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Background Patients treated with biologic DMARDs have to be aware of the specific adverse events and have to be able to manage their treatments in case of infectious disease. Therapeutic Patient Education aims to enable patient to acquire these safety skills. Objectives To assess the impact of Therapeutic Patient Education on safety skills and management of infectious events in patients treated with biologic DMARDs in rheumatology. Methods Bi-centric analytical study comparing safety skills and infectious events between two cohorts of patients (TPE and TPE-naive). Safety skills were assessed by an auto-assessment called “Biosecure”, rated on a scale from 0 to 100. This assessment explores several dimensions of treatment management and was validated for assessment of safety skills in patients treated with biologic DMARDs in rheumatology in a preliminary study. Infectious events and their management were self-reported by patients. A cluster analysis aimed to separate patients into working group based on their shortcomings (on the basis of their answers to the Biosecure Assessment). Results 414 patients answered the assessment. The median Biosecure Score was 70.98/100 (Q1Q3: 60.97–84.63) 47% attended Therapeutic Patient Education. The median Biosecure Score was significantly higher in the TPE group than in the TPE-naive group (74.88 versus 67.20/100; p Cluster analysis based on Biosecure assessment separated patients into 3 level groups but failed to identify specific patient profiles. Conclusions Therapeutic Patient Education could provide better safety skills and better treatment management in patients treated with biologic DMARDs in rheumatology. Prospective studies may confirm the impact of TPE on treatment management during infectious events. Further studies may assess the impact of TPE on incidence of serious infectious events. References [1] Orefice D, Beauvais C, Gossec L, Flipon E, Fautrel B, Marguerie L, et al. Cross-sectional study of self-care safety skills in 677 patients on biodrugs for inflammatory joint disease. Jt Bone Spine Rev Rhum. dec 2014;81(6):502. [2] Rat A-C, Fautrel B, Flipon E, Gossec L, Marguerie L, Nataf H, et al. Factors associated with knowledge and safety skills of arthritis patients receiving biologics: A survey of 677 patients. Joint Bone Spine. Disclosure of Interest None declared

International audience

Background The efficacy of antitumour necrosis factor alpha (anti-TNF-α) treatment is well recognised in rheumatoid arthritis (RA) but remains controversial in systemic lupus erythematosus (SLE). Therefore, the role of anti-TNF-α treatment in ‘Rhupus’, a disease sharing features of RA and SLE, is still debated. Objective To evaluate the efficacy and tolerance of anti-TNF-α in patients with rhupus. Methods Fifteen patients with rhupus with Disease Activity Score 28 (DAS 28) >3.2 despite conventional disease-modifying anti-rheumatic drugs were included in an open-label study. Patients were monitored at months (M) 3, 6, 12, 24 and 60 with SLE Disease Activity Index (SLEDAI) and DAS 28. Statistical analyses were performed using Bayesian methods and Prob >97.5% was considered significant. Results Twelve patients were treated with etanercept for a median duration of 62.5 (range: 6–112) months and three patients by adalimumab during 36.0 (range: 4–52) months. At baseline, median DAS 28 and SLEDAI were 5.94 (4.83–8.09) and 6 (4–8), respectively. DAS 28 and SLEDAI decreased significantly after 3 months, respectively, to 3.70 (1.80–6.42) and 4 (0–6) (Prob >99.9%, for both). These changes persisted at M6, M12, M24 and M60 (Prob >99.9%, for all). Median prednisone dose decreased significantly from 15 (5–35) mg/day to 5 (0–20) mg/day after 6 months and over the follow-up (Prob >99.9%, for all). Tolerance was acceptable, with a severe infection rate of 3.0 per 100 patient-years. Conclusion This pilot study suggests that anti-TNF-α is effective in patients with rhupus with refractive arthritis and has an acceptable safety profile. journal article 2017 2017 12 29 imported

Background Coexisting fibromyalgia (FM) in axial spondyloarthritis (axSpA) can represent therapeutic challenges, particularly when evaluating the treatment effect of biologics (i.e. TNF alpha blockers (TNFb)). Indeed, since FM patients often report high levels of pain and disability there is the risk of classifying such patients as refractory to TNFb (ie, as not reaching a significant improvement in disease activity). Objectives To evaluate the impact of concomitant FM on the TNFb treatment effect in axSpA. Methods Design: Prospective observational national study with 2 visits 3 months apart (baseline and 12 weeks after TNFb initiation) (Predict-SpA study ClinicalTrials.gov: NCT03039088). Patients: axSpA patients (diagnosis according to treating rheumatologist) initiating a TNFb. Data collection: the FiRST questionnaire (Fibromyalgia Rapid Screen Test) which screens for FM, patients and disease characteristics and effectiveness measures (e.g. ASAS response components). Statistical analysis: FM positive screening was defined by a FIRST score ≥5/6; the primary efficacy outcome was the ASAS 40. Non-responder imputation and baseline observation carried forward imputation (for binary and continuous outcome variables, respectively) was performed. Impact of FM on the TNFb treatment effect was evaluated by multivariable logistic regression, with ASAS 40 as the dependent variable and FM as the independent variable; were also included in the model other factors previously reported in the literature as associated with treatment efficacy (i.e. X-ray and MRI sacroiliitis, abnormal CRP (>6mg/L), HLA B-27, smoking status, previous TNFb exposure, age Results Among the 527 patients enrolled in the study, 508 patients were analysed. Mean age was 41.4 (±11.6), 237 (46.7%) were women, with a 6.1±8.5 mean disease duration. Among them, 192 (37.8%) were screened as FM by the FiRST questionnaire. Overall efficacy of the TNFb was good (ASAS40, 201/508 (39.6%)) though 50 patients (9.8%) patients discontinued the TNFb before the follow-up visit and were considered as non-responders. Patients with FM presented less frequently an ASAS 40 response (87/192 (45.3%) vs 171/316 (54.1%), for the FM vs non-FM groups according to the FIRST definition. Presence of FM was independently associated with poorer ASAS40 response (adjusted odds ratio, OR =0.5 [95% CI 0.3 – 0.8]) while X-ray sacroiliitis (1.8 [1.2 – 2.8]), abnormal CRP (1.6 [1.0 - 2.4]) and absence of previous exposure to TNFb (1.7 [1.1 – 2.6]) were found to be associated with an ASAS40 response. Conclusions This study 1) confirms the “conventional” predisposing factors of TNFb treatment response such as X-ray sacroiliitis, abnormal CRP and absence of previous exposure to TNFb; and 2) suggests that concomitant FM influences treatment response. FM deserves to be screened in axSpA, in particular in case of a decision to initiate a TNFb therapy. References Perrot S, Bouhassira D, Fermanian J; Cercle d9Etude de la Douleur en Rhumatologie. Development and validation of the Fibromyalgia Rapid Screening Tool (FiRST). Pain. 2010;150:250–6. Acknowledgements The PredicSpA study was supported by MSD thanks to an unrestricted grant. Disclosure of Interest None declared

