Abu Al Saoud, S.

Adrovic, Amra

Alexeeva, Ekaterina

Anton, Jordi

Appenzeller, Simone

Avcin, Tadej

Battagliotti, Christina

Berntson, Lillemor

Bica, Bianca

Brunner, Jürgen

Costa-Reis, Patricia

Eleftherioul, Despina

Feldman, Brian

Foeldvari, Ivan

Harel, L.

Helmus, Nicola

Horneff, Gerd

Janarthanan, Mahesh

Johnson, Sindhu

Kaiser, Daniela

Kallinich, Tilmann

Kasapcopur, Ozgur

Katsikas, Maria

Khubchandani, Raju

Klotsche, Jens

Kostik, Mikhail

Lazarevic, D.

Lehman, Thomas

Malcova,  Hana

Marrani, Edoardo

Minden, Kirsten

Moll, Monika

Nemcova, D.

Nielsen, Stine

Nuruzzaman, Farzana

Opsahl Hetlevik, S.

Pain, Clare

Patwardhan, Anjali

Sakamoto, Ana Paula

Santos, Maria Jose

Sawhney, Sujata

Schonenberg‐Meinema, Dieneke

Sifuentes‐Giraldo, Walter‐Alberto

Smith, Vanessa

Stanevicha, Valda

Sztajnbok, Flavio

Terreri, Maria Teresa

Torok, Kathryn S.

