ObjectiveTo evaluate the application of the EULAR/ACR-2019 criteria to monogenic lupus patients and compare its performance against the SLICC-2012 criteria. MethodsIn a multicenter retrospective cohort study, consecutive patients with monogenic lupus from three tertiary lupus clinics were enrolled. The diagnosis of monogenic lupus was based on the expert physician's opinion or ful lling the SLICC-2012 criteria. All enrolled patients had genetic variants. A control group of sporadic childhood SLE (cSLE) and non-SLE patients, were included. A descriptive data analysis was conducted, and the EULAR/ACR-2019 and SLICC-2012 criteria were applied to both groups. ResultsForty-nine patients with monogenic lupus with a median age at diagnosis of 6.0 (IQR 3.0-10.8) years, and 104 controls (55 patients with cSLE and 49 non-lupus patients with a median age at diagnosis of 10.0 and 5.0 respectively) were included. Forty-four (89.8%) patients with monogenic lupus ful lled the EULAR/ACR-2019 with a mean score of 22.3±8.9. The most frequent domains were immunologic (93.9%), musculoskeletal and renal (each 57.1%), and mucocutaneous (55.1%). Fifty-four (98.2%) cSLE patients and six (12.2%) non-lupus patients met the EULAR/ACR-2019 criteria with a mean score of 22.5±9.2 and 8.5±5.2, respectively. The sensitivity of the EULAR/ACR-2019 criteria in monogenic lupus was 95.6% (95% CI: 0.8-0.9) while the speci city was 75.0% (95% CI: 0.5-0.9). ConclusionThis is the rst and largest cohort of monogenic lupus patients testing the performance of the 2019-EULAR/ACR criteria. It e ciently classi es monogenic lupus patients, irrespective of the underlying genetic variants. Further studies are needed before these criteria are adopted worldwide.

Background Systemic juvenile idiopathic arthritis (sJIA) is a diagnosis of exclusion. The complex nature and clinical variety of the disease, as well as the vast clinical variation of disease presentation, may lead to difficulties in disease detection and subsequent delays in treatment. Aim To provide a consensus guidance on the management of newly diagnosed sJIA patients among pediatric rheumatologists in Arab countries. Methods This work was conducted in two phases. The first phase utilized an electronic survey sent through an email invitation to all pediatric rheumatologists in Arab countries. In the second phase, a Task Force of ten expert pediatric rheumatologists from Arab countries met through a series of virtual meetings. Results obtained in phase one were prioritized using a nominal group and Delphi-like techniques in phase two. Results Seven overarching principles and a set of recommendations were approved by the Task Force to form the final consensus. Conclusion This is the first consensus on a clinical approach for pediatric rheumatic diseases among Arab pediatric rheumatologists. It is presented as a guidance on the clinical approach to sJIA that requires further evidence, and future updates are anticipated.

Objective: Subcutaneous methotrexate injections are considered to be more effective or work faster than oral methotrexate. Therefore, the extent and the kinetics of response were analyzed in juvenile idiopathic arthritis patients treated with oral versus subcutaneous methotrexate. Methods: The BIKER databank was searched for biologics-naive juvenile idiopathic arthritis patients treated with methotrexate as initial treatment. The Juvenile Arthritis Disease Activity Score-10 definition of remission and the pediatric American College of Rheumatology's response parameters were utilized as outcome criteria. Result: A total of 410 polyarticular juvenile idiopathic arthritis patients receiving oral methotrexate were compared to 384 patients receiving subcutaneous methotrexate. Rheumatoid factor-negative polyarthritis was the most common juvenile idiopathic arthritis category (50%/51%) in this cohort followed by extended oligoarthritis (27%/26%), polyarticular psoriatic arthritis (18%/16%), and few had rheumatoid factor-positive polyarthritis (5%/8%). The oral cohort’s disease duration (2.3 ± 3.0 vs. 1.9 ± 2.7) was significantly longer ( P = .04), although their age at onset and baseline were similar. Furthermore, at baseline, disease activity (Juvenile Arthritis Disease Activity Score-10 16.5 ± 7.2 vs. 14.7 ± 8.2; P = .001 due to a higher active joint count 9.0 ± 10.1 vs. 7.4 ± 7.7; P = .011) was higher in the subcutaneous cohort. The weekly methotrexate doses were comparable with 13.6 ± 5.4 mg/m 2 and 13.3 ± 4.5 mg/m 2 , respectively. With oral/subcutaneous methotrexate, a pediatric American College of Rheumatology’s 90 was achieved in 98(38.3%)/128(40.4%), while 96(38.1 %)/75(40.1%) attained Juvenile Arthritis Disease Activity Score remission after 12 months of therapy. There was no difference in the early kinetics of response according to Kaplan–Meyer analysis. Adverse events including nausea, vomiting, and increased transaminases were considerably more common after methotrexate subcutaneous administration than after oral treatment. Conclusion: In terms of effectiveness, but not safety, our retrospective analysis found some advantages of subcutaneous methotrexate. Adverse effects limit treatment continuance and thus must be considered a disadvantage. Furthermore, oral methotrexate eliminates the need for injections, which is especially essential for younger children. Controlled, randomized prospective trials in children and juvenile patients are necessary for definitive recommendations for the subcutaneous route of administration of methotrexate therapy.

