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International audience; ObjectivesLupus nephritis (LN) is a frequent complication of systemic lupus erythematosus (SLE). Severe (proliferative) forms of LN are treated with induction immunosuppressive therapy (IST), followed by maintenance IST, to target remission and avoid relapses. The optimal duration of maintenance IST is unknown. The WIN-Lupus trial tested whether IST discontinuation after 2‒3 years was non-inferior to IST continuation for two more years in proliferative LN.MethodsWIN-Lupus was an investigator-initiated multicentre randomised controlled trial. Patients receiving maintenance IST with azathioprine or mycophenolate mofetil for 2–3 years, and hydroxychloroquine, were randomised (1:1) into two groups: (1) IST continuation and (2) IST discontinuation. The primary endpoint was the relapse rate of proliferative LN at 24 months. Main secondary endpoints were the rate of severe SLE flares, survival without renal relapse or severe flare, adverse events.ResultsBetween 2011 and 2016, 96 patients (out of 200 planned) were randomised in WIN-Lupus: IST continuation group (n=48), IST discontinuation group (n=48). Relapse of proliferative LN occurred in 5/40 (12.5%) patients with IST continuation and in 12/44 (27.3%) patients with IST discontinuation (difference 14.8% (95% CI −1.9 to 31.5)). Non-inferiority was not demonstrated for relapse rate; time to relapse did not differ between the groups. Severe SLE flares (renal or extrarenal) were less frequent in patients with IST continuation (5/40 vs 14/44 patients; p=0.035). Adverse events did not differ between the groups.ConclusionsNon-inferiority of maintenance IST discontinuation after 2‒3 years was not demonstrated for renal relapse. IST discontinuation was associated with a higher risk of severe SLE flares.Trial registration numberNCT01284725.

Background Occurrence of pulmonary arterial hypertension (PAH) in idiopathic inflammatory myopathies (IIM) without extensive interstitial lung disease (ILD) has been rarely described. Objectives This study aimed to report all cases with association of PAH and IIM in the French Pulmonary Hypertension (PH) Registry, to identify IIM features associated with PAH, and to describe treatment modalities of these patients. Methods All cases of IIM-PAH were retrieved from the French PH Registry, which gathers PAH patients prospectively enrolled by 21 referral centers across France. Patients were excluded if they had an extensive ILD or overlap syndrome. Characteristics of IIM-PAH patients were compared to a control group of IIM patients without PH. Results Among the 5223 PAH patients in the Registry, 34 had a diagnosis of IIM. Among them, 3 IIM-PAH patients had no sign of extensive ILD or overlap syndrome, and were included in this study. In these patients, dermatomyositis (DM) was the only identified IIM. Two of them had a myopathic DM, confirmed by electromyography and biopsy. The last patient had an amyopathic DM, associated with anti-Ku antibodies. Pre-capillary PH was hemodynamically proven in all patients. PAH always developed after IIM onset, was severe in all cases, and led to a marked functional impairment. By pooling our cases with 6 previously reported patients, and comparing them to a control cohort of 35 IIM patients without PH, we identify several IIM characteristics possibly associated with PAH occurrence, including DM subtype (78% vs. 46%, p =0.02), skin involvement ( p =0.04), anti-SSA antibodies ( p =0.05) and peripheral microangiopathy ( p =0.06). Overall, IIM-PAH patients were managed by corticosteroids and/or immunosuppressants, either alone or combined with PAH therapy. Patients that did not receive PAH therapy did not seem to respond to IIM treatment alone. Conclusions Our study reports for the first time the rare but possible association of PAH and IIM in a large prospective PH Registry. In that setting, PAH seems associated with DM, skin involvement, peripheral microangiopathy and anti-SSA positivity. The best therapeutic strategy for IIM-PAH remains to be defined. Disclosure of Interest None declared

