculaire dans 2 %, douleurs rhizomeliques dans 13 cas (30 %), et toux dans 12 cas (27 %). Les patients n’ayant pas de signes cephaliques presentaient tous des signes inflammatoires compatibles avec une ACG sur la biopsie de l’artere temporale (BAT). Trente patients (68 %) presentaient des le diagnostic d’arterite temporale des signes cliniques atypiques evoquant une VAA : signes ORL chez 13 patients (30 %), atteinte pulmonaire chez 10 patients (23 %), renale chez 8 patients (18 %), neuropathie peripherique dans 6 cas (13 %), signes digestifs chez 5 patients (11 %), signes cutanes chez 4 patients (9 %), atteintes oculaire et cardiaque dans 2 cas (5 %) chacun. La CRP etait elevee dans 98 % des cas (mediane 108,5 mg/L). Quand ils etaient realises au diagnostic de l’arterite temporale (n = 28), les ANCA etaient presents dans 89 % des cas, de specificite anti-MPO dans 60 % des cas et anti-PR3 dans 40 % des cas. La BAT etait anormale dans 26 cas mais presentait des signes de VAA (necrose, vascularite des petits vaisseaux) dans 9 cas (35 %). Concernant la VAA, il s’agissait d’une GPA le plus souvent (28 cas, 64 %), d’une PAM dans 14 cas (32 %) et d’une GEPA dans 2 cas (4 %). Vingt-quatre patients (55 %) etaient sous corticotherapie lors de la survenue de la VAA. Les manifestations au moment du diagnostic de VAA etaient : signes generaux dans 24 cas (55 %), atteinte ORL dans 22 cas (50 %), atteinte pulmonaire dans 20 cas (46 %), neuropathie peripherique dans 15 cas (34 %), atteinte renale, ophtalmique ou cephalique dans 12 cas (27 %) chacun, atteinte cutanee dans 8 cas (18 %), atteinte neurologique centrale dans 5 cas (11 %) et atteinte digestive dans 4 cas (9 %). Trois patients presentaient un pachymeningite (7 %). Au diagnostic de la VAA, les ANCA etaient realises chez tous les patients. Ils etaient positifs chez 39 patients (89 %), avec une specificite anti-MPO dans 25 cas, anti-PR3 dans 13 cas et sans specificite dans un cas. Une preuve histologique de la VAA etait disponible dans 27 % des cas dans un autre site que la BAT. Tous les patients ont recu une corticotherapie, et 37 (84 %) ont recu au moins un immunosuppresseur, incluant du cyclophosphamide (57 %), de l’azathioprine (27 %), du rituximab (27 %) ou du methotrexate (11 %). Apres un delai moyen de suivi de 39,7 mois, 12 patients avaient presente une rechute de la VAA (5 majeures et 7 mineures) et 5 patients sont decedes. Conclusion Devant un tableau d’arterite temporale, chez un patient de plus de 50 ans, une vascularite necrosante doit etre recherchee au meme titre qu’une ACG, notamment en cas de manifestations cliniques inhabituelles de l’ACG. Les ANCA doivent etre systematiquement recherches et la biopsie de l’artere temporale est preconisee.

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.

