AB1076 TREATMENT REVIEW OF ADULT-ONSET STILL’S DISEASE IN A TERTIARY HOSPITAL
Authors: Maria Dolores Arcila Duran, Clara Aguilera Cros, Lara Mendez, Isabel Madroñal García, Marina Gomez Vargas, Alberto Ruiz Román, Esteban Rubio Romero, Juan Povedano Gomez
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown aetiology, and approximately 60% or 70% of the patients can develop a chronic poliphasic form of the disease or a chronic polyarthritis. Due to the rarity of this disease, the treatment of AOSD is not based on a controlled study, but in the experience based on real cases. <h3>Objectives</h3> Describe the different treatments employed in a patient cohort diagnosed with adult-onset Still's disease (AOSD). <h3>Methods</h3> Descriptive, restrospective study of patients treated in our Hospital (2008-2018), diagnosed with AOSD according to the classification criteria of Yamaguchi. The data were achieved by the review of the clinical records. <h3>Results</h3> Twenty-four patients (15 women), average age of 41±13 years, were included. Two women, with presentation on the symptoms at 8 and 3 years old, first diagnosed with systemic juvenile idiopathic arthritis (S-JIA), and then with AOSD. The initial treatment was based in non-steroidal anti-inflammatory drugs (96%) and glucocorticoids (0,5-1 mg/kg/day) (96%) for symptom control, with the necessity to add oral or subcutaneous methotrexate at a dose of 15 mg per week in 13 patients (54%). Only two patients used acetyl salicylic acid as initial treatment, with no improvement. Five patients used also biological treatments with a standard doses, with the neccesity of various drugs to achieve their clinical remission. Nowadays, all patients have their clinical remission. Patient 1: Infliximab, rituximab, tocilizumab and baricitinib. Patient 2: Etanercept and rituximab. Patient 3: Etanercept, adalimumab and infliximab. Patient 4: Etanercept, infliximab and tofacitinib. Patient 5: Infliximab, tocilizumab, baricitinib and sarilumab (great responde to a anti-IL6, tocilizumab were removed because of a local reaction in the injection's spot, althought it had a good response too). Two patients with clinical remission with JAK-kinase inhibitors (baricitinib and tofacitinib, respectively), one patients with anti-TNF (infliximab) and another one with anti-CD20 (rituximab). <h3>Conclusion</h3> In general, our results match with what it is published in the literature. For the treatment of AOSD has been used high doses of ASA (4g/day) or NSAID. However, the required doses (with their respective adverse effects), its limited responses and the frequent relapses after its suppression make difficult to maintain it. Nowadays, the systemic glucocorticoids are our first choice (0,5 to 1 mg/kg/day). A high average of our patients have a positive response with it, but in a 54% of the cases were neccesary to add methotrexate or others DMARDs because of a partial response with steroids. In the physiopathology of the AOSD there is an increase of pro-inflammatory cytokines, as the tumor necrosis factor, IL-1 e IL-6. The use of therapies that inhibit these molecules (anti-TNF, anakinra or canakinumab as anti-IL1 or tocilizumab or sarilumab as anti IL-6) is being a progress. The inhibitors of IL-1 can be more efficient for systemic manifestations, while the inhibitors of IL-6 are for articular and systemic affectation. The TNF inhibitors should being used for the articular affectation only. In our patient cohort there is no patient with anti-IL1, a patient in clinical remission with anti-TNF and another one with anti-IL-6. Prospective studies with a higher number of patients is neccesary to define better the AOSD treatment. <h3>Disclosure of Interests</h3> None declared