AB0995 JUVENILE ONSET SYSTEMIC LUPUS ERYTHEMATOSUS WITH SJÖGREN'S SYNDROME: CLINICAL AND LABORATORY FEATURES.

Abstract

Background: Systemic lupus erythematosus with juvenile onset (jSLE) with Sjogren's syndrome (SS) in children is a poorly studied and rare combination, the frequency of which, according to the literature, is 7.5-10.0%1. Objectives: To study demographic data, specific features of jSLE with SS in single center. Methods: Retrospective study of all consequently patients (pts) of single-center in pediatric department with combination of jSLE and SS. Results: SS was verified in 14 pts with jSLE (14.3% were boys), which amounted to 15.5% of all pts with jSLE. The median age of jSLE onset was 13.5 y.o. [9.3; 14.9]. The median of disease duration at the time of SS verification was 1.3 y [0.6; 2.9]. Expressed constitutional disorders (fever, weight loss) observed in 13 pts (91.7%). 11 pts (78.6%) had acute cutaneous lupus, 5 pts (35.7%)-chronic cutaneous lupus, 3 (21.4%)-oral and nasal ulcers, 6 (42.8%)-nonscarring alopecia, 9 pts (64.3%)-polyarthritis, 2 pts (14.3%)-renal involvement, 2 pts (14.3%)-serositis, 1 (7.1%)-interstitial lung disease, 5 pts (35.7%)-neuropsychiatric disorder, including psychosis in 2 (14.3%). 4 patients had skin lesions atypical for SLE (1-annular erythema, 1-erythema nodosum, 2-Ro-associated skin vasculitis). 12 pts (85.7%) had generalized lymphadenopathy.12 pts (85.7%) had various hematological disorders: anemia-in 5 pts (35.7%), leukopenia-in 9 (64.3%), isolated lymphopenia in 1 (7.1%), thrombocytopenia-in 4 pts (28.6%). 13 pts had isolated involvement of salivary glands, 1-combined with lacrimal glands. The decrease in salivary gland function was recorded in 50% of cases, hypolacrimia-in one case. Recurrent parotitis was present in only one case (7.1%). ANA were detected in 100% pts, anti-dsDNA-in 10 pts (71.4%), anti-Sm-in 7 pts (50.0%), anti-Ro-in 10 (71.4%), anti-La-in 7 (50%), RNP-70-in 5 pts (35.7%), RF+-in 6 pts (42.9%), hypocomplementemia-in 3 pts (21.4%). The most common was the combination of positive ANA, anti-dsDNA, antiRo with acute cutaneous lupus, polyarthritis, generalized lymphadenopathy and expressed constitutional disorders-8 pts (57.1%). 4 pts (28.6%) had polyclonal hypergammaglobulinemia. 3 pts (21.4%) had concomitant autoimmune non-rheumatic disease; 1-autoimmune hepatitis, 1-type 1 diabetes mellitus, 1-autoimmune thyroiditis. Median disease activity by SLEDAI at the time of jSLE verification was 11.5 scores [9.25;15.7]. Conclusion: According to our results, the frequency of detection of secondary SS in jSLE was higher than the literature data. The clinical features include a high frequency of constitutional disorders, lymphadenopathy, skin manifestations, high frequency of antiRo with a significantly lower incidence of kidney involvement, serositis than jSLE without SS. In pts with a diagnosis of SLE, the possibility of developing secondary SS should be considered (specially in girls with antiRo positive), the early detection of which affects the choice of therapy and prognosis. References: [1]Malagon C, Gomez M, Mosquera C et al. Juvenile polyautoimmunity in a rheumatology setting. Autoimmunity Reviews, Volume 18, Issue 4, 2019, p 369-381. https://doi.org/10.1016/j.autrev.2018.11.006.