International audience; Objectives: The aim of the study was to compare clinical/imaging findings and outcome in patients with idiopathic (isolated aortitis, IA) and with giant cell arteritis (GCA)-related aortitis.Methods: Patients from 11 French internal medicine departments were retrospectively included. Aortitis was defined by aortic wall thickening > 2 mm and/or an aortic aneurysm on CT-scan, associated to inflammatory syndrome. Patients with GCA had at least 3 ACR criteria. Aortic events (aneurysm, dissection, aortic surgeries) were reported, and free of aortic events-survival were compared.Results: Among 191 patients with non-infectious aortitis, 73 with GCA and 44 with IA were included. Patients with IA were younger (65 vs 70y, p = 0.003) and comprised more past/current smokers (43 vs 15%, p = 0.0007). Aortic aneurisms were more frequent (38% vs 20%, p = 0.03) and aortic wall thickening was more pronounced in IA. During follow-up (median = 34 months), subsequent development of aortic aneurysm was significantly lower in GCA when compared to IA (p = 0.009). GCA patients required significantly less aortic surgery during follow-up than IA patients (p = 0.02). Mean age, sex-ratio, inflammatory parameters and free of aortic aneurism-survival were equivalent in patients with IA ≥ 60y when compared to patients with GCA-related aortitis.Conclusions: IA is more severe than aortitis related to GCA, with higher proportions of aortic aneurism at diagnosis and during follow-up. IA is a heterogeneous disease and its prognosis is worse in younger patients < 60y. Most patients with IA ≥ 60y share many features with GCA-related aortitis.

Objective Kawasaki disease (KD) is a vasculitis that mostly occurs in young children and rarely in adults. We analyzed the characteristics of adult-onset KD (AKD) in France. Methods We collected retrospective and prospective data for patients with a diagnosis of KD occurring after the age of 18 years. Cases were obtained via various French medical networks and identified from the international literature. Results We included 43 patients of AKD at 26 institution from 1992 to 2015, with mean (SD) age 30 (11) years (range 18–68) and sex ratio (M/F) 1.2; 34 patients met the American Heart Association criteria and 9 were incomplete AKD. The median time to diagnosis was 13 days (interquartile range 8–21). The main symptoms were fever (100%), exanthema (98%), changes in the extremities (91%), conjunctivitis (77%), oral cavity changes (89%), cervical adenitis (55%) and cardiac abnormalities (45%). Overall, 35% of patients showed large-vessel vasculitis: coronary vasculitis (26%) and coronary aneurysm (19%). Treatment was mostly intravenous immunoglobulins (79%) and aspirin (81%). Four patients showed myocardial infarction due to coronary vasculitis, but none were treated with IVIg because of late diagnosis. After a median follow-up of 5 months (range 1–117), persistent aneurysm was noted in 9% of cases. Damage was significantly lower with early treatment than late or no treatment (p = 0.01). Conclusion Given the high frequency of cardiac involvement and complications in this series of AKD, diagnosis and treatment should not be delayed, and early IVIg treatment seems to improve the outcome

In view of its localization, brain abscess (BA) usually requires medical and surgical care. A broad spectrum of bacteria is involved. Recent reports stress the increasing frequency of anaerobes, but their impact has not been well evaluated. A retrospective review was conducted of all episodes of documented BA admitted in a tertiary-care hospital over a 10 y period. BA due to anaerobic bacteria (group A) were compared with other cases (group B) to determine the frequency and eventual characteristics of BA with isolated anaerobic bacteria. Between 1991 and 2000, BA were diagnosed in 42 patients (28M, 14F, mean age 54.6 y). No differences in clinical features and laboratory findings were found between patients with BA caused by anaerobic (n = 22) and only aerobic (n = 20) bacteria. Using appropriate microbiological techniques, 41 anaerobic bacteria strains were isolated in 22 of 42 patients (52.4%) with BA. Anaerobic bacteria were associated with aerobic strains in 5 patients (12%), whereas in 17 patients (40.5%) only anaerobic strains were isolated in cerebral puncture cultures. The most frequently isolated species were Fusobacterium nucleatum (n = 14), Prevotella sp. (n = 8), Actinomyces sp. (n = 6) and Bacteroides sp. (n = 4). Compared with group B, group A had more cases of a single abscess (p = 0.03) and ear, nose and throat (ENT) as a source of infection (p = 0.04), and seemed to have a better outcome (p = 0.07). These results emphasize the important role that anaerobic bacteria play in BA. The presence of such pathogens must be evoked when faced with a single abscess, an ENT infection, or both. Therapy should take into account this high frequency.