Background Rheumatoid arthritis (RA) is a disabling chronic disease, regarded as the most frequent inflammatory rheumatism in adults, with a prevalence estimated between 0.3 and 1%, and a feminine ascendancy. In metropolitan France this prevalence is estimated from 0.3% to 0.5% of the general population. No precise data is available for French West Indies, and the prevalence of RA in this population of African ancestry is poorly evaluated. Objectives The objective of the study is to estimate RA prevalence in the FWI by a census forward-looking epidemiological survey in the hospital and liberal sectors for one year duration. Methods It is a unique tour with clinical examination, self-administered questionnaires and declaration. Secondary objectives are description of clinical and socio economical aspects of RA and cardiovascular comorbidities. We present the results for Martinique. Our survey was widely distributed (radio, press, patients9 associations…) to ensure a good completeness. Data were analysed using SAS 9.3 software (SAS Institute Inc., Cary, NC, USA). Throrough descriptive analysis of collected variables was conducted. Crude prevalence rates were adjusted to a standard population of Martinique (nationwide census in 2010). The 95% CIs were calculated using the Poisson distribution. Results Our completeness is good. 538 RA were collected, giving a prevalence in Martinique of 0.184% of the adult population (290 000), respectively 0.049 for men and 0.292 for women. 44% of these patients are treated in private practice and 56% in hospital. This cohort is composed of 88% women and 12% men; 92% were born in Caribbean and 7.7% elsewhere. Patients self declared of Afro Caribbean origin in 92.7%, Caribbean White in 1.7%, caucasian in 3% and other in 2.1%. Their mean disease duration was 9.7±10 years. RF and ACPA were positive in 82.2% (FR+ 392, ACPA+349, 35% low level, 65% high level): CCP+FR+ in 306, FR+CCP- in 77, FR-CCP+ in 41, FR-CCP- in 96. 17% had extra articular manifestations. ACR1987 and ACR2010 criteria were pos in 94.4% and 78%. HAQ was 1and 2 in 3%. DAS28 were respectively 5.1 in 3%. Less than 5% are ever smoker. Parodontopathy is infrequent. Cardiovascular risk factors were noticed in 89,4% with a mean of 2 CVRF beside RA. Conclusions This work clarifies the low prevalence of RA in this population of African origin. Some characteristics as: reduced prevalence, strong female representation, strong seropositivity, high levels of anti-CCP, no tobacco, differentiate our patients from other populations and evoke another etiology than tobacco. Disclosure of Interest None declared

Background Neonatal lupus syndrome (NLS) includes congenital heart block (CHB) and cardiomyopathies. Its optimal management is debated. Objectives We analyzed the mortality and morbidity of CHB, with special focus on risk factors. Methods This was a retrospective study of the French national registry of NLS. Inclusion criteria were high-degree CHB associated with maternal anti-SSA/SSB antibodies. Results 214 CHB were included (202 in utero cases and 12 neonatal cases). These 214 fetuses or children were born to 195 mothers anti-SSA (99.5%) and/or anti-SSB antibodies (60%) positive. 51 mothers (26.2%) fulfilled the classification criteria for an autoimmune disease: systemic lupus erythematosus (n=23), Sjogren syndrome (n=14), undifferentiated connective tissue disease (n=7), or other autoimmune disease (n=7). The factors associated with feto-neonatal deaths (15.7%) were hydrops (p During a median follow-up of 7 years [birth to 36.1 years], 148 of 187 surviving children (79.1%) had a pacemaker, 35 (18.8%, one missing data) had dilated cardiomyopathy (DCM), and 22 (11.8%) died. DCM was neonatal (n=13) or late-onset (n=22, diagnosed at a median age of 15.2 months [3.6 months-22.8 years]). Factors associated with neonatal DCM were maternal treatment with hydroxychloroquine, in utero cardiomegaly, in utero DCM, and hydrops. By contrast, only non-white race origin and significant in utero valvulopathy were associated with late-onset DCM. On multivariate analysis, only in utero DCM was associated with neonatal DCM (p=0.0001; HR 15.99 [95%CI: 3.93-65.01]), whereas non-white race origin was associated with late-onset DCM (p=0.0147; HR 3.65 [95%CI: 1.28-10.0]). For children who survived the neonatal period (n=179), the risk of death during follow-up was 7.8%. On multivariate analysis, the only factor associated with late mortality was postnatal DCM (neonatal and late-onset DCM) (p Fluorinated steroid in utero treatment was not associated with CHB regression, survival or absence of late-onset DCM. Conclusions The factors associated with late-onset DCM differ completely from those associated with neonatal DCM. Our findings do not support the use of fluorinated steroids for CHB. Disclosure of Interest None declared

