AB1086 ADULT ONSET STILL’S DISEASE: EXPERIENCE OF THE RHEUMATOLOGY DEPARTMENT OF THE UNIVERSITY HOSPITAL OF CASABLANCA
Authors: Zakaria El Ouali, Saadia Janani, Kawtar Nassar, Ouafa Mkinsi
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Adult onset Still's disease (AOSD) is a rare condition characterized by four cardinal signs: fever, arthralgia/arthritis, rash, and neutrophilic leukocytosis. The absence of specific criteria is currently compensated by pathophysiological advances, leading to the introduction of a promising new therapeutic arsenal represented by biotherapies. <h3>Objectives</h3> The aim of this study was to compare the profiles of this disease in our latitudes with data from foreign literature; and to study the most recent data concerning the pathophysiology and treatment of this disease. <h3>Methods</h3> We conducted a retrospective study of hospital files from January 1993 to October 2018. The diagnosis is retained according to Yamaguchi criteria. Analysis was performed using IPSS. <h3>Results</h3> 17 patients were selected. The sex ratio was 0.54, and the average age was 31.5 years. At the time of diagnosis, fever was found in 94% of cases, arthralgia/arthritis in 100% of cases, and rash in 65% of cases. Concerning rheumatic features, the most frequently affected joints were: wrists (87%), knees (73%), shoulders (67%), proximal interphalangeal (62%), elbows and ankles (60%), hips (47%). The involvement of the hands is dominated by the proximal interphalangeal and metacarpophalangeal (45%). Distal interphalangeal involvement is observed in 23% of cases. Cervical spine involvement is noted in 50% of cases. Biologically, 88% of patients had neutrophilic leukocytosis. Ferritinemia was increased in 79% of patients. The glycosylated fraction of ferritin was measured at diagnosis in 47% of patients, of whom 63% had a fraction ≤ 20%. Elevated transaminases were found in 47% of cases. Therapeutically, 100% of patients received corticosteroids; 65% methotrexate; and 29% biotherapies (tocilizumab, anakinra, infliximab, adalimumab). The evolutionary profile was exploitable in 13 patients, of whom 10 were in complete remission. <h3>Conclusion</h3> Our data are consistent with those in the literature. Some peculiarities emerge concerning the articular features. From 3 series of literature and ours, totaling 135 cases, it appears that the most frequent locations are: wrist (82%), knees (70%), elbows (46%), ankles (45%), proximal interphalangeal (44%), shoulders (42%) and metacarpophalangeal (42%). The pathogenesis of AOSD seems to lie at the crossroads between auto-inflammatory diseases and lymphohistiocyte activation, resulting from a multifactorial predisposition. The central role of innate immunity, the clinical presentation, and the efficacy of blocking the IL-1 pathway comparable to that observed in "monogenic inflammasomopathies" justify the reclassification of AOSD among polygenic auto-inflammatory syndromes. <h3>References</h3> [1] Georgin-Lavialle S, et al. [Clinical overview of auto-inflammatory diseases]. Rev Med Interne. 2018;39(4):214-32. [2] Gerfaud-Valentin M, et al. Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients. Medicine (Baltimore). 2014;93(2):91-9. [3] Wouters JM, et al. Adult-onset Still's disease; clinical and laboratory features, treatment and progress of 45 cases. Q J Med. 1986;61(235):1055-65. [4] Pouchot J, et al. Adult Still's disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore). 1991;70(2):118-36. [5] Ben Taarit C, et al.[Adult Still's disease: study of a series of 11 cases]. J Mal Vasc. 2002;27(1):31-5. <h3>Disclosure of Interests</h3> None declared