SYSTEMIC LUPUS ERYTHEMATOSUS IN CHILDHOOD AND ADOLESCENCE - UPDATE FROM THE NATIONAL PEDIATRIC RHEUMATOLOGY DATABASE

Background: Systemic lupus erythematosus (SLE) is a clinically heterogeneous disease, which begins in childhood and adolescence in 15 - 20% of cases. Since 2004, data on SLE have been collected by means of a disease-specific questionnaire as part of the National pediatric rheumatology database (NPRD) in Germany. Since 2014, kidney biopsy results have been recorded to further specify kidney involvement. Objectives: Evaluation of clinical signs and symptoms, outcome and laboratory data of patients with juvenile systemic lupus erythematosus from a large database in Germany. Methods: Data from patients with SLE recorded in the NPRD in 2017 were considered for the analysis. In addition to age, sex, onset of disease, the criteria that led to the diagnosis, various laboratory parameters, organ involvement (current, ever) and therapy (current, last 12 months), current disease activity (numerical rating scale 0-10, NRS) and ECLAM (score 0-10) were recorded. Patient-reported outcomes included global assessments of overall-wellbeing and fatigue (NRS 0-10) and functional ability (CHAQ). Results: 196 patients (86% female) with a median age of 16 years were documented. Criteria most frequently met at diagnosis included “antinuclear antibodies” (88%), followed by “anti-ds-DNA-Ab” (66%), “butterfly erythema” (42%) and “arthritis” (41%). A positive family history was found in 10% of patients. At documentation, 85% of patients received disease-modifying anti-rheumatic drugs, most frequently hydroxychloroquine (73%), followed by mycophenolate mofetil (32%) and azathioprine (17%). Systemic glucocorticoids obtained 52% of patients, 12% ≥ 0.2 mg/kg/day. Biologics (rituximab 2%) and cyclophosphamide i.v. (3%) were rarely administered during the last 12 months. Disease activity was reported as 1.0 (NRS, median, IQR 0 - 9), ECLAM as 1.0 (median, range 0 - 10). In the laboratory, leukopenia 25 mm in 15% of patients. Mean CHAQ was 0.24, and 86% of patients had a CHAQ score The following organ involvement was ever present: general symptoms 84%, skin/mucosa 72%, joints 73%, thyroid 15%, muscle 25%, lungs 17% and CNS 30%. In 45/190 (24%) patients, a kidney involvement was stated. In 34 patients (75%) a kidney biopsy was performed and histology yielded the following results: Class 1: 6.7%, Class 2: 16.7%, Class 3: 40.0%, Class 4: 23.3%, Class 5: 13.3%. Conclusion: The most common clinical symptoms documented in juvenile SLE patients were skin and joint involvement. In the course of the disease, a quarter of the patients developed kidney involvement, mostly proliferative nephritis. Apparently, azathioprine is increasingly being replaced by mycophenolate mofetil, biologicals have hardly been used so far. Although functional outcome and overall-wellbeing of jSLE patients was good, fatigue was a concern for some patients. Disclosure of Interests: Claudia Sengler: None declared, Martina Niewerth: None declared, Nils Geisemeyer: None declared, Hermann Girschick: None declared, Ariane Klein Consultant of: Celgene, Annette Friederike Jansson: None declared, Markus Hufnagel: None declared, Kirsten Minden Consultant of: GlaxoSmithKline, Sanofi, Speakers bureau: Roche