OBJECTIVE:The treatment landscape for systemic sclerosis-associated interstitial lung disease (SSc-ILD) has evolved with increasingly available immunosuppressive therapies (ISTs) and antifibrotic treatments. However, their real-world use remains unclear. The objective of this study was to analyze treatment trends and the effect of IST and antifibrotic treatments on ILD progression using the European Scleroderma Trials and Research database. METHODS:We included patients with SSc-ILD meeting the 2013 American College of Rheumatology/EULAR criteria with high-resolution computed tomography-confirmed ILD, pulmonary function, and therapy data, grouped into four time periods (≤2006, 2007-2011, 2012-2016, and ≥2017). We analyzed IST initiation, switching, discontinuation, and combination therapy. ILD progression was defined as a decline in the percentage of predicted forced vital capacity of 5% or greater or the percentage of predicted diffusing capacity of the lungs for carbon monoxide of 10% or greater over 12 ± 3 months. RESULTS:Among 1,409 patients, IST use at first evaluation increased significantly from 13.6% (≤2006) to 57.4% (≥2017) (P < 0.001). Mycophenolate mofetil emerged as the most prescribed IST (7% to 57%) (P < 0.001). Combination therapy rose from 17.9% to 26.9% (P < 0.001), whereas ILD progression rates declined from 21.3% (2007-2011) to 12.1% (≥2017) (P < 0.001). In the 2017 and later cohort, logistic regression showed shorter disease duration (odds ratio [OR] 0.991, 95% confidence interval [CI] 0.987-0.996; P < 0.001) and myositis (OR 9.9, 95% CI 1.94-51.76; P = 0.006) were associated with therapy initiation, whereas switching was higher in patients with a higher modified Rodnan skin score (OR 1.03, 95% CI 1.00-1.06; P = 0.035) and in patients with arthritis (OR 3.03, 95% CI 1.55-5.94; P = 0.001). Last, combination therapy was associated with younger age, higher dyspnea class, and arthritis. CONCLUSION:Our findings reveal a significant evolution in clinical practice. However, continued disease progression emphasizes the need for more effective therapeutic approaches.

Background Anifrolumab is approved for adults with moderate-to-severe active systemic lupus erythematosus (SLE) based on Randomised clinical trials (RCTs). While randomised pivotal RCTs have demonstrated their efficacy and safety, real-world evidence (RWE) remains limited.Objectives To describe the clinical characteristics, effectiveness and safety of anifrolumab in clinical practice and to assess findings from published observational studies.Methods Multicentre study of patients with SLE classified according to EULAR/American College of Rheumatology (ACR) 2019. Data were collected from medical records up to 30 April 2025. Variables included demographic characteristics, clinical and serological features, prior and concomitant therapies, disease activity indices including the SLE Disease Activity Index 2000 (SLEDAI-2K), SLE Disease Activity Score (SLE-DAS) and Physician Global Assessment (PGA), damage and disease control measures including the Systemic Lupus International Collaborating Clinics/ACR Damage Index, Lupus Low Disease Activity State (LLDAS) and Definitions of Remission in SLE (DORIS) remission and the association with adverse events (AEs). A review of published observational studies was also conducted.Results We included 206 patients (183 women; mean age 44.6±12.6 years) from 54 Spanish centres. The most common indications for anifrolumab were cutaneous (61.7%), musculoskeletal (48.5%) and haematological (27.2%).A rapid and sustained improvement was observed in disease activity (SLEDAI-2K, SLE-DAS, PGA), LLDAS, DORIS remission, serological markers (anti-double-stranded DNA, C3/C4) and corticosteroid tapering. Organ damage remained stable. After a mean follow-up of 7.5±5.3 months, the most frequent AEs were herpes zoster (n= 5), respiratory infections (n= 6) and headache (n= 3). Twenty patients discontinued treatment. These results were consistent with published observational studies.Conclusion In this large RWE cohort, anifrolumab demonstrated early and sustained clinical benefit, a favourable safety profile and a significant corticosteroid-sparing effect. These findings reinforce the evidence from CT and support the incorporation of anifrolumab into routine care for patients with SLE.