Background Fibromyalgia (FM) can be a comorbid condition in axial spondyloarthritis (axSpA). FM screening questionnaires and classification criteria fulfillment may demonstrate different prevalence and patients9 characteristics may differ. Objectives To evaluate frequency of comorbid FM, and the differences between axSpA patients with/without comorbid FM according to screening (FiRST) and classification tools (ACR1990 FM criteria). Methods A multicenter national study involving 39 rheumatology centers in France included 519 patients with axSpA starting an anti-TNF treatment (PredictSpA study; ClinicalTrials.gov: NCT03039088). Patients (pts) were screened for FM with the FiRST questionnaire and classified as FM by ACR 1990 criteria. Demographic characteristics, comorbidities, axSpA characteristics, CRP and imaging were recorded in all patients. Kappa coefficient (and its 95% CI) was calculated to compare FiRST and ACR1990 criteria. Baseline characteristics associated with FM were evaluated by univariable and multivariable logistic regression (including the variables with a p value Results In the 519 pts (females: 46%, age: 42±12 years, mean BASDAI 5.7±2.0, mean ASDAS-CRP 3.3±0.9), a comorbid FM was screened in 38% and diagnosed in 16% of pts. Agreement between FiRST and ACR1990 was poor, with a Kappa coefficient of 0.2 [0.2–0.3]. In the multivariable analysis, comorbid FM (by the FiRST questionnaire) in axSpA patients was independently associated with a lower education level, more sick leave, more heel pain, more frequent second line anti-TNF. Comorbid FM defined by the ACR1990 classification criteria was independently associated with heel pain and inversely associated with B27 haplotype. (Table) Conclusions FM is frequently associated with active axSpA, with poor correlation between screening (FiRST) and diagnosis (ACR1990) test. Several characteristics are associated with comorbid FM, depending on FM classification and screening tools. References Perrot S et al. Development and validation of the Fibromyalgia Rapid Screening Tool (FiRST). Pain. 2010;150:250–6. Acknowledgements This study was conducted thanks to an unrestricted grant from MSD. Disclosure of Interest None declared

Background Five different sub-cutaneous TNF alpha blockers (TNFb) are available for the treatment of NSAID-refractory and active axial spondyloarthritis (axSpA). The efficacy of these drugs in axSpA has been well reported in several RCT and real-life studies separately or as a group, but only very few head-to-head studies have compared each molecule9s treatment effect in axSpA. Also, some data suggest that TNFb treatment effect in patients failing to a first TNFb is poorer than in TNFb-naive patients, but some other suggest that response to a second TNFb might be comparable. Objectives to describe TNFb prescription and treatment response of each TNFb, and to compare treatment response in TNFb- naive/not naive axSpA patients in a real-life setting. Methods Prospective, multicenter, usual care study with 2 visits (baseline and 12 weeks after TNFb initiation, Predict-SpA study, ClinicalTrials.gov: NCT03039088)). Patients: axSpA patients (diagnosis according to treating rheumatologist) initiating a TNFb. Data collection: Patients and disease characteristics at baseline. Previous exposure to TNFb was collected. Disease activity and function measures were collected at both visits. The choice of the TNFb to be started during the trial was at the treating rheumatologist9s discretion. Statistical analysis: effectiveness was defined by the BASDAI50 response. Non-responder imputation and baseline observation carried forward imputation (for binary and continuous outcome variables, respectively) were performed for patients who discontinued the TNFb treatment between baseline and the follow-up visit. Results Among the 527 patients enrolled in the study, 508 patients were included in the analysis (1 patients was excluded due to missing data on all disease activity measures at baseline, and 18 patients were excluded due loss of follow-up between baseline and the follow-up visit). Mean age was 41.4 (±11.6), 237 (47%) were women, with a 6.1±8.5 mean disease duration. 377/508 (74%) were TNFb-naive while 66 (13%), 35 (7%),19 (3.7%),8 (2%) and 3 (1%) had previously received, 1,2, 3, 4 and >4 TNFb, respectively. In the whole study population the most frequently prescribed TNFb was etanercept (197 (39%)), followed by adalimumab (131 (26%)), golimumab (88 (17%)), infliximab 53 (10%) and certolizumab (39 (8%)); the same order was observed in TNFb naive patients; however, in patients previously exposed to TNFb, the most frequently prescribed TNFb was infliximab (31 (24%)) followed by golimumab (29 (22%)), adalimumab (29 (22%)), certolizumab (22 (17%)) and etanercept (20 (15%)). BASDAI50 responses were comparable for all TNFb molecules: 52%, 52%, 48%, 53% and 49% for etanercept, adalimumab, golimumab, infliximab and certolizumab, respectively. BASDAI 50 response was comparable in patients TNFb-naive and patients previously exposed to only 1 previous TNFb (53% vs. 52%, for naive vs. non-naive patients) but significantly decreased after (i.e. 37% and 36.% for patients previously exposed to 2 and to 3 or more TNFb) Conclusions this study suggests that the TNFb treatment response is similar across the different available molecules but this treatment response decreased in case of a previous use of two TNFb. Acknowledgements This study was conducted thanks to an unrestricted grant from MSD Disclosure of Interest None declared

Background In axSpA, treatment decisions are mainly based on patient-reported symptoms: recommendations are to initiate TNFi in patients with active disease and with physician conviction that treatment is needed (ref). However, the attribution of symptoms to inflammation is difficult to establish in axSpA. Objectives The objective of the present analysis was to explore the link between physician-attributed causality for symptoms and treatment response to TNFi. Methods The PredictSpA study (ClinicalTrials.gov: NCT03039088) was a longitudinal observational multicenter study in France in 2015. Patients with physician-defined definite axSpA and starting a TNFi treatment were included, a TNFi was prescribed according to usual practice and efficacy was assessed at 12 weeks by BASDAI50 response. At baseline, symptoms levels including BASDAI and ASDAS were collected and the physician evaluated the causality of symptoms by answering the following 3 questions: how convinced are you that the symptoms of this patient are due to (A) inflammatory axSpA activity (B) to axSpA severity (eg syndesmophytes, kyphosis) and NOT to disease activity and (C) to other diseases and NOT axSpA. Each question was assessed 0–10 (not convinced at all to absolutely convinced). The link between a score ≥4/10 on each of the 3 questions and BASDAI50 response was assessed by univariate logistic regression. Patients interrupting the TNFi before 3 months were considered as non-responders and missing data were imputed using non-responder imputation. Results In all, 519 patients were included and 508 had data over 3 months: mean age 41.3 (SD 11.6) years, mean disease duration 6.1 (SD 8.4) years, 237 (46.7%) were women, 424 (83.5%) satisfied the ASAS criteria for axSpA of whom 379 (74.6%) were in the imaging arm and 45 (8.9%) in the clinical arm. Symptom levels were high: mean BASDAI was 5.7 (SD 1.8) and mean ASDAS-CRP was 3.3 (SD 0.9) with only 6 (1.2%) patients in inactive disease state according to ASDAS. The physician-attributed causality of symptoms was mostly related to inflammatory activity: mean scores for (A), (B) and (C) were respectively, 7.4 (SD 2.0), 2.3 (SD 2.5) and 2.1 (SD 2.2). When physicians attributed causality to non-axSpA (score (C) ≥4/10), BASDAI50 response was less frequent: 45/118 (38.4%) vs 213/390 (54.6%), odds ratio 0.5 [95% CI 0.3, 0.8]. Conclusions In axSpA patients starting a TNFi with high symptom levels, physician-attributed causality of symptoms was mainly related to inflammatory activity of the axSpA. When physician-attributed causality was more oriented towards non-axSpA causes, BASDAI50 response after 3 months of a TNFi was lower. This confirms the validity of the ASAS-EULAR recommendations for starting a TNFi which rest on a level of symptoms but associated to physician conviction of the indication. References van der Heijde D, et al. 2016 update of the ASAS-EULAR management recommendations for axial spondyloarthritis. Ann Rheum Dis. 2017 [Epub ahead of print]. Acknowledgements This study was conducted thanks to an unrestricted grant from MSD. Disclosure of Interest None declared

