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BackgroundIn Adult-onset Still’s disease (AOSD), cases refractory to typical DMARDs, Canakinumab (an anti-IL-1ß monoclonal antibody) has been reported to be effective in a limited number of refractory cases.1 ObjectivesThe aim of this retrospective study was to represent AOSD patients treated with Canakinumab in 3 centres.MethodsThe follow up data of 10 AOSD patients (8 female, 2 male), who were followed up in outpatient clinics of 3 tertiary centres were reviewed retrospectively. The initial characteristics and follow up findings were reported.ResultsThe mean timespan between the initial diagnosis and Canakinumab treatment 45.2±29 months (mean ±SD). Before the onset of Canakinumab therapy, all patients were exposed to methotrexate, 1 to leflunomide, 8 to Tocilizumab and 8 to Anakinra. As for the biologic agents, 3 patients were also treated beforehand with Infliximab, 2 with Adalimumab, 2 with Etanercept and 2 with Rituximab. Canakinumab therapy was initiated in all patients with the indication of refractory disease under other medications, except for the one in whom neutropenia became evident under anakinra. The mean number of Canakinumab injections was 9.3±8. The mean follow-up period of patients treated with Canakinumab was 43.1±33 months. Seven out of 10 patients are still being treated with Canakinumab of 150 mg/month and one of 150 mg/every 2 months. One patient had a single injection and was fully controlled. The mean ferritin measure of 9 patients was reduced from 1292.3±1530 ng/ml to 354±530.2 ng/ml following the Canakinumab therapy (p=0.035). The mean of patient-reported global visual analogue scale (PG-VAS) scores was reduced from 7.4±2.4 to 2.3±2.2 with Canakinumab (p<0.001). Mean Erythrocyte sedimentation rate (ESR) was reduced from 44.2±35.1 to 22.7±26.5 with the help of Canakinumab therapy (p=0.005). Six patients are still on prednisolone at a maximum dose of 10 mg/day. The indication of therapy termination in the remaining 1 patient was the diagnosis of tuberculosis at 9th month of the treatment despite isoniazid prophylaxis. The patient was also treated with multiple biological agents beforehand, therefore it is not easy to conclude that treatment with Canakinumab induces tuberculosis flares.ConclusionsCanakinumab treatment seems to be effective in refractory AOSD patients who were previously treated with various agents. We state that an IL-1 blocking agent, Canakinumab is a relatively safe and effective alternative in managing refractory AOSD cases. On the other hand, randomised controlled trials are needed to further investigate the role of Canakinumab in these cases as well as its use as the first choice of biologic agents.Reference[1] Kontzias A, Efthimiou P. The use of Canakinumab, a novel IL-1β long-acting inhibitor, in refractory adult-onset Still’s disease. Semin Arthritis Rheum2012;42(2):201–5.Disclosure of InterestNone declared

BackgroundInterstitial lung disease (ILD) is a severe complication of systemic sclerosis (SSc). Immunosuppressives such as cyclophosphamide (CYC) and mycophenolate mophetil (MMF) are used in its treatment with no proven efficacy (1). Rituximab (RTX) appears to be an emerging agent according to case series.ObjectivesThis retrospective study aims to evaluate the efficacy of RTX on SSc-ILD in a group of patients followed in our center.MethodsA chart review revealed 18 patients (16 women, 2 men; mean age 50.3±12.1 SD years (range 30–72), mean disease duration 8.3±9.3 SD years) with SSc who have been diagnosed as having ILD (confirmed by high-resolution thorax computed tomography and pulmonary function tests) and have been treated with one or more cycles of RTX. Efficacy was evaluated according to the criteria of the American Thoracic Society: improvement= an increase in FVC≥10% or DLCO≥15%; worsening= a decrease in FVC≥10% or DLCO≥15%; stabilization= changes in FVC less than 10% or DLCO less then 15% (2).ResultsTable 1Demographic findings of the patients and their response to RTX treatmentGroup 1Group 2All patients (ILD with short duration and naive to treatment)(ILD with long duration and previous IS therapy) Number of patients4 (22.2%)14 (77.7%)18Sex (F/M)3/113/116/2Mean disease duration2±0.8 SD years10.2±9.8 SD years8.3±9.3 SD yearsFollow-up time after initiation of RTX12.2±6.8 SD months21±12.4 SD months19±11.8 SD monthsBaseline FVC%69.2±20.965.1±14Last FVC%66.7±1361.6±19.6Baseline DLCO%57.7±24.143.5±12.2Last DLCO%52±1841.2±21.8Outcome: Stable/Improvement (n)279Worsening (n)257Death011Unable to do PFT (n)011Four patients were treatment naive for ILD when they received RTX (Group 1). The mean duration between the diagnosis of ILD and RTX treatment in Group 1 was 3.5 months (range 0–14 months). The average RTX cycle in this group was 2 with 1 patient also receiving mycophenolate mophetil in combination with RTX. The mean follow-up time after the initiation of RTX in this group was 12.2±6.8 SD months (range 7–22 months). FVC/DLCO was stable or improved in 2/4 compared to baseline and worsened in 2/4 at the end of follow-up at group 1.Fourteen patients had a 10.2 years-history of SSc and have been treated with immunosuppressives (cyclophosphamide, azathioprine, methotrexate, MMF) for ILD before RTX (Group 2). The mean duration between the diagnosis of ILD and RTX treatment in Group 2 was 71.2 months (range 5–246 months). These patients received a mean of 3 cycles of RTX with 5 receiving MMF (n=3) or AZA (n=2) in addition to RTX. One patient died after 3 months following the first RTX cycle (unknown reason) and 1 was unsuitable for spirometry because of microstomia. Of the remaining 12 patients in Group 2, improvement or stabilisation was seen in 7 and worsening was seen in the remaining 5 patients.ConclusionsRTX appears to be modestly effective for ILD of SSc. The duration of ILD as well as the presence or absence of previous immunosuppressive therapy do not appear as playing a role in response.References Avouac J et...