Background Lumbar canal stenosis (LCS) is a clinicopathological and radiological disease first described in 1949, causing a neurovascular compression leading to back pain, radicular, sensory or motor deficit which treatment is difficult. Objectives The objectives of our study were appraisal of a protocolized treatment for 3 months and identification of predictive factors for failure of medical treatment. This is a prospective nonrandomized study with no control group. All patients satisfying inclusion criteria were included after clinical and radiological evaluation. Methods Clinical inclusion criteria were neurogenic claudication, radiculopathy at rest with or without back pain, mono or multirooted sensory, motor or reflex abnormalities of the lower limbs. Radiological inclusion criteria were: anteroposterior dural sac diameter 2 , reduction in the anteroposterior diameter of the spinal canal >25% on myelography in supine extended position. Results Included patients had at least one clinical and one radiological criteria. Treatment was 2 epidural injections of 125 mg hydrocortancyl 15 days apart, combined with NSAIDs or analgesic, associated with physiotherapy sessions over 2 months. 147 patients were included. Mean age was 62±13 years. F/M sex ratio is 1.3. Mean BMI was 27.5±4.3, overweight was present in 72.1%. At baseline, mean duration for back pain and radicular pain were 9.3±10.5 and 4.4±5.7years. Primary objective was evolution of the Oswestry [1] score (a low back pain disability questionnaire) at 3 months as compared to baseline. Secondary objective was the evolution of pain. At the end of treatment, the mean value for the Oswestry score decreased from 41.2 to 39.7 (p=0.17). Mean VAS for lumbar pain decreased from 53.6 to 42.1 (p BMI, duration of back and radicular pain, mean lumbar radicular VAS and mean Oswestry score at inclusion were not significantly different in improved and non improved patients. Predictors of good response to treatment were the initial presence of impulsivity cough and nocturnal pain. Conclusions In conclusion with this approach there was no significant decrease in Oswestry score, however there was a significant decrease in mean VAS lumbar and radicular pain. No predictor of poor response has been highlighted. Predictors of good response were the initial presence of impulsivity at cough and nocturnal pain. These results show the limits of medical treatment in the management of SCL. References Niskanen RO, The Oswestry Low Back Pain Disability Questionnaire. a two-year follow-up of spine surgery patients. Scand J Surg, 2002 91 (2):. P. 208-11. Disclosure of Interest None declared

Background No study describes the frequency of Behcet’s disease (BD) in the populations of African origin and its characteristics. Objectives To retrospectively assess by retrieval of multiple sources, the epidemiology and characteristics of BD in the mainly African descent population of Martinique, a French Island in the Caribbean. Methods In 2011 we consulted 5 different sources. Patients were included if they lived in Martinique, were classified as certain if they fulfilled the international criteria for BD. The diagnosis was considered only as probable if they had recurrent oral ulcers with at least one other international criteria but no other possible diagnosis. The incidence during the 1997-2011 interval and prevalence at December 31, 2010 were calculated. Basic and BD-related characteristics were recorded. Results 48 patients were indicated as BD. Only 36 (female, n=22; male, n=14) fulfilled the criteria. Mean age at diagnosis was 38.5 years old (range: 8-64), 36.6 for women and 41.6 for men. Disease onset was over 50 years old for 7 patients. Mean time between first signs and diagnosis was 4.2 years (range: 0-22). Twenty eight began their disease between 1997 and 2011 allowing a total crude mean annual incidence estimation of 0.72 new cases/10 5 inhabitants (0.79/10 5 for female and 0.59/10 5 for males, 0.37/10 5 for certain cases and 0.35/10 5 for probable). In December 2010, 28 patients were alive, 5 lost to follow up and 3 deceased: the crude prevalence was estimated as 7.04/10 5 inhabitants (Female: 7.49; male: 6.51). Genital ulcers were found in 75.6%. Dermatological manifestations were: pseudofolliculitis (52%), erythema nodosum (13.5%). Pathergy test was done for 6 patients and positive for 4. Nine patients had ophthalmologic involvement: anterior uveitis (55.5%, n=5), panuveitis and posterior uveitis (11.1%, n=1 each), conjunctival ulceration (22.2%, n=2). Parenchymal brain involvement was more frequent than non-parenchymal (7 versus 3 patients) without any cerebral venous thrombosis. Rheumatic manifestations were occurred in 20 patients (55.6%) mainly females (60%): polyarthritis (38.9%), monoarthritis and spondylarthropathy (11.1% each). Deep vein thromboses were seen in 2 cases and superficial in 1, central retinal artery occlusion in 1 patient. The colon was involved in 6 cases and the ileo-cecal region in one. Conclusions In Martinique, BD seems to be a rare disease compared to Mediterranean and most Asian countries. The incidence is comparable to Spain and Switzerland. The prevalence is higher than in most European countries and USA. Black population seems to have particular manifestations of BD: older age at onset and rare uveitis. Disclosure of Interest None Declared