Background Fibromyalgia (FM) is a frequent comorbid condition in axial spondyloarthritis (axSpA). It is not known how FM comorbidity may respond to the management of SpA, and especially to anti-TNF agents. Objectives To evaluate the change of comorbid FM status of axSpA patients starting an anti-TNF treatment. Methods A prospective multicenter national study involving 39 rheumatology centers in France, analyzing 519 patients with axSpA requiring anti-TNF therapy (ClinicalTrials.gov: NCT03039088). Patients were screened for FM with the FiRST questionnaire before and after 3 months of anti-TNF. Kappa coefficient was calculated to determine the agreement of the FiRST at M0 and M3. Response to anti-TNF (BASDAI50 response was compared according to positive screening for FM or not, at both time-points using chi2 tests. Results Of the 519 enrolled pts, 504 (with complete data on the FiRST questionnaire) were analyzed at M3. A positive screening for comorbid FM was found in 192 pts (38%) at M0 and in 127 (25%) pts at M3. Correlation between FiRST at M0 and M3 was weak with a Kappa coefficient correlation of 0.4 [0,3 - 0,5]. Four groups were identified: group [+/+] with comorbid FM at M0 and M3: N=93 (18%; group [+/-] with comorbid FM at M0 but not at M3: N=99 (20%); group [-/-] without comorbid FM at M0 and M3: N=278 (55%); group [-/+] without comorbid FM at M0 but at M3: N=34 (7%) Changes in the status of comorbid FM (disappearance or appearance) was observed in 134 pts (26%). In the 193 pts with baseline comorbid FM, after 3 months of anti-TNF treatment, comorbid FM was no longer found in 99 (51%)%.Efficacy at M3 was significantly better, according to BASDAI 50, in patients without comorbid FM at M3 (Table). Conclusions There is a high frequency of comorbid FM screened by the FiRST in active axSpA, decreased by 51%f after 3 months of anti-TNF treatments. Persistence of FM after 3 months of anti-TNF treatment is associated with lower anti-TNF response. Further studies are required to analyze the impact of screening FM before starting anti-TNF therapy in axSpA. References Perrot S, et al. Development and validation of the Fibromyalgia Rapid Screening Tool (FiRST). Pain. 2010;150:250–6. Acknowledgements This study was conducted thanks to an unrestricted grant from MSD. Disclosure of Interest None declared

Background Currently, the European Medicine Agency (EMA) is recommending the use of anti-TNF in case of active NSAID refractory axial (ax) spondyloarthritis (SpA) only in the presence of either structural radiographic damage of the sacroiliac Joints (SIJ) or objective signs of inflammation (either elevated CRP or inflammation at MRI-SIJ) probably mainly because of the fear of a concomitant fibromyalgia (FM). Objectives To evaluate the interaction of a concomitant FM on the anti-TNF response with regard to the application of the EMA recommendation Methods Study design: Prospective, multicenter, longitudinal (12 weeks) open trial (Predict-SpA, ClinicalTrials.gov: NCT03039088). Patients (pts): axSpA requiring an anti-TNF. Data collected: At baseline, 1/Radiographic SIJ damage 2/ MRI-SIJ inflammation 3/ Elevated CRP 4/ FM according to the FiRST (Fibromyalgia Rapid Screen Test) questionnaire. At baseline and after 12 weeks of therapy: BASDAI. Statistical analysis: Non Responder Imputation (NRI), Baseline Carried Forward Technic (BOCF) in case of missing data. Interaction test between the presence of FM and the fulfillment of the EMA recommendation (defined by the presence of either structural radiographic damage of the SIJ or objective signs of inflammation (either elevated CRP or inflammation at MRI-SIJ)) on the anti TNF treatment response (BASDAI50) Results Of the 508 enrolled pts (females: 46.7%, age: 41.4+11.6 years old, HLA B27 positive: 57.5%, X-Ray sacroiliitis: 53.2%, inflammation at MRI-SIJ: 52.0%), only 59 had no structural damage at SIJ-X-Rays and no inflammation at MRI-SIJ (12%). A concomitant FM was noticed in 42% vs 37% of pts treated in accordance vs not with the EMA recommendation, respectively (p=0.44). The anti-TNF treatment effect was higher in the groups of pts treated in accordance (vs not) with the EMA recommendation (BASDAI 50: 52% vs 39%, respectively; p=0.054). There was a trend (but not statistically significant) suggesting an interaction of the concomitant FM on the anti TNF treatment response with regard to the application of the EMA recommendation (interaction p value=0.140). In the 59 pts not treated in accordance with the EMA recommendation, the treatment effect in the sub-groups of pts without ( vs with) concomitant FM was 38% vs 40% respectively, p=0.891. In the 449 pts treated in accordance with the EMA recommendation, the treatment effect in the sub-groups of pts without ( vs with) concomitant FM was 56% vs 46%, p=0.042). Conclusions This study suggests that 1/ French rheumatologists are applying the EMA recommendation in daily practice 2/ these recommendations result in a better outcome in terms of short term symptomatic treatment effect. In this study, concomitant FM was not more frequently observed in patients without ( vs with) objective sign of structural damage or inflammation and the impact of a concomitant FM was not more pronounced (or even lower) in pts without (vs with) objective sign of structural damage or inflammation. References Perrot S, et al. Development and validation of the Fibromyalgia Rapid Screening Tool (FiRST). Pain.2010;150:250–6. Acknowledgements This study was conducted thanks to an unrestricted grant from MSD. Disclosure of Interest None declared

Resume Introduction Le tocilizumab (TCZ) est un anticorps anti-recepteur de l’IL-6 humanise, indique dans le traitement de la polyarthrite rhumatoide (PR) active, moderee a severe, la maladie de Still de l’adulte, la maladie de Castleman et, de facon plus recente, l’arthrite juvenile idiopathique dans sa forme systemique. De meme que les anti-TNFα, le rituximab et plus rarement l’abatacept, le TCZ est capable d’induire des reactions cutanees paradoxales de type psoriasique essentiellement a type de pustulose palmo-plantaire. Observation Nous rapportons l’observation d’une patiente suivie pour un rhumatisme psoriasique qui developpait sous anti-TNFα puis sous tocilizumab une eruption psoriasiforme palmo-plantaire. Conclusion Le mecanisme de ces reactions reste imparfaitement compris, mais des reactions croisees avec d’autres biotherapies sont observees.

Las calcinosis y osificaciones cutaneas se caracterizan por la presencia de depositos dermicos o hipodermicos de hidroxiapatita. Bajo estos dos terminos se agrupan enfermedades muy diversas y heterogeneas, sin relaciones aparentes entre ellas. En este articulo se ha adoptado la clasificacion propuesta por Cribier, que se basa en los mecanismos fisiopatogenicos conocidos o supuestos: calcinosis relacionadas con trastornos del metabolismo fosfocalcico, calcinosis exogenas o secundarias a una alteracion tisular y calcinosis idiopaticas. Por ultimo, se distinguen las osificaciones secundarias a una lesion cutanea previa, las asociadas a trastornos del metabolismo fosfocalcico y los osteomas primarios aislados o que acompanan a una enfermedad general.