BackgroundMethotrexate is approved and recommend as first line disease modifying antirheumatic drug (DMARD) in polyarticular juvenile idiopathic arthritis (JIA). In can be used orally or via s.c. injection.ObjectivesS.c MTX is thought to be more efficious or act more rapidly than oral MTX. Thus we want to analyse the kinetic of response in JIA patients treated with oral versus s.c. MTX.MethodsIn the German BIKER registry a cohort of biologics naïve JIA patients starting treatment with MTX was built. The data bank was screened for patients treated with MTX orally vs. s.c for the first time. The JIA-ACR 90 and the JADAS10 definition of remission were used as outcome parameters.Results410 JIA patients received treatment with oral MTX and 384 received s.c. MTX. RF negative polyarthritis was the most common JIA category (50%/51%) followed by by extended oligoarthritis (27%/26%), polyarticular psoriatic arthritis (18%/16%) and RF positive polyarthritis (5%/8%). Disease duration (2.3+/-3.0 vs 1.9+/-2.7 was statistically higher in the oral cohort (p=0.04) but age at onset and baseline were similar. The baseline disease activity was higher in the s.c. cohort (JADAS10 16.5+/-7.2 compared to 14.7+/-8.2; p<0.001 and active joint count 9.0+/-10.1 vs. 7.4+/-7.7; p=0,011). The weekly MTX dosages were comparable with 13.6+/-5.4mg and 13.3+/-4.5 mg. Concomitant treatment with NSAIDS (95%/89%), oral steroids (24%/25%) or intraarticular steroids (6%/8%) were comparable.After 12 months of treatment, 150 (38.3%) reached a JIA ACR90 with oral MTX and 131 (35%) with s.c MTX while 86 (21. 8%) and 72 (19.5%) reached JADAS-remission (JADAS10≤1). By Kaplan-Meyer- analysis no difference in the early kinetic of response was found. Upon total observation for up to 7.5 years in the intention to treat population (patients discontinuing MTX due to inefficacy or intolerance or starting a biologic were calculated as non-responders) more patients in the oral cohort reached a JADAS-remission (162; 41%) than with s.c MTX (126; 34%) which was stastically borderline significant (p=0,05; odd's ratio 1.2 [95CI 1.0–1.8]).ConclusionsData from the BIKER registry out of the clinical practice do show a high rate of JIA patients reaching a significant JIA-ACR response as well as JADAS-remission upon MTX as a sole DMARD. However, on the long term more patients with oral MTX reached JADAS remission. By Kaplan Meyer analyses we did not observe a superiority of s.c. MTX in the kinetic of response. The limitations of our analysis lie the character of a registry study, the lack of randomisation and study protocol leaving all decisions to start or to stop MTX by the responsible rheumatologist. Thus such data are preliminary and should be confirmed by randomized studies.Disclosure of InterestNone declared

ObjectiveTo evaluate the application of the EULAR/ACR-2019 criteria to monogenic lupus patients and compare its performance against the SLICC-2012 criteria. Methods In a multicenter retrospective cohort study, consecutive patients with monogenic lupus from three tertiary lupus clinics were enrolled. The diagnosis of monogenic lupus was based on the expert physician’s opinion or fulfilling the SLICC-2012 criteria. All enrolled patients had genetic variants. A control group of sporadic childhood SLE (cSLE) and non-SLE patients, were included. A descriptive data analysis was conducted, and the EULAR/ACR-2019 and SLICC-2012 criteria were applied to both groups.Results Forty-nine patients with monogenic lupus with a median age at diagnosis of 6.0 (IQR 3.0-10.8) years, and 104 controls (55 patients with cSLE and 49 non-lupus patients with a median age at diagnosis of 10.0 and 5.0 respectively) were included. Forty-four (89.8%) patients with monogenic lupus fulfilled the EULAR/ACR-2019 with a mean score of 22.3±8.9. The most frequent domains were immunologic (93.9%), musculoskeletal and renal (each 57.1%), and mucocutaneous (55.1%). Fifty-four (98.2%) cSLE patients and six (12.2%) non-lupus patients met the EULAR/ACR-2019 criteria with a mean score of 22.5±9.2 and 8.5±5.2, respectively. The sensitivity of the EULAR/ACR-2019 criteria in monogenic lupus was 95.6% (95% CI: 0.8-0.9) while the specificity was 75.0% (95% CI: 0.5- 0.9). ConclusionThis is the first and largest cohort of monogenic lupus patients testing the performance of the 2019-EULAR/ACR criteria. It efficiently classifies monogenic lupus patients, irrespective of the underlying genetic variants. Further studies are needed before these criteria are adopted worldwide.