Introduction Le syndrome de Cogan est une pathologie rare dont la prise en charge reste mal codifiee. L’objectif de l’etude etait de determiner l’efficacite de l’infliximab en comparaison aux DMARDs et la corticotherapie seule. Patients et methodes Nous avons mene une etude retrospective francaise nationale incluant des patients atteints du syndrome de Cogan (n = 40) et une revue de la litterature des cas (n = 22) en analysant l’efficacite des DMARDs et des anti-TNFa, en particulier l’infliximab. Les donnees etaient analysees en considerant les differentes lignes de traitements poolees pour tous les patients. Resultats Nous avons inclus 62 patients (31 femmes) (âge median de 37 ans [2–76]). Au moment du diagnostic, 61 patients (98 %) avaient des symptomes vestibulo-auditifs, avec une surdite bilaterale dans 41 %. Les signes oculaires etaient presents chez 57 patients (92 %), avec une keratite interstitielle dans le 31 (51 %). Le traitement de premiere ligne se composait de corticoides seuls (n = 43 ; 70 %) ou associes a d’autres immunosuppresseurs (n = 18 ; 30 %). Une reponse vestibulo-auditive etait note dans 32/39 (82 %) et 15/19 (79 %) avec corticoides seuls et associes a des immunosuppresseurs, une reponse oculaire dans 23/28 (82 %) et 10/14 (71 %) et une reponse systemique dans 13/43 (30 %) et 4/18 (22 %), respectivement (p = 0,8). Soixante et un patients avaient recu au total 126 lignes de traitement, consistant en corticoides seuls (n = 51 lignes), avec DMARDs (n = 65) et l’infliximab (n = 10). Une reponse vestibulo-auditive etait significativement plus frequente avec l’infliximab que DMARDs ou corticoides seuls (80 % contre 39 % et 35 %, respectivement), tandis que les taux de reponse systemique et oculaire etaient similaires. L’infliximab etait le seul facteur predictif d’amelioration vestibulo-auditif (odd 20,7 [intervalle de confiance a 95 % : 1,65 ; 260], p = 0,019). Conclusion Infliximab pourrait induire une reponse vestibulo-auditive a la difference des DMARDs dans le syndrome de Cogan, mais des etudes prospectives sont necessaires.

Introduction L’arterite a cellule geante (ACG) est associee a une aortite dans 45 a 65 % des cas. Au diagnostic, environ 80 % des aortites sont asymptomatiques. Cependant, au cours de l’evolution de l’ACG, le risque de developper un anevrisme de l’aorte thoracique est augmente (risque relatif jusqu’a 17,3). Ces anevrismes se compliquent dans un tiers des cas de dissection, avec un taux de mortalite de l’ordre de 50 %. Actuellement, aucun facteur ne permet de predire la survenue d’evenement aortique grave au cours du suivi. Plusieurs etudes suggerent que l’ACG a biopsie de l’artere temporale (BAT) negative a des caracteristiques phenotypiques differentes des ACG a BAT+, avec une prevalence moins elevee de signes « cephaliques » (cephalees, claudication de la mâchoire, troubles visuels) et une prevalence plus elevee d’atteinte arterielle des gros troncs. L’objectif de notre travail est de comparer l’aortite associee aux formes d’ACG a BAT negative a celle des formes a BAT positive. Patients et methodes Nous avons mene une etude multicentrique retrospective a partir d’une cohorte de 80 patients presentant une ACG et une aortite diagnostiquees depuis 2000. La positivite de la BAT etait definie par la presence d’un infiltrat inflammatoire parietal. L’aortite etait definie par un epaississement aortique circonferentiel > 2 mm, sans atherome adjacent. Les caracteristiques cliniques, biologiques, radiologiques et l’evolution ont ete comparees dans les deux groupes. Resultats Cinquante-six patients avaient une BAT+ et 24 avaient une BAT−. Le suivi median etait de 44 mois (2–173) dans le groupe BAT+ et de 51 mois (44–220) dans le groupe BAT−. Dans le groupe BAT−, l’âge au diagnostic d’ACG etait significativement plus faible que dans le groupe BAT+ (66,6 ± 8,8 ans versus 70 ± 6,7, p = 0,03), de meme que l’âge au diagnostic d’aortite (67 ± 8,9 ans versus 72 ± 7,4 ans, p = 0,01). Dans le groupe BAT+, on notait 57 % de rechutes, versus 50 % dans le groupe BAT− (p = 0,63). Au cours du suivi, 25 % des patients developpaient un anevrisme aortique dans le groupe BAT+, contre 12,5 % dans le groupe BAT− (p = 0,25). La mortalite etait de 7,1 % dans le groupe BAT+ versus 12,5 % dans le groupe BAT− (p = 0,66). La survie sans deces, sans rechutes, et sans apparition d’anevrysme aortique n’etait pas significativement differente entre les 2 groupes. Conclusion Dans cette etude, les patients presentant une aortite associee a une ACG avec BAT negative etaient plus jeunes au moment du diagnostic d’ACG. En revanche, le profil evolutif ne parait pas different en termes de mortalite, de rechute ou d’apparition d’anevrysme aortique selon que les patients ont une aortite de l’ACG a BAT+ ou a BAT−.