The treatment landscape for systemic sclerosis‐associated interstitial lung disease (SSc‐ILD) has evolved with increasing immunosuppressive (IST) and anti‐fibrotic therapies available. However, their real‐world use remains unclear. To analyze treatment trends and the effect of IST and anti‐fibrotic therapies on ILD progression using the EUSTAR database. We included SSc‐ILD patients meeting the 2013 ACR/EULAR criteria with high‐resolution CT‐confirmed ILD, pulmonary function, and therapy data, grouped into four time periods (≤2006, 2007–2011, 2012–2016, ≥2017). We analyzed IST initiation, switching, discontinuation, and combination therapy. ILD progression was defined as a decline in %FVC ≥5% or %DLCO ≥10% over 12 ± 3 months. Among 1,409 patients, IST use at first evaluation increased significantly from 13.6% (≤2006) to 57.4% (≥2017, p<0.001). Mycophenolate mofetil emerged as the most prescribed IST (7% to 57%, p<0.001). Combination therapy rose from 17.9% to 26.9% (p<0.001), while ILD progression rates declined from 21.3% (2007–2011) to 12.1% (≥2017, p<0.001). In the ≥2017 cohort, logistic regression showed shorter disease duration (Odds ratio (OR) 0.991, 95%CI 0.987–0.996, p <0.001) and myositis (OR 9.9, 95% CI 1.94‐51.76, p=0.006) were associated with therapy initiation, while switching was higher in patients with a higher mRSS (OR 1.03, 95%CI 1.00–1.06, p=0.035) and in patients with arthritis (OR 3.03, 95% CI 1.55–5.94, p=0.001). Lastly, combination therapy was associated with younger age, higher dyspnea class and arthritis. Our findings reveal a significant evolution in clinical practice. However, continued disease progression emphasizes the need for more effective therapeutic approaches. image

To evaluate the main outcomes of disease activity and their association with other measures of activity, damage, and quality of life in patients with idiopathic inflammatory myopathy (IIM) according to time since diagnosis and positivity to antisynthetase autoantibodies (ASAs). Cross-sectional multicenter study within the Spanish Myo-Spain registry. Cases were classified as incident (≤ 12 months since diagnosis) and prevalent. The main outcomes of disease activity were the Myositis Disease Activity Assessment visual analogue scale (MYOACT), the Manual Muscle Test 8 (MMT-8), physician global activity (PhGA), and extramuscular activity. Other measures of activity, damage, and quality of life included patient global disease activity, MYOACT muscular, creatine phosphokinase, Health Assessment Questionnaire, physician and patient global damage, global damage of the Myositis Damage Index, and the 12-item Short-Form Health Survey (SF-12). We analyzed associations using a multivariate generalized linear model and a simple linear regression model. A total of 554 patients with different diagnostic subgroups of IIM were included (136 incident and 418 prevalent cases), with 215 ASA-positive patients (58 incident and 157 prevalent cases). All measures of disease activity were higher in the incident cases (p < 0.05), except for MYOACT muscular and creatine phosphokinase, for which no differences were recorded in ASA-positive patients. No differences were found between incident and prevalent cases for measures of damage. Values for the physical component of the SF-12 were higher in the prevalent cases (p < 0.05). The multivariate model was initially significant overall for the main activity outcomes. Positivity to ASAs was positively and negatively associated with the MYOACT index and MMT-8, respectively (p < 0.05), although no association was recorded with PhGA and extramuscular activity. Prevalent cases were negatively associated with the main outcomes of activity, except with MMT-8, for which the association was positive (p < 0.05). The main activity outcomes validated in polymyositis and dermatomyositis could also be used in other subtypes of IIM, such as antisynthetase syndrome. Recent diagnosis is associated with greater disease activity, as assessed based on these activity outcomes. PhGA and extramuscular activity are not modified by ASA positivity, thus supporting their preferred use for assessing treatment response in IIM with ASAs.

Objective: To investigate the prevalence and clinical spectrum of atypical or non-classical complications in adult-onset Still’s disease (AOSD) beyond macrophage activation syndrome (MAS) and to identify factors linked to their occurrence. Methods: Multicenter cross-sectional study of AODS cases included in the Spanish registry on Still’s disease. Results: This study included 107 patients (67% women), of whom 64 (59.8%) developed non-classical complications. These include macrophage activation syndrome in 9.5%, atypical skin manifestations in 38.8%, cardiac involvement in 22.7% (comprising pericarditis, myocarditis, pulmonary arterial hypertension, and noninfectious endocarditis), pleuritis in 28.9%, transient pulmonary infiltrates in 4%, significant headache in 14.1%, lower abdominal pain with evidence of peritonitis in 8.4%, and secondary amyloidosis in 0.9%. In the multivariate logistic regression analysis, lymphadenopathy (OR 2.85, 95% CI 1.03–7.91, p = 0.044) and the systemic score system (SSC) index (OR 1.86, 95% CI 1.29–2.69, p = 0.001) were independently associated with the development of non-classical clinical manifestations. In contrast, typical exanthema was associated with a reduced risk of these complications (OR 0.32, 95% CI 0.11–0.95, p = 0.041). Conclusions: In addition to the typical clinical manifestations and MAS, a significant proportion of patients with AOSD develop uncommon complications, some of which can be potentially life-threatening. These should be considered in the evaluation and follow-up of patients. Early recognition and prompt management are crucial to significantly reduce morbidity and mortality.