Objective.Ischemic digital ulcers (DU) are frequent and severe complications of systemic sclerosis (SSc). The purpose of our study was to assess the effect of DU on hand disability and pain in patients with SSc.Methods.The Evaluation of the Impact of Recurrent Ischemic DU on Hand Disability in Patients with SSc (ECLIPSE) is a prospective, multicenter, noninterventional study with a 2-year followup. Patients with SSc who experienced at least 1 DU in the previous year and received bosentan therapy were included between October 2009 and March 2011. This cohort is described at the time of inclusion.Results.There were 190 patients (132 females) from 53 centers. Mean age ± SD was 43 ± 15 years at SSc diagnosis and 53 ± 15 years at inclusion. In 105 patients (56.2%), DU were the first non-Raynaud symptoms of SSc. The mean time interval between the occurrence of Raynaud phenomenon and the first DU episode was 6.6 ± 9.1 years. The mean numbers of active DU and fingers affected per patient for both hands were 2.3 ± 1.8 and 2.2 ± 1.6, respectively. Presence of active DU at inclusion was significantly associated with pain and impaired hand function: Visual Analog Scale for pain (0 to 10) was 6.2 ± 2.6 versus 2.5 ± 2.4 (p < 0.0001) and Cochin Hand Function Scale for hand disability (0 to 90) was 38 ± 20 versus 25 ± 19 (p < 0.0001), respectively.Conclusion.DU represent a major sign of SSc, often affecting multiple fingers and both hands. They are significantly associated with pain and hand disability.

Pour etre utilise en therapeutique, notamment pour la cicatrisation des plaies aigues et /ou chroniques comme les escarres, le miel doit presenter certaines caracteristiques : une contamination microbienne la plus limitee possible, une bonne capacite d’inhibition des germes habituellement rencontres en milieu hospitalier, un potentiel de cicatrisation et une stabilite garantie par une conservation adequate a l’abri de la lumiere, de la chaleur et de l’humidite des la mise en pot.

Lyme borreliosis is a multisystem infectious disease caused by a tick-transmitted spirochete, Borrelia burgdorferi.We describe two cases of unusual cutaneous manifestations of late Lyme borreliosis.A 64-year-old female patient presented with a history of multiple lesions of anetoderma which had developed within the past 9 months. She had no relevant history and mentioned that she had been bitten by a tick two years previously. The skin examination revealed multiple elliptic grouped atrophic macules [...]

Resume Introduction Les plaies chroniques (ulceres de jambe, escarres, maux perforants plantaires) representent un reel probleme de sante publique engendrant un cout economique et humain important. La dependance des personnes âgees et la configuration geographique regionale nous ont conduits a creer un outil informatique d’aide a la prise en charge des plaies chroniques en EHPAD afin de diminuer le nombre de transports aller-retour en ambulance. Materiels et methodes Un appareil photographique numerique et une adresse de messagerie unique et specifique etaient fournis a chaque etablissement selectionne afin de teletransmettre des photographies anonymisees. Les patients atteints de plaies chroniques posant un probleme de soins etaient inclus. Le nombre total d’avis de tele-expertise, le type de plaies chroniques, le nombre d’hospitalisations et de consultations directes, le nombre de trajets en ambulance evites ont ete colliges. Une evaluation du projet a ete effectuee apres un an. Resultats Sur 40 EHPAD invites, 22 s’etaient engages mais seuls les dix premiers a avoir repondu ont ete retenus pour realiser l’etude pilote de faisabilite. Un financement (CPAM Haute-Vienne avec « Prix sanitaire et social ») permettait l’achat des appareils photographiques. Depuis le 15 avril 2010, dix EHPAD ont teletransmis des photographies de 34 patients avec 26 plaies chroniques. Dix escarres, deux maux perforants plantaires et 14 ulceres de jambes ont ete tele-expertises. Neuf patients ont ete hospitalises pour diagnostic. Discussion et conclusion En deux ans, cette experience de tele-expertise a permis d’eviter 20 deplacements de patients dependants et l’orientation rapide de neuf patients vers le CHU en optimisant la prise en charge des patients porteurs de plaies chroniques en EHPAD.

Resume Introduction La maladie de Whipple (MW) est une maladie infectieuse rare responsable de signes cliniques variables tant dans leur localisation que dans leur severite. Elle peut mimer un rhumatisme inflammatoire chronique de type polyarthrite rhumatoide ou spondyloarthrite. L’instauration d’une biotherapie a la suite d’une erreur diagnostique dans ce contexte n’entraine pas toujours des complications immediates. Parfois, l’aggravation fait suite a une amelioration initiale encourageant la poursuite du traitement immunosuppresseur et conduisant par consequent a un retard diagnostique. Observations Nous rapportons deux nouvelles observations de MW diagnostiquees en raison de l’echec de traitement anti-TNFα mis en route pour rhumatisme inflammatoire actif. Dans la premiere observation, il s’agissait d’un homme de 53 ans traite par etanercept puis adalimumab pour une spondylarthropathie axiale et peripherique et dans la seconde d’un homme de 42 ans traite par adalimumab et etanercept pour une spondylarthropathie peripherique. Conclusion Une MW doit etre evoquee devant tout patient suspect de rhumatisme inflammatoire chronique non repondeur ou partiellement repondeur aux anti-TNFα et dont le syndrome inflammatoire biologique persiste sous traitement.

Objective: To identify nailfold videocapillaroscopic features and other clinical risk factors for new digital ulcers (DUs) during a 6-month period in patients with systemic sclerosis (SSc). Methods: In this multicenter, prospective, observational cohort study, the videoCAPillaroscopy (CAP) study, we evaluated 623 patients with SSc from 59 centers (14 countries). Patients were stratified into 2 groups: a DU history group and a no DU history group. At enrollment, patients underwent detailed nailfold videocapillaroscopic evaluation and assessment of demographic characteristics, DU status, and clinical and SSc characteristics. Risk factors for developing new DUs were assessed using univariable and multivariable logistic regression (MLR) analyses. Results: Of the 468 patients in the DU history group (mean ± SD age 54.0 ± 13.7 years), 79.5% were female, 59.8% had limited cutaneous SSc, and 22% developed a new DU during follow-up. The strongest risk factors for new DUs identified by MLR in the DU history group included the mean number of capillaries per millimeter in the middle finger of the dominant hand, the number of DUs (categorized as 0, 1, 2, or ≥3), and the presence of critical digital ischemia. The receiver operating characteristic (ROC) of the area under the curve (AUC) of the final MLR model was 0.738 (95% confidence interval [95% CI] 0.681–0.795). Internal validation through bootstrap generated a ROC AUC of 0.633 (95% CI 0.510–0.756). Conclusion: This international prospective study, which included detailed nailfold videocapillaroscopic evaluation and extensive clinical characterization of patients with SSc, identified the mean number of capillaries per millimeter in the middle finger of the dominant hand, the number of DUs at enrollment, and the presence of critical digital ischemia at enrollment as risk factors for the development of new DUs. © 2016, American College of Rheumatology

International audience; IntroductionIn chronic inflammatory rheumatic diseases (CIRDs), comorbidities such as cardiovascular disease and infections are sub-optimally managed. EULAR recently developed points to consider to collect and report comorbidities. The objective of this present study was to develop a pragmatic guide to collect, report and propose management recommendations for comorbidities, from a rheumatologist perspective.MethodsThe collection and reporting of comorbidities and risk factors was adapted from the EULAR points to consider. To develop management recommendations, the process comprised (1) systematic literature reviews by 3 fellows and (2) a 2-day consensus process involving 110 experts (rheumatologists and health professionals). Votes of agreement (Likert 1-5 where 5 indicates full agreement) were obtained.ResultsThe six selected comorbidities were ischemic cardiovascular diseases, malignancies, infections, diverticulitis, osteoporosis and depression. The literature review retrieved 97 articles or websites, mostly developed for the general population. The consensus process led to reporting presence of comorbidities, current treatment, risk factors (e.g. hypertension), screening (e.g. mammography) and prevention (e.g. vaccination). Management recommendations include physical examination (e.g. blood pressure or lymph node examination), prescribing screening procedures, and interpreting results to refer in a timely manner to appropriate other health professionals. Agreement was high (mean ± standard deviation, 4.37 ± 0.33).ConclusionsUsing an evidence-based approach followed by expert consensus, this initiative furthers the dissemination in France of the EULAR points to consider, and clearly defines what part of the management of comorbidities is potentially within the remit of rheumatologists. This initiative should facilitate systematic management of patients with CIRDs.