Introduction La maladie associee aux IgG4 (MAG4) est une maladie de systeme dont l’etiopathogenie reste inconnue. Elle est souvent diagnostiquee devant l’existence de tumefactions d’organes. Le diagnostic doit etre confirme par l’histologie comportant une etude immuno-histochimique qui quantifie la presence de plasmocytes IgG4+. Les criteres diagnostiques de 2012 prennent egalement en compte l’elevation des IgG4 seriques [1] . Apres la pancreatite, dont elle est l’atteinte historique, les pseudotumeurs inflammatoires orbitaires et les dacryoadenites font partie des manifestations cliniques frequentes. L’objectif de cette etude est d’evaluer les caracteristiques demographiques, clinico-biologiques et la reponse aux traitements des patients atteints d’orbitopathies associees aux IgG4. Patients et methodes Il s’agit d’une etude retrospective descriptive issue des observations du recueil national francais des MAG4. Les criteres d’inclusions etaient : la reponse aux criteres CDC d’une MAG 4 definie ou probable [2] ; l’existence d’une atteinte orbitaire de type pseudotumeur inflammatoire (PTI) orbitaire ou dacryoadenite au diagnostic ; la presence de donnees demographiques, clinico-biologiques et therapeutiques suffisantes. Resultats Parmi les 74 patients MAG4 analysables, 20 presentaient une atteinte orbitaire. Aucun resultat histologique n’etait disponible pour 3 d’entre eux. Parmi eux, un patient objectivait 4 atteintes d’organes et un taux d’IgG4 > 12 g/L. Dix-sept patients etaient donc inclus, representant environ 23 % des observations recueillies. La moyenne d’âge etait de 58 ans au diagnostic. Le sex-ratio H/F etait de 1,4/1. Cinq patients avaient des antecedents atopiques. Quatre-vingt-deux pour cent presentaient une MAG4 definie selon les criteres CDC. L’âge moyen a l’apparition des premiers symptomes etait de 53 ans. Le delai moyen de diagnostic etait de 73 mois [4–288 mois]. Les patients presentaient en moyenne 3,37 atteintes d’organes. Onze patients presentaient un PTI orbitaire, 11 une dacryoadenite. Cinq d’entre eux avaient une double localisation orbitaire. Parmi eux, aucun ne presentait de fibrose retro-peritoneale, mediastinale, mesenterique ni de pseudotumeur inflammatoire hepatique, pulmonaire, mammaire ou meningee. Soixante-quatre pour cent des patients presentaient des adenopathies cliniques ou decouvertes par l’imagerie. Six avaient une pancreatite auto-immune, 9 une sialadenite. Onze sur 17, soit 64,7 %, possedaient un taux d’IgG4 seriques > 1,35 g/L au diagnostic ou au cours de l’evolution. Le taux moyen de CRP etait a 21,65 mg/L [extremes 3–64 mg/L]. Tous ont beneficie d’une corticotherapie entre 0,5 et 1 mg/kg/j avec une cortico-sensibilite pour 15/15 ; efficacite non renseignee pour 2 patients. Cinq presentaient une rechute, locale ou a distance, a la decroissance ou a l’arret de la corticotherapie. Deux patients ont beneficie d’une cure de rituximab avec une reponse clinique, et biologique ainsi qu’une reponse radiologique pour l’un des deux. Conclusion Un quart des patients MAG4 presentent une orbitopathie ce qui en fait une atteinte frequente de la maladie. Les patients issus du recueil de cas national semblent presenter des caracteristiques proches des donnees rapportees dans la litterature. Ces atteintes presentent une importante cortico-sensibilite avec, cependant, un risque de rechute dans 1 cas sur 3. Des etudes prospectives sont necessaires pour confirmer ces donnees.