Uziel, Yosef

Vasquez‐Canizares, Natalia

Annals of the Rheumatic Diseases

English

Riga Stradins university

Scientific Abstracts  289Conclusion: Serious infections are a major cause of mortality and damage accrual in SLE. Constitutional symptoms, gastrointestinal involvement, current and cumulative steroid dose and cyclophosphamide use predict serious infec-tions. TB prophylaxis in patients with SLE should be considered in endemic areas, especially when using high-dose steroid therapy.Table 1. Cox proportional hazard models for predictors of infectionCovariates Hazard ratio 95% CIFever* 8.51 1.17-61.45Serositis 0.81 0.41-1.59Gastrointestinal involvement* 4.76 1.94-19.94Major organ manifestation 1.07 0.49-2.31SLEDAI-2K 0.99 0.95-1.05Daily steroid dose (/10mg)* 1.36 1.14-1.62Mean cumulative steroid dose* 1.004 1.002-1.005Albumin 1.01 0.66-1.55Absolute lymphocyte count 0.99 0.99-1Cyclophosphamide use* 2.22 1.11-4.46*-significant baseline predictors of serious infection on follow up. Major organ manifestation referes to presence of nephritis or neuropsychiatric lupus.Figure 1. A. The Kaplan meier survival curve depicts time to first serious infection. B. The Kaplan meier survival curve shows the overall survival difference between those with any serious infection ever compared to those with no serious infection in the disease course.REFERENCES: NIL.Acknowledgements: NIL.Disclosure of Interests: None Declared.DOI: 10.1136/annrheumdis-2023-eular.5338POS0138 EFFICACY AND SAFETY OF BELIMUMAB IN ADULTS WITH CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS: PRELIMINARY RESULTS FROM A PROSPECTIVE OBSERVATIONAL STUDYKeywords: Outcome measures, bDMARD, Systemic lupus erythematosusD. Dimopoulou1, M. Trachana2, P. Pratsidou-Gertsi2, A. Theodoridou1, T. Dimitroulas1, N. Kougkas1, A. Garyfallos1. 1Aristotle University, 4th Academic Department of Internal Medicine Clinic, Ippokrateio General Hospital of Thessaloniki, Thessaloniki, Greece; 2Aristotle University, 1st Academic Department of Pediatrics, Aristotle University, Thessaloniki, GreeceBackground: Childhood-onset Systemic Lupus Erythematosus (cSLE) is a rare autoimmune disease with multi-system manifestations, more severe disease course and higher frequency of morbidity than adult-onset SLE. Belimumab is the first treatment for cSLE approved for children ≥5 years of age.Objectives: To investigate the efficacy and safety of Belimumab in adult patients with cSLE.Methods: A prospective observational (non-interventional) study, involving adult patients with cSLE was conducted. During the 9-yearstudy period (01/2015 to 12/2022), adults with a cSLE diagnosis and Belimumab receivers (by intravenous or subcutaneous administration) for >12 consecutive months were enrolled. All patients met the revised 1997 American College of Rheumatology (ACR) or 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria and were followed at regular intervals (up to 6 months) in the Transition Rheuma-tology Outpatient Clinic. SLE activity was defined according to SLEDAI-2K and the SELENA-SLEDAI Physician Global Assessment (PGA), scale 0–3 [1]. At the last follow-up visit, response to therapy was assessed by Lupus Low Disease Activity State (LLDAS), remission state and SLE Responder Index (SRI4). LLDAS was defined as: i) SLEDAI-2K ≤4, with no activity in major systems ii) no new lupus disease activity compared to previous evaluation iii) a SELENA-SLEDAI PGA ≤1 iv) current prednisolone (or equivalent) dose ≤7.5 mg daily and v) standard mainte-nance doses of immunosuppressive drugs. [2]. Remission was defined as i) clinical SLEDAI-2K=0 ii) dose of prednisone ≤5 mg/day according to the DORIS definition [3]. SRI4 was defined as i) ≥4-point reduction from baseline in SELENA-SLEDAI score, ii) no worsening in PGA and iii) no new BILAG A organ domain score or 2 new BILAG B organ domain scores compared with baseline [4].Results: A total of 15 patients (14 females) were enrolled in the study. At baseline, the patients’ current mean (SD) age was 25.4 (7) years and the interval from disease onset to first Belimumab administration 12.4 (7.3) years. Half of the patients had a history of lupus nephritis and 42.8% were aPL positive. All patients were under hydroxychloroquine treatment and 80% of them were additionally receiving a sec-ond immunosuppressive agent (Methotrexate, MMF, Azathioprine). Glucocorticoids (GCs) were concomitantly administered in 13 (86.6%) patients in a dose of ≥7.5mg in 57.1% of them. The patients’ SLEDAI-2K mean (SD) score before Belimumab initiation was 12.1 (2.3), indicating high disease activity. The mean (SD) duration of Belimumab administration was 36.2 (1.9) months. At the last follow up visit, ongoing therapy with Belimumab was recorded in 66.7% of the cohort. SLEDAI-2K mean (SD) score was reduced to 3.5 (2.3), 1 patient (6.6 %) was in remission, 9 (60%) patients had mild and none high disease activity. The majority (78.5%) met the LLDAS definition and 78.5% were SRI4 Responders. Half of the patients (53.8%) achieved lower doses or discontinuation of GCs and 26.7% accomplished a reduc-tion of the immunosuppressant’s dose. Reasons for Belimumab discontinuation included pregnancy issues, infection and low adherence (3, 1, 1 patient respec-tively). None of the patients experienced a serious adverse event.Conclusion: In this cohort of adult patients with cSLE, Belimumab was well-tol-erated and effectively reduced disease activity allowing the downstream of GCs’s dose.REFERENCES: [1] MOSCA M, BOMBARDIERI S: Assessing remission in systemic lupus ery-thematosus. Clin Exp Rheumatol. 2006;24:99-104.[2] FRANKLYN K, et al: Definition and initial validation of a Lupus Low Disease Activity State (LLDAS). Ann Rheum Dis. 2016;75:1615-21.[3] VAN VOLLENHOVEN R et al: A framework for remission in SLE: consensus findings from a large international task force on definitions of remission in SLE (DORIS). Ann Rheum Dis. 2017;76:554-61.[4] KMA C Luijten et al: The systemic Lupus Erythematosus Responder Index (SRI); a new SLE disease activity assessment. Autoimmun Rev 2012;11:326-9Acknowledgements: NIL.Disclosure of Interests: None Declared.DOI: 10.1136/annrheumdis-2023-eular.5470POS0139 DIFFUSE JUVENILE SYSTEMIC SCLEROSIS PATIENTS SHOW DISTINCT ORGAN INVOLVEMENT, ANTIBODY PATTERN AND HAVE SIGNIFICANTLY MORE SEVERE DISEASE IN THE LARGEST JSSC COHORT OF THE WORLD. RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORTKeywords: Systemic sclerosisI. Foeldvari1, J. Klotsche2, K. Torok3, O. Kasapcopur3, A. Adrovic3, M. T. Terreri3, A. P. Sakamoto3, B. Feldman3, F. R. Sztajnbok3, J. Antón3, V. Stanevicha3, S. Johnson3, R. Khubchandani3, D. Schonenberg3, E. Al-Abadi3, E. Alexeeva3, M. Katsikas3, S. Sawhney3, V. Smith3, S. Appenzeller3, T. Avcin3, M. Kostik3, T. Lehman3, H. Malcova3, E. Marrani3, C. Pain3, A. Patwardhan3, W. A. Sifuentes-Giraldo3, N. Vasquez-Canizares3, P. Costa Reis3, M. Janarthanan3, M. Moll3, D. Nemcova3, M. J. Santos3, S. Abu Al Saoud3, C. Battagliotti3, L. Berntson3, B. Bica3, J. Brunner3, D. Eleftheriou3, L. Harel3, G. Horneff3, D. Kaiser3, T. Kallinich3, D. Lazarevic3, K. Minden3, S. Nielsen3, F. Nuruzzaman3, S. Opsahl Hetlevik3, Y. Uziel3, N. Helmus1. 1Hamburg Centre for Pediatric and Adolescence Rheumatology, An der Schön Klinik Hamburg Eilbek, Hamburg, Germany; 2German Rheumatism Research Center, German Rheumatism Research Center, Berlin, Germany; 3jSSc collaborative group, Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, GermanyBackground: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. In adult patients there are significant dif-ferences between the clinical presentation of diffuse and limited subtypes. We reviewed clinical differences in presentation of subtypes in patients in the juvenile systemic scleroderma inception cohort (jSScC).Objectives: To study the clinical presentation of jSSc patients with diffuse (djSSc) and limited (ljSSc) subtypes.Methods: We reviewed the baseline clinical characteristics of the patients, who were recruited to the jSScC till December 2022. jSScC is a prospective cohort of jSSc patients, who developed the first non-Raynaud´s symptom before the age of 16 years and are under the age of 18 years at the time of inclusion.Results: The JSScC included 232 patients, 68% (n=159) had diffuse subtype. The median age at onset of Raynaud phenomenon was 10.4 years (7.3-12.9), at the first non-Raynaud symptom 10.9 years (7.3-13.0) and median disease duration 2.5 years (1.0-4.6). The female/male ratio was significantly lower in the djSSc subtype  on November 17, 2023 by guest. Protected by copyright.http://ard.bmj.com/Ann Rheum Dis: first published as 10.1136/annrheumdis-2023-eular.2037 on 30 May 2023. Downloaded from 290  Scientific Abstracts(3:1 versus 5:1, p<0.001). Antibody profile was similar, with the exception of a sig-nificantly higher number of