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]Malagón 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.Disclosure of Interests:None declared
A new allele of the lpr locus, lprcg, that complements the gld gene in induction of lymphadenopathy in the mouse.