Summary Objectives The aim of the present study was to assess the outcome in anti-PL12 patients with antisynthetase syndrome (ASS). Methods The medical records of anti-PL12 (n = 5) patients with ASS were retrospectively analyzed without prior selection. To exclude false-positive patients, we included patients who were successively tested positive for anti-PL12 antibody at least twice by immunodot and/or Western blot. Results Anti-PL12 patients experienced: myositis (n = 2), Raynaud's phenomenon (n = 2), mechanic's hands (n = 1), joint impairment (n = 4), digestive involvement (n = 2), and interstitial lung disease (ILD) (n = 4). The two patients with myositis exhibited deterioration of muscle manifestations despite therapy. As regards outcome of ILD, patients developed resolution (n = 1), stabilization (n = 1) or deterioration (n = 2) of pulmonary status. One patient died of pyogenic pneumonia. Conclusion Our series underscores that the presence of anti-PL12 antibody is associated with a particular phenotype of ASS characterized by: (1) less frequent although severe/steroid refractory myositis; (2) less common mechanic's hands and calcinosis cutis; (3) both frequent and severe ILD. Taken together, our findings suggest that PM/DM patients should routinely undergo the search for anti-PL12 antibody as this autoantibody appears to impact patients’ prognosis. Furthermore, ILD patients with anti-PL12 antibody should routinely undergo clinical screening for underlying ASS.

International audience; The aims of the present study were to: compare the characteristics between antisynthetase syndrome (ASS) patients with anti-Jo1 antibody and those with anti-PL7/PL12 antibody. The medical records of 95 consecutive patients with ASS were reviewed. Seventy-five of these patients had anti-Jo1 antibody; the other patients had anti-PL7 (n=15) or anti-PL12 (n=5) antibody. At ASS diagnosis, the prevalence of myalgia (p=0.007) and muscle weakness (p=0.02) was significantly lower in the group of anti-PL7/PL12-positive patients than in those with anti-Jo1 antibody; median value of CK (p=0.00003) was also lower in anti-PL7/PL12 patients. Anti-Jo1 positive patients developed more rarely myositis resolution (21.3% vs. 46.2%); in addition, the overall recurrence rate of myositis was higher in anti-Jo1 positive patients than in patients with anti-PL7/PL12 antibody (65.9% vs. 19.4%). Anti-Jo1-positive patients, compared with those with anti-PL7/PL12 antibody, more often experienced: joint involvement (63.3%vs. 40%) and cancer (13.3% vs. 5%). By contrast, anti-PL7/PL12 positive patients, compared with those with anti-Jo1 antibody, more commonly exhibited: ILD (90% vs. 68%); in anti-PL7/PL12 positive patients, ILD was more often symptomatic at diagnosis, and led more rarely to resolution of lung manifestations (5.6% vs. 29.4%). Finally, the group of anti-PL7/PL12 positive patients more commonly experienced gastrointestinal manifestations related to ASS (p=0.02). Taken together, although anti-Jo1 positive patients with ASS share some features with those with anti-PL7/PL12 antibody, they exhibit many differences regarding clinical phenotype and long-term outcome. Our study underscores that the presence of anti-Jo1 antibody results in more severe myositis, joint impairment and increased risk of cancer. On the other hand, the presence of anti-PL7/PL12 antibody is markedly associated with: early and severe ILD, and gastrointestinal complications. Thus, our study interestingly indicates that the finding for anti-Jo1 and anti-PL7/PL12 antibodies impacts both the long-term outcome and prognosis of patients with ASS.

