AB0971 Cervical Spine Involvement as a Presenting Manifestation in Juvenile Idiopathic Arthritis

BackgroundCervical spine involvement as the first manifestation of Juvenile Idiopathic Arthritis (JIA) is uncommon. Only 7 cases of JIA with this feature were found in a systematic review of the literature between 1956-2014.ObjectivesTo describe a multicenter cohort of patients with JIA and cervical arthritis, confirmed by image, as the first manifestation of the disease.MethodsA multicenter, retrospective and descriptive study was conducted. Patients who presented cervical spine involvement as sole initial manifestation of JIA were included. Data regarding demographic, disease characteristics, image study performed, treatment received and outcome were recorded.ResultsSeven patients were included (5 girls, 2 boys). All of them presented cervical spine involvement as the first manifestation of JIA. The mean age at disease onset was 4.6 years ±2.3. The mean time of delay in diagnosis of JIA was 7.3 months (range 1 to 30). Patients were classified according to the International League of Associations for Rheumatology (ILAR) criteria: Oligoarticular-persistent (4/7), Poliarticular Rheumatoid Factor (RF) negative (2/7) and Undifferentiated (1/7). The mean duration of JIA was 3.6 years (range 0.5-7). Antinuclear antibodies were positive in two of the patients. None of them were positive for antiCCP antibodies, RF or HLA B27. The most prevalent symptoms were torticollis (100%), range of motion limitation (71%) and pain (57%). After the diagnosis 3 patients had chronic anterior uveitis (1/3 unilateral and 2/3 bilateral). Imaging evaluation of the cervical spine was conducted by means of conventional radiography in 6 patients, Magnetic Resonance Imaging (MRI) in all of them, 1 bone scintigraphy and 1 computed tomography scan. The most common findings on MRI were synovitis C1-C2 (100%) and subluxation (43%). One of the patients presented bone erosions at the time of diagnosis. As first-line pharmacological treatment NSAIDS, oral glucocorticosteroids and Methotrexate (6/7 patients) were prescribed.In these 6 patients an anti-TNF agent (5/6) or Leflunomide (1/6) was added to therapy. In all cases Etanercept was the first choice between the biological treatments. Two patients were switched to Adalimumab because of uveitis. Six children followed a rehabilitation program and/or used a cervical collar. Surgical arthrodesis was required in one patient with C1-C2 unstable subluxation. At last follow-up clinical disease activity was inactive in 5/7 patients. One child had di