AB0621 Epidemiology, clinical characteristics and therapy apporaches of a retrospective cohort of paediatric systemic lupus erythematosus in a tertiary centre

Background Systemic lupus erythematosus (SLE) is a chronic autoimmune disease, potentially severe, with broad clinical spectrum, which can affect multiple organs and systems. Objectives To analyse the initial manifestations, laboratory examinations and therapeutic approaches of patients diagnosed with childhood onset systemic lupus erythematosus (c-SLE) followed in a tertiary hospital in the last 14 years. Methods Retrospective chart review. Inclusion criteria were: children under 18 years diagnosed with c-SLE between January 2003 and January 2017. Results During the study period, 38 patients were identified (ratio female/male: 3/1). The mean age at the onset of disease was 11.5 years (range 6–17). All had caucasic origin, except 5 coming from South America, 2 from India and 1 from Africa. Onset of the disease took place in spring in 22 (58%) patients, while in 10 (26%) patients onset was in summer, 5 (13%) in autumn and only one (3%) in winter season. The most frequently clinical manifestations found at the debut were cutaneous involvement (66%, predominantly in the form of a malar rash), renal (65%) and joint (60%, 48% arthralgia and 52% polyarthritis). Other manifestations were fever (50%), cytopenias (39%), asthenia (36%), serositis and neurological clinic (both 26%) and oral aphthosis (23%). Among the neurological manifestations, 4 patients showed bradypsychia, 3 headache, 2 seizures and 1 manifested a paralysis of the sixth cranial nerve. Only 1 patient presented macrophage activation syndrome after primoinfection by Ebstein Barr Virus. During the study period, no deaths ocurred. Ten patients also had some other associated autoimmune disease: 6 hypothyroidisms, 2 IgA deficiency, 1 vitiligo and 1 celiac disease. No case of diabetes mellitus was identified. Regarding immunology, in all cases, positive antinuclear antibodies (ANA) were detected, although with variable titers (1/80 – 1/5120). Of the 25 patients who presented renal disease at the onset, 19 associated Anti-dsDNA antibodies positive at the initial time of the determination. Although antibodies related to antiphospholipid syndrome (APS) (anticardiolipin, anti-2glycoprotein and lupus anticoagulant) were detected in 12 patients (32%), only two developed associated clinical manifestations (both deep vein thrombosis in the lower limbs). Regarding treatment, all patients required corticosteroids. Therapy with acetylsalicylic acid was indicated in all patients with APS-associated immunology. Only 6 patients received treatment with corticosteroids and hydroxychloroquine exclusively. Nineteen (50%) patients initially received azathioprine therapy, being necessary to switch to mycophenolate mofetil for lack of response in eleven, receiving the last treatment 20 patients finally (52%). Eighteen patients (47%) received cyclophosphamide therapy, 16 of them as a consequence of their renal involvement. In addition, biologic therapy (rituximab and belimumab, respectively) was used in two multirefractory patients. Conclusions As widely already reported, SLE is a disease that affects predominantly women. Moreover, as it has been previously described in the literature the most frequently initial manifestations found in c-SLE are cutaneous, renal and articular. However, a large variability of onset symptoms exists, thus c-SLE should be ruled out in patients with multisystemic involvement Disclosure of Interest None declared