IgG4-related systemic disease is now recognized as a systemic disease that may affect various organs. The diagnosis is usually made in patients who present with elevated IgG4 in serum and tissue infiltration of diseased organs by numerous IgG4+ plasma cells, in the absence of validated diagnosis criteria. We report the clinical, laboratory, and histologic characteristics of 25 patients from a French nationwide cohort. We also report the treatment outcome and show that despite the efficacy of corticosteroids, a second-line treatment is frequently necessary. The clinical findings in our patients are not different from the results of previous reports from Eastern countries. Our laboratory and histologic findings, however, suggest, at least in some patients, a more broad polyclonal B cell activation than the skewed IgG4 switch previously reported. These observations strongly suggest the implication of a T-cell dependent B-cell polyclonal activation in IgG4-related systemic disease, probably at least in part under the control of T helper follicular cells.

Introduction La maladie de Kawasaki (MK) ou syndrome adeno-cutaneo-muqueux est une vascularite touchant les arteres de moyen et petit calibre qui survient principalement chez les enfants et exceptionnellement les adultes. Des complications cardiaques ou vasculaires peuvent survenir lors de la phase initiale ou plus tardivement notamment en l’absence de traitement precoce. Nous rapportons ici les donnees d’un observatoire de la MK de l’adulte (MKA). Patients et methodes Depuis juillet 2014, cet observatoire multicentrique a inclus des MKA certaines selon les criteres internationaux (fievre inexpliquee durant plus de 5 jours et 4 criteres parmi exantheme, modification des muqueuses orales, conjonctivite, adenopathies cervicales et modification des extremites), et egalement des MKA consideres comme probables en cas d’association d’une fievre inexpliquee, d’une atteinte arterielle et 3 des criteres ou d’une fievre inexpliquee avec 3 criteres, apres elimination des diagnostics differentiels. Resultats Vingt quatre patients ont ete inclus avec un âge median de 29 ans (22–39) et un sexe ratio (H/F) a 2,42. Douze avaient une MK certaine, 9 une MK probable devant l’atteinte cardiaque ou vasculaire febrile associee a 3 autres criteres, et 3 une MK probable sur l’association d’une fievre persistante avec 3 criteres en l’absence d’autres etiologies retrouvees. Le delai diagnostique median etait de 13 jours (10–20,5). Les patients provenaient de differents services : medecine interne ( n = 17), cardiologie ( n = 4), maladies infectieuses ( n = 2) et dermatologie ( n = 1). Les principales manifestations etaient : une fievre a 39°C–40°C (100 %), une modification des extremites ( n = 21, 87,5 %), des desquamations en doigt de gants ( n = 19, 79 %) survenant apres une mediane de 15 jours (11–22), un exantheme diffus ( n = 22, 92 %), une conjonctivite ( n = 16, 66 %), une cheilite ( n = 15, 63 %), une langue framboisee ( n = 11, 46 %), des adenopathies ( n = 10, 42 %), des anomalies cardiaques ( n = 11, 46 %) dont un choc cardiogenique ( n = 1), une myo-pericardite ( n = 3) et une insuffisance cardiaque gauche ( n = 1), des signes digestifs ( n = 11, 46 %) et des arthralgies ( n = 10, 42 %). La mediane de la CRP etait a 228 mg/L (166–311), les ASAT a 68 UI/L (51–139), les ALAT a 125 UI/L (69–190), les plaquettes a 372 G/L (209–630) et les leucocytes a 16 100/mm 3 (8325–20 