Summary Background Imatinib mesylate is a potent inhibitor of platelet-derived growth factor and transforming growth factor-β signalling pathways which may play a role in systemic sclerosis (SSc)-associated skin changes. Objectives We aimed primarily at assessing the efficacy of imatinib mesylate in scleroderma skin fibrosis. Methods We performed a phase II double-blinded trial on patients with scleroderma with either morphoea involving > 20% of body surface area or SSc with extensive skin involvement: modified Rodnan Skin Score (mRSS) ≥ 20/51. Each patient was randomized to receive either imatinib mesylate 400 mg or placebo daily for a total of 6 months, and then was followed up 6 months after therapy discontinuation. Skin fibrosis was assessed by mRSS and measurement of the dermal thickness using skin biopsies performed at inclusion and at 6 months of treatment. In addition, quality of life (Dermatology Life Quality Index and modified Health Assessment Questionnaire for Scleroderma) was recorded at each visit, and pulmonary function before and after intervention. Results Twenty-eight patients were included in the study with a mean age of 48·9 years (range 30–71): 25 had a diagnosis of a SSc and three of diffuse cutaneous scleroderma. Demographic data, frequency of organ involvement of SSc and mRSS were comparable between groups. At 6 months, the proportion of variation of mRSS from inclusion was not statistically significantly different between the two groups (median +0·10 in imatinib group vs. −0·16 in placebo group, P = 0·098). Similarly, changes in dermal thickness, quality of life and diffusion capacity for carbon monoxide were not significantly different between groups. Conclusions This study failed to demonstrate the efficacy of imatinib 400 mg daily to improve skin fibrosis of diffuse scleroderma after 6 months of treatment based on validated outcome measurements.

Resume Introduction Lorsqu’il est utilise dans la litterature medicale francaise pour qualifier un etat pathologique, l’adjectif « historique » s’applique le plus souvent a des tumeurs qui presentent par leur taille un aspect impressionnant. Nous avons du mal a comprendre, dans notre imaginaire et notre raison, comment un malade peut laisser evoluer une telle lesion. Nous avons cherche a definir ce concept. Patients et methodes Deux dermatologues ont effectue un tri des photographies de tumeurs cutanees malignes prises entre 1970 et 2008, pour selectionner celles correspondant a leur conception de la tumeur « historique », definie par une taille inhabituelle compte tenu du diagnostic anatomopathologique. Les donnees socioprofessionnelles, demographiques, anatomo-cliniques et psychologiques, les traitements et l’evolution ont ete colliges. Resultats Vingt-sept patients (11 hommes et 16 femmes), d’âge moyen 74 ans (34 a 99 ans), presentaient une tumeur « historique ». Douze vivaient en milieu rural ; cinq etaient cadres. La duree moyenne d’evolution de la tumeur etait de 4,5 ans (six a 420 mois). Les tumeurs etaient des carcinomes epidermoides ( n = 9) et des melanomes (sept). Leur taille moyenne etait de 13 cm (six a 30 cm). On notait chez 13 patients l’existence de troubles psychiatriques, l’appartenance a une secte ou une demence. Le traitement avait ete chimiotherapique, radiotherapique ou chirurgical. Dix-huit patients sont decedes, en moyenne 13 mois apres le diagnostic. Discussion La tumeur maligne « historique » (TMH) est une realite. Compte tenu des diagnostics, la taille correspond a une evolution tumorale lente et/ou une prise en charge tardive, expliquee surtout par des troubles psychiatriques. Les donnees socioprofessionnelles montrent que la tumeur « historique » est autant observee en zone rurale qu’urbaine.

Resume Introduction Le traitement de reference de la maladie de Paget extramammaire (MPEM) est la chirurgie mais celle-ci est parfois mutilante et incomplete, avec un taux eleve de recidive. La phototherapie dynamique topique (PDT) offre une alternative therapeutique avec des taux de remission complete interessants, sans limitation de dose ni sequelle fonctionnelle ou esthetique. L’objectif de notre etude etait d’evaluer l’efficacite et la tolerance de la PDT dans le traitement de la MPEM. Patients et methodes Il s’agit d’une serie monocentrique de cas consecutifs traites entre le 1er decembre 2005 et le 31 decembre 2010. Tous les patients ayant une MPEM confirmee histologiquement etaient inclus dans l’etude. Les patients etaient traites par un cycle de deux seances PDT espacees de trois semaines. Un deuxieme cycle etait realise a S6 si la reponse etait partielle. D’autres seances etaient programmees en cas de rechute. Le protocole de traitement consistait en l’application d’une creme a l’aminolevulinate de methyle (MAL) suivie trois heures plus tard d’une illumination de dix minutes en lumiere rouge (570–670 nm) avec une fluence de 37 J/cm2. Resultats Huit patients (sept femmes, un homme) d’âge moyen 69 ans ont ete inclus consecutivement et traites pour MPEM vulvaire (n = 6), perianale (n = 1) ou axillaire (n = 1). Apres deux cycles de deux seances, sept patients etaient en remission clinique complete a M3 et une en remission partielle. Cinq patients etaient toujours en remission a M6. Tous les patients ont rechute apres une moyenne de 8,6 mois (quatre a 14 mois). Le facteur limitant parait etre la douleur pendant l’illumination. Les patients etaient satisfaits, avec une disparition des symptomes et une amelioration notable de leur qualite de vie. Discussion Le taux de remission clinique complete a six mois, apres deux cycles de PDT, etait proche de celui de la chirurgie. Le taux de rechute reste eleve mais le traitement peut etre repete sans consequence esthetique ou fonctionnelle, a l’inverse de la chirurgie. La PDT permet d’obtenir pendant six mois une disparition des symptomes douloureux lies a la maladie et contribue ainsi a l’amelioration de la qualite de vie des patients.

Objective. We previously showed that DU significantly increased global and hand disability with a significant impact on activities of daily living (ADLs) and work disability. This study aims to evaluate the impact of digital ulcer (DU) healing on disability and daily activity limitations in SSc. Methods. From January 2008 and June 2009, we prospectively evaluated 189 SSc patients for DU history, disability, employment and occupational status during meetings of the French SSc Patient Association (n = 86, 45.5%) or during hospitalization (n = 103, 54.5%)1. Among the 60 patients with at least one active DU at baseline (M0), 40 patients were followed longitudinally over 6 (3) months. These patients were evaluated for DU history, global and hand disability, health-related quality of life (HRQoL), daily activity limitation and employment status. Results. The median (IQR) age was 57.5 (43.5-68) years and the median (IQR) disease duration was 8.3 (3-16.5) years. Twenty-two (55%) patients had diffuse SSc and 34 (85%) were females. At baseline, a mean of 2.9 (2.8) DU per patient was reported. Thirty-three (82.5%) patients had ischaemic DU, 7 (17.5%) patients had >1 DU associated with calcinosis and 13 (32.5%) patients had mechanical DU. Thirteen (32.5%) patients had >4 DU at baseline. Among the 40 patients, 16 (40%) patients showed complete ulcer healing. In these patients with DU, the presence of calcinosis was associated with a lower probability of healing (P = 0.03). Comparison between healed and no-healed DU patients showed an improvement of hand disability provided by an improvement of the Cochin Hand Function score (P = 0.05)) and a trend towards HAQ domain dressing and grooming (P = 0.06) between M0 and M6 (3) visit in healed patients but not in no-healed patients. Concerning HRQoL, there were no difference for Mental and Physical component Scores of SF-36 but significant improvement of Bodily Pain score (P = 0.04) and Physical Role score (P = 0.05) between M0 and M6 (3) visit in patients with healed DU. The absence of healing was associated with significantly decreased work productivity (P = 0.05), whereas the performance in ADL was not significantly decreased (P = 0.15). Patients who were on sick-leave and who received some help for household tasks at the time of active DU were more likely to heal. Conclusion. For the first time, we provide prospective data with evidence that DU healing is associated with an improvement in hand function. Sick leave was associated with better healing of DU