anticentromere positive patients in the ljSSc. Decreased FVC<80% was found in approximately 30% and decreased DLCO<80% was found in around 40% in both subtypes. Abnormal HRCT findings were found in 44% of patients. Pulmonary hypertension assessed by ultrasound occurred in approx-imately 5% in both groups and gastrointestinal involvement in 43% of djSSc and 36% in ljSSc (p=0.303). Patients with djSSc had significantly higher modified Rodnan Skin Score, more frequently sclerodactyly, a history of digital ulceration active ulceration, telangiectasia, a decreased Body Mass Index z score ≤ -2 and decreased joint range of motion. Patients with ljSSc had significantly higher rate of cardiac involvement. Regarding patient related outcomes assessed by VAS 0-100 djSSc patients had more severe disease also physician related outcome assessed by VAS 0-100 were significantly higher in djSSc (see Table 1).Table 1. Comparison of subtypes at time of inclusion in the cohort Whole GroupN=232Diffuse SubtypeN=159Limited SubtypeN=73P value Anticentromere 5% (7/156) 2% (2/106) 10% (5/50) 0.022 MRSS, median (IQR) 10 (4 – 20) 16 (8 - 27) 4 (0 – 8) 0.001 Gottron Papules 26% (59/228) 31% (48/155) 15% (11/73) 0.011 Sclerodactyly 75% (165/219)  85% (127/150) 55% (38/69) <0.001 Telangiectasia 37% (77/209)  44%(62/141) 22% (15/68) 0.002 History of ulceration 52% (119/229) 62% (98/158) 30% (21/71) <0.001 Active ulceration 17% (39/229) 21% (33/158) 8% (6/71) 0.021 Only Cardiac involvement5% (12/232) 3% (4/159) 11% (8/73) 0.007 BMI < - 2 z score 15% (33/217) 20% (29/148) 6% (4/69) 0.008 Joints with decreased range 59% (136/231)  64% (101/158)  48% (35/73) 0.022Physician Reported(Median, IQR)     Physician global disease activity30 (20 – 45)n=19735 (20– 50)n=13820 (10 – 30)n=590.001 Physician global disease damage30 (15 – 40)n=19530 (20 – 45)n=13820 (5 – 30)n=570.004 Physician ulceration activity0 (0 – 16)n=2165 (0 – 20)n=1540 (0 – 0)n=620.018Patient Reported(Median, IQR)     Patient global disease activity40 (20 – 50)n=17840 (20 – 50)n=12930 (15 – 55)n=490.024 Patient global disease damage30 (15 – 60)n=17740 (20 – 60)n=12825 (5 – 55)n=490.001 Patient Raynaud activity30 (10 – 60)n=20230 (10 – 60)n=14515 (0 – 55)n=570.001 Patient ulceration activity0 (0 – 30)n=20310 (0 – 30)n=1450 (0 – 20)n=580.001Conclusion: In the largest jSSc cohort in the world, djSSc patients have a sig-nificantly more severe disease. Patients and physician related outcomes were significantly more severe in djSSc group. Interestingly, we found no differences regarding interstitial lung disease, pulmonary hypertension or gastrointestinal involvement, although the number of patients with decreased BMI ≤ -2 z score was significantly higher in the djSSc patients.REFERENCES: NIL.Acknowledgements: NIL.Disclosure of Interests: None Declared.DOI: 10.1136/annrheumdis-2023-eular.2037POS0140 PATIENT AND PHYSICIAN REPORTED OUTCOMES OF JUVENILE SYSTEMIC SCLEROSIS PATIENTS SIGNIFICANTLY IMPROVE OVER 24 MONTHS OBSERVATION PERIOD IN THE JUVENILE SYSTEMIC SCLERODERMA INCEPTION COHORTKeywords: Systemic sclerosisI. Foeldvari1, J. Klotsche2, O. Kasapcopur3, A. Adrovic3, K. Torok3, M. T. Terreri3, A. P. Sakamoto3, J. Antón3, B. Feldman3, R. Khubchandani3, M. Kostik3, T. Lehman3, E. Marrani3, M. Katsikas3, D. Nemcova3, M. J. Santos3, F. R. Sztajnbok3, S. Appenzeller3, C. Battagliotti3, L. Berntson3, J. Brunner3, L. Harel3, G. Horneff3, S. Johnson3, T. Kallinich3, K. Minden3, M. Moll3, F. Nuruzzaman3, A. Patwardhan3, D. Schonenberg3, N. Helmus1. 1Hamburg Centre for Pediatric and Adolescence Rheumatology, An der Schön Klinik Hamburg Eilbek, Hamburg, Germany; 2German Rheumatism Research Center, German Rheumatism Research Center, Berlin, Germany; 3jSSc collaborative group, Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, GermanyBackground: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prev-alence of 3 in 1 000 000 children. The Juvenile Systemic Scleroderma Inception cohort (jSScC) is the largest cohort of jSSc patients in the world. The jSScC collects data prospectively in jSSc, allowing the evaluation of the development of organ involvement and patient and physician reported outcomes in jSSc over time.Objectives: To review the changes in the clinical characteristics, and patient and physician reported outcomes, over a 24 month observation period from the time of inclusion into the cohort.Methods: The jSScC cohort enrolls jSSc patients who