AB0977 DISEASE COURSE AND TREATMENT RESPONSES IN JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS; A SINGLE CENTER EXPERIENCE
Authors: B. Sözeri, F. Demir, D. Kilit, C. Pehlivanoğlu
Journal: Annals of the Rheumatic Diseases (2020)

Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease that may cause morbidity and mortality by affecting multiple systems. The 10-20% of patients have juvenile onset and this cluster have may more severe kidney, neuropsychiatric or hematological involvement. Objectives: The aim of this study was to assess the clinical and laboratory characteristics, disease activity, and treatment response of patients with juvenile SLE (jSLE). Methods: This is a retrospective study involving patients between 1 July 2016 and 1 January 2020. The data of patients diagnosed with jSLE and followed up for a minimum of 6 months, were collected. The SLEDAI-2K scores at initiation and at the follow-up (1st, 3rd, 6th, and 12th months of treatment) were examined. The SLEDAI-2K score was considered to be ≤4, for disease remission status. Results: A total of 49 children were included in to the study. The female/male ratio was 4.4/1 and the median age of the patients at the diagnosis was 13 (IQR: 11.1–15.2) years. The median follow-up of patients was 19 (IQR: 12–25) month. Four of the patients were diagnosed with monogenic SLE. Two siblings were diagnosed with c3 deficiency and two were diagnosed with familial chilblain lupus. The most common clinical findings were found musculoskeletal complaints (69.4%), malar rash (51%), oral ulcers (38.8%), and fever (30.6%), respectively in over all the group. The frequency of involvement of the system and organs was as follows; mucocutaneous 77.6%, musculoskeletal 69.4%, renal 44.9%, hematological 34.7%, serous membranes 16.3%, neuropsychiatric 12.2%, respectively. All patients had anti-nuclear antibody positivity, while 46.9% had anti-ds DNA, 14.3% had anti-Sm and 8.2% had antiphospholipid antibody positivity. While all patients received hydroxychloroquine treatment, 22.4% of the patients were received were mycophenolate mofetil, 22.4% were azathioprine, 14.3% cyclophosphamide, 12.2% methotrexate and 10.2% were rituximab. The median SLEDAI-2K score was 14 (IQR: 10–18.5) at admission, besides it was found to 6 (IQR: 4–12), 4 (IQR: 2–6), 2 (IQR: 0–6) in the 1st, 6th and 12th months of treatment, respectively. While 98% of the patients had active disease at admission, 67.3% at 1 months, 32.7% at 6 months and 22.4% at 12 months still had active disease (SLEDAI-2K &gt;4). Patients with initially high SLEDAI-2K scores had significantly lower remission rates in the first month (p=0.003). It was observed that patients with high SLEDAI-2K scores in admission were more resistant to conventional immunosuppressive treatments and the use of rituximab was more frequent in these patients. At least one major organ (renal, hematological, neurological) were affected in 57% of patients. The remission rate of these patients at 6 months was found significantly decreased compared to the others (p &lt;0.005). Renal biopsy was performed in 21 patients (42.9%). 12 of them had type 4 lupus nephritis (LN), 5 had type 2, 2 had type 3, and 1 had type 5. It was observed that patients with renal involvement were the group that reached remission latest. Conclusion: The presence of high initial SLEDAI-2K scores and the major organ involvement have poor predictive value to achieve inactive disease. References: [1]Yee CS, Farewell VT, Isenberg DA, Griffiths B, Teh LS, Bruce IN, et al. The use of Systemic Lupus Erythematosus Disease Activity Index-2000 to define active disease and minimal clinically meaningful change based on data from a large cohort of systemic lupus erythematosus patients. Rheumatology (Oxford) 2011;50:982-8. [2]Romo-Tena J, la Garza RR, Bartnicki-Navarrete I, Alcocer-Varela J, Gómez-Martin D. Factors associated with remission in patients with systemic lupus erythematosus: new insights into a desirable state. Clin Rheumatol 2018;37:3033-3042. Disclosure of Interests: None declared