A rise in the incidence of bullous pemphigoid (BP) was documented recently in Europe, and the main risk factors for BP remain unknown. We conducted a multicenter case-control study to evaluate risk factors for BP. We identified 201 incident BP cases and 345 controls individually matched for age, gender, center, and place of residence (home, nursing home, or extended-care facility). We used univariate and multivariate logistic regression analyses to compare drugs used for over 3 months, comorbidities, and physical and cognitive impairments between cases and controls. Mean age of BP patients was 84.2 (±8.7) years. Factors independently associated with BP by multivariate analysis were major cognitive impairment (odds ratio (OR), 2.19; 95% confidence interval (95% CI), 1.24-3.87), bedridden condition (OR, 2.19; 95% CI, 1.23-3.89), Parkinson's disease (OR, 2.16; 95% CI, 1.09-4.27), unipolar or bipolar disorder (OR, 5.25; 95% CI, 1.21-22.86), and chronic use of spironolactone (OR, 2.30; 95% CI, 1.20-4.46) or phenothiazines with aliphatic side chains (OR, 3.70; 95% CI, 1.21-11.34). Chronic analgesic use was associated with a lower risk of BP (OR, 0.49; 95% CI, 0.30-0.81). Thus, risk factors for BP include neurological disorders, particularly dementia and Parkinson's disease, psychiatric disorders (unipolar and bipolar disorders), bedridden condition, and chronic use of several drugs.

Introduction Les lupus actifs et corticodependants malgre un traitement immunosuppresseur conventionnel, representent un defi therapeutique. Aujourd’hui, si seul le belimumab a ete autorise par une AMM, d’autres biomedicaments sont parfois utilises au lieu a titre derogatoire dans des lupus refractaires. Etant donne le manque de donnees prouvant l’efficacite de ces biomedicaments dans certaines situations rencontrees en clinique, nous avons decide d’etablir des recommandations d’experts pour l’utilisation de biomedicaments dans le lupus erythemateux systemique. Materiels et methodes Nous avons developpe ces recommandations par « consensus formalise d’experts ». Le but de cette methode est de formaliser le degre d’accord entre experts en identifiant, par une cotation iterative avec retour d’information, les points de convergence, de divergence ou d’indecision entre experts. Cette methode comporte plusieurs etapes chronologiques consecutives (phase de pilotage, de cotation, de lecture, de finalisation et diffusion) realisees par des groupes de travail independants. Au travers de 3 reseaux dedies aux maladies auto-immunes systemiques (FLEUR, IMIDIATE, FAI2R) et sous l’egide du CRI, nous avons recueilli les opinions de 59 experts francophones du lupus exercant en Algerie, Belgique, France, Italie, Maroc, Suisse et Tunisie. Les specialites medicales representees etaient la medecine interne (49 %), rhumatologie (34 %), nephrologie (7 %), dermatologie (5 %), pediatrie (3 %) et medecine vasculaire (2 %). Deux methodologistes et 3 groupes d’experts strictement independants ont contribues a ces recommandations : un groupe de pilotage (GP) (n = 9), un groupe de cotation (GC) (n = 28) et un groupe de lecture (GL) (n = 23). Les propositions de recommandations ont ete redigees par le GP et soumises au GC. Chaque membre du GC a indique son degre d’accord de 1 a 9 (1 : totalement inapproprie ; 9 : totalement approprie ; 5 : indecision) et ses commentaires pour chaque recommandation. Apres 2 tours de cotations, le GP a reformule une version initiale des recommandations qui a ete soumise au GL. La distribution des scores et les commentaires du GL ont permis au GP de rediger une version finale des recommandations ( Tableau 1 , Tableau 2 , Tableau 3 , Tableau 4 ). Resultats Dix-sept recommandations finales ont ete elaborees par le GP, en tenant compte de tous les scores et commentaires faits par chaque membre du GC, du GL et des 2 methodologistes de l’etude. Ces recommandations definissent notamment 4 domaines : – le type de patients necessitant un biomedicament ; – le type de biomedicament (belimumab, rituximab, etc.) et de co-traitement a utiliser en fonction de l’atteinte d’organe ; – l’information a donner aux patients ; – la facon d’evaluer l’efficacite du traitement. Un algorithme de choix de biomedicament a ete realise a partir des recommandations finales du domaine 2. Conclusion Dix-sept recommandations pour l’utilisation de biomedicaments dans le lupus erythemateux systemique ont ete formulees par un large panel d’experts pour donner des conseils aux cliniciens dans la pratique quotidienne. Ces recommandations seront regulierement mises a jour en fonction des resultats de nouveaux essais cliniques randomises et de l’experience clinique croissante.

Introduction Quelques etudes ouvertes suggerent une efficacite du rituximab, sur l’amelioration de la sclerose cutanee et des capacites pulmonaires dans le sclerodermie systemique [1] . Nous presentons trois patients ayant beneficie au cours de leur suivi, de cures de rituximab en raison de 1 g a j0 et j14 pour une sclerodermie systemique refractaire ou intolerante au traitement habituel. Observation Cas no 1. Il s’agissait d’une femme de 68 ans, aux antecedents de cancer du sein gauche traite par radio-chimiotherapie et hormonotherapie et d’un emphyseme centrolobulaire post-tabagique, pour qui un diagnostic de sclerodermie systemique diffuse de forme rapidement progressive a ete pose en 2014 avec anticorps anti-ARN polymerase III positif. Elle presentait une atteinte digestive (œsophagite et estomac en pasteque), cutanee et cardiaque (sclerose aortique). Apres 4 cures de cyclophosphamide (CYC) entre juin et septembre 2015, on notait une aggravation de la sclerose cutanee distale engendrant un handicap fonctionnel et une erythrodermie apres la derniere cure de CYC. Apres 3 cures de rituximab entre decembre 2015 et decembre 2017 avec une bonne tolerance clinique, on notait une amelioration du score de Rodnan modifie (passant de 42 a 31) et de certains parametres fonctionnels respiratoires (capacite vitale forcee [CVF] passant de 62 a 84 %, capacite de diffusion [DLCO] passant de 32 a 52 %). Cas no 2. Un homme de 51 ans presentait une sclerodermie systemique seronegative avec atteinte cutanee, digestive (gastrite, prolapsus rectal, diarrhees chroniques), pulmonaire (HTAP et syndrome interstitiel) et cardiaque (fibrose endomyocardique). Il avait beneficie de 10 cures de CYC entre 2012 et 2013, avec un relais par mycophenolate mofetil (MMF) entre 2013 et 2016 en entretien. Devant la majoration de l’atteinte cutanee, cardiaque et digestive, un traitement par rituximab est entrepris en association a des immunoglobulines intraveineuses. Apres 2 cures de rituximab entre juillet 2016 et aout 2017, on notait une amelioration du score de Rodnan modifie (passant de 30 a 8) et une stabilite des explorations fonctionnelles respiratoires. Cas no 3. Une femme de 64 ans, presentant une sclerodermie systemique seronegative diagnostiquee en 1996 avec atteinte digestive (gastroparesie et reflux gastro-œsophagien), cutanee, pulmonaire (syndrome interstitiel), recevait des cures de CYC entre 1998 et 2002 avec efficacite initiale sur l’atteinte pulmonaire, puis d’un traitement par MMF en 2013 sur apparition d’une fibrose endomyocardique. Devant une aggravation de l’atteinte interstitielle pulmonaire et de l’atteinte cutanee, le rituximab est introduit. Apres 4 cures de rituximab entre decembre 2013 et septembre 2017, on notait une amelioration du score de Rodnan modifie (passant de 31 a 13) et un ralentissement de l’atteinte pulmonaire. Conclusion Plusieurs etudes, dont l’etude cas-temoin EUSTAR en 2014 portant sur 63 patients sclerodermiques, montraient une tendance a l’amelioration de la sclerose cutanee evaluee par le score modifie de Rodnan et du syndrome restrictif par l’amelioration de la CVF, voire de la DLCO au cours de l’utilisation du rituximab dans les sclerodermies systemiques diffuses, associee a une bonne tolerance clinique et biologique [2] . Or, l’etude randomisee et controlee portant sur des formes precoces de sclerodermie systemique de Boonstra et al. ne retrouve pas d’efficacite significative sur le plan cutane et pulmonaire entre rituximab et placebo [3] . Chez nos patients, l’introduction de l’anticorps monoclonal anti-CD20 semble ameliorer la sclerose cutanee avec une tendance a la diminution du score de Rodnan modifie et d’une amelioration des capacites pulmonaires ou du moins de leurs stabilisations dans les deux derniers cas. Bien que l’amelioration du score de Rodnan dans ce contexte puisse etre expliquee par l’histoire naturelle de la forme systemique diffuse et par son evaluation operateur-dependant, le rituximab pourrait etre une alternative therapeutique interessante et bien toleree dans la sclerodermie systemique. D’autres etudes randomisees sont cependant necessaires pour evaluer son efficacite ainsi que la posologie optimale.