875). Une atteinte cardiaque etait recherchee chez 23 patients (96 %) par la realisation de 20 echocardiographies trans thoracique, 6 coro scanners, 5 coronarographies, 2 IRM cardiaques et 2 epreuves d’effort. Onze patients (46 %) presentaient une atteinte vasculaire arteritique (peripherique ou coronarienne). Les atteintes arterielles etaient : des anevrismes coronaires ( n = 8 ; 33 %), une arterite coronaire ( n = 10 ; 42 %) et une arterite peripherique ( n = 2 ; 8,3 %) : aux membres inferieurs avec ischemie aigue ( n = 1, 4,2 %) et sur les arteres splenique et gastro duodenale compliques d’anevrismes et d’infarctus spleniques ( n = 1, 4,2 %). Les patients recevaient : des immunoglobulines par voie intra veineuse (IgIV) ( n = 17 ; 71 %) : 1cure unique ( n = 15, 88 %), 2 cures ( n = 1, 6 %), plusieurs cures ( n = 1, 6 %) entrainant une apyrexie apres une mediane de 2 jours (1–3), de l’aspirine ( n = 21 ; 88 %) a dose anti-inflammatoire ( n = 9 ; 38 %) et a dose anti-agregante ( n = 15 ; 63 %). Seize patients ont ete traites par l’association IgIV et aspirine ( n = 67 %). Ils recevaient aussi des corticoides ( n = 3 ; 12,5 %), des AINS ( n = 2 ; 8,3 %) et de la colchicine ( n = 1 ; 4,2 %). Un traitement a visee cardiaque a ete prescrit ( n = 7 ; 29,2 %) incluant une anticoagulation curative ( n = 4 ; 16,7 %), des IEC ( n = 3 ; 12,5 %), un beta bloquant ( n = 3 ; 12,5 %), du spironolactone ( n = 2 ; 8,3 %), du furosemide ( n = 1 ; 4,2 %) et de l’amiodarone ( n = 1 ; 4,3 %). Concernant l’evolution, il persistait une insuffisance cardiaque au dernier suivi ( n = 1 ;4,3 %), un anevrisme apres 6 mois ( n = 5 ; 20,8 %) et un anevrisme lors du dernier suivi ( n = 3 ; 12,5 %). De l’aspirine etait poursuivie au dernier suivi ( n = 9 ; 37,5 %). Conclusion La MKA est une affection rare qui peut se compliquer et engager le pronostic vital a court ou long terme et laisser des sequelles irreversibles. Elle necessite un diagnostic et une prise en charge en urgence. Une etude plus approfondie des caracteristiques cliniques, therapeutiques et evolutives est en cours. Un recueil dans le registre national KAWANET est mis en place avec comparaison des formes de l’adulte avec celles de l’enfant.

International audience; Unlabelled - Revascularization aims at improving walking ability in patients with arterial claudication. The highest measured distance between 2 stops (highest-MDCW), the average walking speed (average-WSCW), and the average stop duration (average-DSCW) can be measured by global positioning system, but their evolution after revascularization is unknown.We included 251 peripheral artery diseased patients with self-reported limiting claudication. The patients performed a 1-hour stroll, recorded by a global positioning system receiver. Patients (n = 172) with confirmed limitation (highest-MDCW

Infections due to subspecies arizonae Salmonella enterica are rare. These infections are transmitted by reptiles. We report the case of S. arizonae urinary tract infection that occurred in an immunocompetent woman.An 82 year-old woman was admitted for confusion. A urinary tract infection due to Salmonella arizonae was diagnosed. No neurological, iatrogenic or metabolic cause could explain the confusion. Treatment with ciprofloxacin was given. The confusion and the infectious syndrome disappeared.The prevalence of infections is probably underestimated because the digestive problems they generate are usually benign.