Schnitzler syndrome is a rare acquired systemic disease with a chronic evolution and difficult treatment. We report a 50-year-old woman with Schnitzler syndrome for 10 years, with major impact on her quality of life and refractory to conventional therapies. The patient was started on anakinra, an IL-1 receptor antagonist, with a rapid and sustained remission of the syndrome manifestations.

Recruitment of patients in randomized controlled trials is a well-known problem. The recruitment of patients may be difficult and often takes more time than expected. Most trials adapt their recruitment target or extend the inclusion period. Two recent reports from the United Kingdom noted that only 56 resp. 69% of the trials achieved their original recruitment target (1).

Phosphaturic mesenchymal tumors are the main cause of tumor-induced osteomalacia, a distinctive paraneoplastic syndrome mediated by overproduction of fibroblast growth factor 23, that leads to renal phosphate wasting and hypophosphatemia. Diagnosis of this mesenchymal tumors is difficult and usually delayed for several years. We present the case of a 70-years-old-male with generalized bone pain, multiple pathological fractures and persistent hypophosphatemia, diagnosed with tumor-induced osteomalacia after 4 years of the onset of symptoms. The tumor was localized in the forefoot using Gallium 68-DOTANOC positron emission tomography-computed tomography and successfully surgically treated. This case report highlights the importance of recognizing these rare tumors, as early diagnosis can prevent long-term morbidity.

BackgroundGLORIA is an ongoing large pragmatic trial that examines harm, benefit and costs of low-dose glucocorticoids added to the standard treatment of RA patients of 65 years or older. The eligibility criteria are non-restrictive: RA, age ≥65 years, disease activity score (DAS28) of ≥2.6, and no current glucocorticoid treatment. Patients with comorbidity are expressly included, and the impact of trial procedures on normal care is minimal. Nevertheless, inclusion proves to be challenging. We have prospectively sampled all the reasons for ineligibility across a number of centres in different countries participating in the GLORIA trial.MethodsRheumatologists from 8 centres in Germany, Hungary, The Netherlands, Portugal and Romania screened the patient list of at least two full clinic days. For each patient, the eligibility and all possible reasons of exclusion were recorded.Abstract AB0364 – Table 1Percentage of patients ineligible for the GLORIA trial, per reason (patients can have more than one reason)ResultsIn total, 385 patients were screened. Of these patients, 15 (4%) were eligible to participate in the GLORIA trial. In Germany, Romania and Portugal (Lisbon) none of the screened patients proved eligible.The most common reasons for ineligibility were inactive disease and age (both 58%) (table 1). Current glucocorticoid use was reported in 28%, 5% had a temporary reason (i.e. recent switch of therapy or glucocorticoid use), and 51% had more than one reason for ineligibility. We found remarkable differences between the sites in the distribution of the main reasons for ineligibility (table 1).Of the eligible patients, 1 was already participating, 3 were included after this screening, and 2 were currently considering participation; 9 declined participation (most common reasons: fear of glucocorticoids, not interested to participate, preference for GC injections or declining additional therapy).ConclusionsIn this prospective study, we found remarkable differences between countries in reasons for non-participation in our ongoing GLORIA trial. The willingness of eligible patients to participate was low in this elderly population, despite the pragmatic design. Earlier studies also showed that it is challenging to include elderly patients in a clinical trial.1 2 Pre-screening of patients in potential sites can provide important information on the potential to recruit patients in a trial, but the actual willingness of patients to participate remains hard to predict.References[1] Calamia M, et al. I’d Do Anything for Research, But I Won’t Do That: Interest in Pharmacological Interventions in Older Adults Enrolled in a Longitudinal Aging Study. PLoS One2016;11:e0159664.[2] Denson AC, et al. Participation of the elderly population in clinical trials: barriers and solutions. Cancer Control2014;21:209–14.AcknowledgementsThis project has received funding from the European Union’s Horizon 2020 research and innovation programme under grant agreement No 6 34 886.Disclosure of InterestNone declared

BackgroundTreatment of Paget's Disease of Bone (PDB) has been revolutionized by the use of zolendronic acid (ZA). Patients usually have a dramatic response to treatment with normalization serum alkaline phosphataise (ALP) levels and a longer period of clinical remission, compared with other class agents. Data from long-term use are scarse.ObjectivesEvaluate the effectiveness and safety of ZA in PDB patients, as well as remission, re-treatment rates and side effects in our outpatient population since 2005.MethodsA retrospective study of PDB patients treated with 5 mg ZA intravenous infusion at our day-care center. Follow up time, demographic and clinical characteristics, previous therapeutic agents, rate of response, number and reasons of re-treatment(s) and rates of adverse events were collected. A descriptive statistic analysis was made.Results48 patients, 60% female, mean age of 75 years, with a median time since the diagnosis of 12.3 years. The disease was poliostotic in 73% of the patients and pelvis (65%), skull (29%) and spine (27%) were the most common pagetic localizations. Deafness was present in 12.5% and 65% had hip involvement. 44% patients had been treated with another biphosphonate agent previously. Response rates were 97.9% at 1 year, 87.2% after 2 years and 95.1% after 3 years. The mean ALP levels before ZA infusion was 290 UI/L and after 112 UI/L. Sixteen patients needed a re-treatment in the period of follow up, minimum of 1 year after the ZA infusion and maximum of 8 years after. 56.3% due to raised of ALP levels and 43.8% due pain/ hip involvement. Four patients needed a third infusion due to hip involvement, and 2 of them a forth infusion due to the same reason. All of the patients re-treated due to hip involvement had severe hip involvement at time of diagnosis. In our population, 2 patients achieved 10 years remission, 5 patients 9 years remission and 10 patients 8 years remission with a single ZA infusion. Recording adverse effects were: 14.6% Flu like symptoms (7 patients), 2% assintomatic hypocalcemia (1 patient) and no reports of osteonecrosis or fractures. All of these effects were reported after the first ZA infusion.ConclusionsIn our population, we find high long-term sustained remission rate. Only sixteen patients needed re-treatment. Patients maintained sustained remission up to 10 years of a single ZA infusion. Incidence of adverse events was similar to the reported in the literature.ReferencesReid IR, Miller P, Lyles K et al. Comparison of a Single Infusion of Zolendronic Acid with Risendronate for Paget's Disease. N Eng J Med. 2005 Set:353(9):898–908Reid IR, Brown JP, Levitt N et al. Re-treatment of relapse Paget's disease of bone with zolendronic acid: results from an open-label study. Natur BoneKEy Report 2. 2013 Nov: 442: 1–3Reid IR, Lyles K, Brown JP et al. A Single Infusion of Zolendronic Acid Produces Sustained Remissions in Paget Disease: Data from 6.5 years, JBMR. 2011 Sep 26 (9):2261–70Devogelaer JP, Geusen P, Daci E et al. Remission over 3 years in patients with Paget d...