Intra-articular osteoid osteoma is rare and difficult to diagnose. We report a 38-year-old woman who presented with an inflammatory monoarticular arthritis in the right ankle in the context of a spondylarthropathy, otherwise well-controlled by leflunomide. This monoarthritis was resistant to all types of treatment. Investigations led to the diagnosis of an intra-articular osteoid osteoma. This original observation is the first description of an osteoid osteoma developing in a patient already known to have inflammatory rheumatism. All previous reported cases were monoarthritis consecutive to the osteoid osteoma itself and interpreted as incipient inflammatory rheumatism.

Introduction : Les patients traites par biotherapie doivent en connaitre les complications. L'acquisition de competences de securite est l'un des objectifs des programmes d'education therapeutique (ETP) dans les rhumatismes inflammatoires chroniques (RIC). Objectifs : Evaluation de l'impact du programme ETP RHIN-RIC sur les competences de securite et d'autosoins de patients sous biotherapie pour un RIC. Methodes : Appreciation par le questionnaire Biosecure des competences de 80 patients sous biotherapie intraveineuse pour un RIC en hopital de jour de Rhumatologie du Groupe Hospitalier de la region Mulhouse-Sud-Alsace. Resultats : Parmi les patients, 10 % jugeaient l'information qui leur avait ete prodiguee insuffisante. Le score moyen etait de 65,9/100 (ecart-Type 19,15). Il variait significativement avec le niveau d'education (p = 0,009). Le score moyen etait significativement plus eleve chez les patients dont le traitement avait ete introduit recemment (p = 0,043). Parmi les patients, 37,5 % avaient integre le programme d'ETP. Le score Biosecure moyen etait significativement plus eleve dans le groupe ETP (71,87/100 contre 62,32/100 ; p = 0,038) et ce egalement dans le sous-groupe de niveau d'education moindre. Il n'y avait pas de difference significative sur le controle de la pathologie ou la survenue d'infections. Conclusion : Les programmes d'ETP permettent une meilleure maitrise des competences de securite, et une meilleure implication du patient dans la gestion de sa pathologie et de son traitement.

Introduction La maladie de Still de l’adulte (MSA) se caracterise par la triade : fievre, arthralgies et eruption cutanee typiquement pseudo urticarienne, contemporaine des poussees febriles. D’autres types d’eruption ont ete rapportes et sont consideres comme un facteur de severite de la maladie. L’objectif de notre travail etait de preciser les caracteristiques et la valeur pronostique de ces eruptions cutanees non typiques (ECNT). Materiel et methodes Les criteres d’inclusion dans cette etude multicentrique retrospective entre 1997 et 2017 etaient les suivants : (1) âge > 18 ans ; (2) MSA repondant aux criteres diagnostiques de Yamaguchi ; (3) eruption cutanee vue par un dermatologue avec description semeiologique et/ou photographies ± biopsie ; (4) suivi minimum de 6 mois. Ont ete exclus, les patients dont la MSA avait debute avant l’âge de 15 ans et les patients sans argumentaire diagnostique detaille dans leur dossier medical. Les ECNT ont ete definies comme toute eruption ne correspondant pas a la description classique : exantheme maculopapuleux, fugace, siegeant principalement sur le tronc et les membres. A la date de point (9/06/2017), la survenue d’un cancer a ete investiguee. Resultats Au total, 58 patients ont ete inclus, 43 femmes et 15 hommes, dont 18 afro-caribeens, d’âge moyen 43 ± 16 ans avec une mediane de suivi de 4 ans (Q1–Q3 : 1–8 ans). 14 patients (24 %) ont developpe une complication viscerale grave (CVG) necessitant un transfert en reanimation (n = 10) ou un syndrome d’activation macrophagique (n = 4). Une ECNT a ete observee chez17/58 patients (29 %). Fixe dans tous les cas, l’ECNT correspondait dans 4 cas a l’exantheme papuleux pigmente decrit comme « atypique » dans la litterature, a une urticaire profuse et fixe (n = 2), a un purpura vasculaire (n = 1), a une erythrodermie oedemateuse (n = 1), a un exantheme maculopapuleux evocateur de toxidermie (n = 6), a un placard oedemateux de l’avant-bras mimant un syndrome de Sweet (n = 1), a un erytheme periorbitaire (n = 2). Une dermatose neutrophilique urticarienne etait observee dans 12/17 cas. La comparaison des caracteristiques cliniques et biologiques des patients avec et sans CVG trouvait les facteurs de risque suivants : une ECNT (p = 0,003-OR : 5,9), une hypoalbuminemie 1000 (p Discussion Notre etude illustre le spectre de l’expression dermatologique de la MSA en rapportant des manifestations inhabituelles qui semblent avoir une signification pronostique. Le sur risque de cancer a long terme decrit ponctuellement dans litterature n’est pas observe dans notre serie. Conclusion La presence d’une ECNT lors du diagnostic de MSA semble etre associee a une evolution aigue plus severe.