developed the first non-Raynaud´s symptom before the age of 16 years and are under the age of 18 years at the time of inclusion. We reviewed jSScC patient clinical data and patient and physician reported outcomes, who had 24 months follow up from the time of inclusion until 1st of December 2022.Results: We extracted data from 90 patients, 77% of them had the diffuse sub-type. The female/male ratio was 3.5:1. Median age of onset of Raynaud´s was 9.4 years and the median age of onset of non-Raynaud´s was 10.0 years. Eighty-nine percent of the patients were treated with disease modifying anti-rheumatic drugs (DMARDs) at time of inclusion in the cohort (T0) and 96% after 24 months (T24). Median disease duration was 2.4 years at T0. No patient died during the follow up. Antibody profile stayed unchanged. Only 3 clinical parameters changed and all improved significantly, the median modified Rodnan skin score improved from 11 to 8 (p=0.021), number of patients with joints with pain on motion decreased from 21% to 10% (p=0.04) and the number of patients with muscle weakness decreased from 13% to 4% (p=0.03). All other organ involvement did not show any statistically signif-icant change from T0 to T24. All collected patient reported outcomes improved sig-nificantly from T0 to T24: the patient reported disease activity by VAS 0-100 from 40 to 20 (p=0.001), the patient reported disease damage by VAS 0-100 from 35 to 20 (p=0.027), patient reported ulceration activity by VAS 0-100) from 8 to 0 (p=0.001) and the patient reported Raynaud activity by VAS 0-100 from 20 to 10 (p=0.002). Two of the three physician reported outcomes improved significantly, the physician global disease activity by VAS 0-100 from 30 to 20 (p=0.001) and physician reported ulceration activity by VAS 0-100 from 5 to 0 (p=0.017). (Table 1.)Table 1. Items with significant improvement over 24 months observation period in the cohort 0 monthN=9024-month follow-upN=90P ValueMRSS, median (IQR) 11(5 – 20)8(2 – 20)0.021Presence of jointswith pain onmotion 21%(19/89) 10%(9/89)0.040Muscle Weakness  13%(10/75)4%(3/81)0.030Physician globaldisease activity30 (20 – 45)n=7120 (10 – 33)n=830.001Physicianulceration activity5 (0 – 19)n=860 (0 – 10)n=890.017Patient globaldisease activity40 (20 – 54)n=6620 (10 – 30)n=760.001Patient globaldisease damage35 (20 – 60)n=6620 (10 – 35)n=760.027PatientRaynaud activity20 (0 – 50)n=8310 (0 – 26)n=840.002Patientulceration activity8 (0 – 28)n=840 (0 – 10)n=840.001Conclusion: Skin and musculoskeletal clinical features improved over 24 months, with almost all patients on DMARDs, supporting the response of these features to therapy. It was promising that internal organ involvement, like cardiac, lung and gastrointestinal, did not significantly worsen or increase. The most strik-ing observation is the positive direction and improvement all patients and two of the three physician reported outcome measures over 24 months in this large international cohort.REFERENCES: NIL.Acknowledgements: NIL.Disclosure of Interests: None Declared.DOI: 10.1136/annrheumdis-2023-eular.2073POS0141 APPLICATION OF CRISS SCORE, REVISED CRISS SCORE AND RCID SCORE IN PATIENTS WITH JUVENILE SYSTEMIC SCLEROSISKeywords: Systemic sclerosisJ. Klotsche1, I. Foeldvari2, K. Torok3, F. Del Galdo4, D. Furst5, O. Kasapcopur6, A. Adrovic6, B. Feldman6, M. T. Terreri6, A. P. Sakamoto6, F. R. Sztajnbok6, J. Antón6, M. Katsikas6, V. Stanevicha6, S. Appenzeller6, T. Avcin6, S. Johnson6, M. Kostik6, H. Malcova6, E. Marrani6, W. A. Sifuentes-Giraldo6, R. Khubchandani6, D. Nemcova6, M. J. Santos6, D. Schonenberg6, C. Battagliotti6, L. Berntson6, B. Bica6, J. Brunner6, D. Eleftheriou6, L. Harel6,  on November 17, 2023 by guest. Protected by copyright.http://ard.bmj.com/Ann Rheum Dis: first published as 10.1136/annrheumdis-2023-eular.2037 on 30 May 2023. Downloaded from 

DIFFUSE JUVENILE SYSTEMIC SCLEROSIS PATIENTS SHOW DISTINCT ORGAN INVOLVEMENT, ANTIBODY PATTERN AND HAVE SIGNIFICANTLY MORE SEVERE DISEASE IN THE LARGEST JSSC COHORT OF THE WORLD. RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT

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DIFFUSE JUVENILE SYSTEMIC SCLEROSIS PATIENTS SHOW DISTINCT ORGAN INVOLVEMENT, ANTIBODY PATTERN AND HAVE SIGNIFICANTLY MORE SEVERE DISEASE IN THE LARGEST JSSC COHORT OF THE WORLD. RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT

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