Resume Introduction Depuis l’avenement des dermocorticoides d’activite tres forte (DCF) dans le traitement de la pemphigoide bulleuse (PB) en 2002, peu d’etudes longitudinales se sont interessees au devenir des patients a long terme. Or, la morbimortalite associee a ce traitement n’est pas negligeable. Nous decrivons l’evolution de cette affection en Limousin, a partir de donnees retrospectives des PB diagnostiquees entre 2002 et 2004. Malades et methodes Il s’agissait d’une etude retrospective monocentrique du devenir des PB diagnostiquees au Centre Hospitalo-Universitaire (CHU) de Limoges sur une periode de trois ans. Les parametres etudies etaient l’âge, le sexe, les types de traitement recus et l’evolution a six et 12 mois de traitement. A chaque etape du suivi etaient evalues le nombre de deces, de perdus de vue et de rechutes, ainsi que le nombre de patients en remission complete et leur traitement. Resultats Cinquante-quatre nouveaux cas de PB (36 femmes et 18 hommes) ont ete pris en charge au CHU de Limoges entre 2002 et 2004. L’âge median etait de 84 ans. Quarante malades ont ete traites par DCF seuls et 14 autres ont necessite soit une corticotherapie orale (n = 6), soit un traitement « immunosuppresseur » (par methotrexate, dapsone, mycophenolate mofetil ou leflunomide) (n = 8). Seuls 26 malades etaient evaluables a six mois, les 28 autres etant perdus de vue (n = 8) ou decedes (n = 20). Cette mortalite etait essentiellement observee dans les trois mois suivant le diagnostic. Parmi les patients evaluables, 17 etaient en remission et neuf en rechute. A un an de suivi, 23 malades restaient evaluables, les autres etant perdus de vue (n = 2) ou decedes (n = 1). Le taux de survie a un an dans cette etude etait de 56 %. Parmi les 23 patients evaluables a un an, 13 etaient en remission complete dont deux sans traitement, trois sous DCF et huit sous immunosuppresseur. Seize malades avaient rechute au cours de leur suivi, dont la moitie a deux reprises. Conclusion Malgre l’utilisation des DCF en premiere intention, le pronostic de la PB reste severe, avec un risque majeur de rechute la premiere annee et une mortalite elevee dans les trois mois suivant le diagnostic.

Resume Introduction Les vascularites infectieuses representent une cause rare d’accident ischemique cerebral (AIC). Nous rapportons un cas de meningovascularite de Lyme compliquee de plusieurs AIC successifs. Observation Un homme de 64 ans, sans facteur de risque cardiovasculaire, developpa brutalement une hemiparesie droite avec une hypodensite thalamique gauche sur le scanner cerebral initial, evoquant en premier lieu un AIC, dont l’etiologie ne fut pas retrouvee. Devant l’aggravation clinique rapide du patient avec apparition d’une alteration des fonctions cognitives et d’un syndrome cerebelleux bilateral, une imagerie par resonance magnetique (IRM) cerebrale avec des sequences arterielles d’angio-IRM (ARM) fut realisee, montrant des infarctus multiples et des signes de vascularite cerebrale. L’etude du liquide cerebrospinal mit en evidence une meningite lymphocytaire et une serologie de Lyme positive avec un index de synthese intrathecale d’immunoglobulines specifiques dirigees contre Borrelia burgdorferi positif. Le traitement antibiotique permit une amelioration biologique totale et clinicoradiologique partielle. Conclusion La neuroborreliose de Lyme doit etre evoquee dans le diagnostic etiologique des AIC dans les zones de haute endemie, notamment lorsque le bilan de premiere intention reste negatif.

Calciphylaxis is a rare necrotizing calcifying arteriolopathy, with a poor prognosis, for which there is currently no effective treatment. One of the major challenges of the therapy is normalizing the calcium-phosphate balance. Therefore, cinacalcet, which inhibit the production of parathormone by negative feedback, was considered a treatment option to control the evolution of calciphylaxis in a dialysed patient suffering from cholangiocarcinoma.

Resume Introduction La chirurgie, la cryotherapie et les agents anticancereux locaux sont les traitements actuellement valides dans la maladie de Bowen (MB). Ils sont efficaces mais souvent responsables de cicatrices importantes. Les auteurs rapportent leur experience de la phototherapie dynamique topique (PDT) dans cette indication. Malades et methodes Il s’agissait d’une etude retrospective monocentrique concernant 22 patients, et 38 MB cutanees, traites par PDT entre 2001 et 2006. L’agent photosensibilisant utilise etait le methylester d’acide 5-aminolevulinique (Metvix ® ). Les criteres etudies etaient l’âge, le sexe, la topographie et la surface des lesions, le nombre de patients ayant une MB multiple et/ou des lesions de grande taille, la tolerance immediate du traitement, la reponse clinique et le resultat cosmetologique a trois, six et 12 mois. La probabilite d’absence de survenue d’une rechute entre zero et 60 mois a ete etablie par analyse de survie selon la methode actuarielle pour intervalles variables. Resultats Sept hommes et 15 femmes, mediane de l’âge : 76 ans, intervalle interquartile : [69–82], ayant 38 MB ont ete traites par PDT. Les lesions predominaient aux membres inferieurs ( n = 24) et au visage ( n = 10). Les autres lesions etaient situees sur le tronc ou dans le dos ( n = 4). La surface moyenne des lesions traitees etait de 8,62 cm 2 (1–96 cm 2 ). Une MB multiple, definie par l’existence de trois lesions ou plus, etait diagnostiquee chez huit patients. Une grande surface lesionnelle etait notee dans 11 cas. L’effet secondaire le plus frequent etait une sensation de brulure immediate dans sept cas. Une remission a trois mois etait observee dans 100 % des cas avec une cicatrisation cutanee sans atrophie, ni sequelle esthetique. A six mois, le taux de remission etait de 100 %, a 12 mois, de 95 % ( n egal a une rechute traitee par quatre seances supplementaires de PDT). A 24 mois le taux de remission etait de 85 % ( n egal a cinq rechutes au total). Discussion Cette etude ouverte retrospective confirme les bons resultats en termes d’efficacite et de resultat esthetique de la phototherapie dynamique dans le traitement de la MB. Elle permet de traiter plus facilement que la chirurgie des lesions de grande taille et/ou multiples. Elle semble mieux adaptee a une population âgee que les topiques anticancereux qui necessitent une observance prolongee et que la chirurgie ou la cryotherapie qui occasionne des plaies postoperatoires longues a cicatriser. Conclusion Le traitement photosensibilisant etant particulierement couteux, il nous semble justifie de reserver la phototherapie dynamique aux lesions de grande taille et/ou multifocales ainsi qu’aux lesions de membres inferieurs survenant sur un terrain d’insuffisance veineuse chronique.

Vertebral osteonecrosis classically presents with an intravertebral vacuum cleft phenomenon or a fluid-filled cleft on MR images. These clefts are usually found in older patients presenting with more severe fractures, more significant collapse and instability. Therefore, although considered for a long time as pathognomonic for vertebral osteonecrosis, vertebral clefts are now considered to represent fracture non-union. The double-line sign is classically described for osteonecrosis of long bones, but has been reported in one case of concurrent spinal cord and vertebral bone marrow radionecrosis. We present a case of a histologically confirmed multilevel vertebral osteonecrosis manifesting as a double-line sign in the absence of an associated vertebral collapse and unrelated to radiotherapy.

Resume L’osteonecrose vertebrale est classiquement visible sous la forme d’un vide intrasomatique sur les radiographies simples voire d’une fente a contenu liquidien sur les images par resonance magnetique. Ces dissections sont surtout frequentes chez les malades âges porteurs de fractures graves avec des tassements importants responsables d’instabilite. Longtemps consideree pathognomonique d’osteonecrose vertebrale, la dissection intravertebrale est maintenant interpretee comme un signe de pseudarthrose. Le signe de la double ligne, signe classique d’osteonecrose des os longs, a ete decrit dans un cas de necrose radio-induite concomittant de la moelle epiniere et du corps vertebral. Nous decrivons une observation d’osteonecrose vertebrale etagee se traduisant par un signe de la double ligne en l’absence de tassement vertebral et d’antecedent de radiotherapie.