Background: Anifrolumab (ANI) is a human monoclonal antibody that binds to the type I interferon receptor subunit 1 (IFNAR1), thus blocking the biological activity of type I IFNs. ANI was approved by Spanish authorities on June 1, 2023. Its use is indicated in adults with moderately to severely active autoantibody-positive systemic lupus erythematosus (SLE) in combination with standard treatment. Objectives: To describe in Spanish clinical practice since its approval a) SLE profile of patients, b) effectiveness and c) safety. Methods: Descriptive, retrospective, multicenter study in patients diagnosed with SLE according to EULAR/ACR 2019, SLICC and/or ACR 1997 diagnostic criteria. Data regarding were collected from medical records (June 2023-January 2024). Demographic features, clinical and laboratory variables, previous and concomitant therapy, activity index (SLE-DAS, SLEDAI-2k, PGA), organic damage index (SLICC SDI) and safety were assessed. Results: Baseline characteristics of patients and therapy before ANI are summarized in Table 1. A total of 91 patients (82 women/9 men), mean age 32.7±11.5 years (range 15-59 years) (38 hospitals) were included.The main reason for starting ANI was: skin activity (n=60, 65.9%), joint activity (n=52, 57.1%), hematological activity (n=25, 27.5%), renal activity (n=2, 2.2%), corticosteroids dependence (n=5, 5.5%) and serious side effects with belimumab (BLM) (n=2, 2.2%).All patients received 300 mg/4 w of ANI except one patient who received a loading dose (900 mg/4 w x3 months and after 300 mg/4 w) due to renal involvement.Concomitant treatments with ANI were: corticosteroids (n=78), antimalarials (n=68), mycophenolate mofetil (MMF) (n=23), methotrexate (MTX) (n=13), azathioprine (AZA) (n=5), tacrolimus (n=5), leflunomide (LFN) (n=2), rituximab (RTX) (n=1), cyclophosphamide (CYM) (n=1), sulfones (n=2) and anakinra (n=1).A rapid and maintained significant decreases in SLE-DAS, SLEDAI-2k, PGA and anti-dsDNA antibodies were observed from 1 month until the last visit. Complement C3 and C4 levels also increased significantly (Figure 1). No increase in the chronicity index was observed.After a follow-up of 4.8±3.6 months the main side effects observed were: herpes zoster (n=3), arterial hypotension (n=2), headache (n=2), suppurative hidradenitis (n=1), influenza A pneumonia (n=1), skin reaction (n=1), herpes simplex virus infection and urinary infection (n=1). In follow-up, 7 patients discontinued treatment due to primary failure (n=3), secondary failure (n=2), severe pneumonia (n=1), arterial hypotension (n=1). Conclusion: In our cohort of SLE patients in a real-world setting, ANI has showed a rapid effectiveness, and a relatively good safety. Therefore, ANI seems to be a good choice to treat patients refractory to other therapies. ANI in severe and refractory patients even was used combined with other biologic therapy. REFERENCES: [1] Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis. 2019 Sep;78(9):1151-1159. Acknowledgements: NIL. Disclosure of Interests: Vanesa Calvo-Río Abbie, Lilly, Grünenthal, AMGEN, MSD, Novartis, Galápagos, Vifor, GSK, Otsuka, Janssen, M. Retuerto-Guerrero: None declared, Judit Font: None declared, Ivette Casafont-Solé: None declared, A. Mayo-Juanatey: None declared, Juan Jose Alegre Sancho: None declared, Dalifer Freites: None declared, Cristina Hormigos: None declared, Noemí Garrido-Puñal: None declared, Guillermo Gonzalez Arribas: None declared, Juan Roberto Miguelez Sanchez: None declared, Andrea García-Valle: None declared, Marta Ibañez: None declared, Fernando Lozano Morillo: None declared, Ángel García Manzanares: None declared, S. Sandoval-Moreno: None declared, Josefina Cortés-Hernández: None declared, Deseada Palma Sanchez: None declared, Leticia Lojo: None declared, Evelin Cecilia Cervantes Pérez: None declared, Paz Collado: None declared, Cristina Arciniega Larios: None declared, Luis Sala Icardo: None declared, Eztizen Labrador-Sánchez: None declared, Cilia Peralta-Ginés: None declared, Nahia Plaza-Aulestia: None declared, Miguel Medina Malone: None declared, Jose Rosas Gómez de Salazar: None declared, Montserrat Corteguera: None declared, Laura Cebrián-Méndez: None declared, Fred Antonio Anton Pages: None declared, Jose Ramón Lamúa Riazuelo: None declared, Maria Dolores Fábregas Canales: None declared, María José Alados Hernández: None declared, Marta Garijo Bufort: None declared, Anna Pàmies: None declared, Luis Sarabia De Ardanaz: None declared, Rodrigo Aguirre-del-Pino: None declared, Jose Angel Cabezas Lefler: None declared, Alvaro Seijas-Lopez: None declared, Maria del Carmen Carrasco Cubero: None declared, Ana Lopez-Ceron Cofiño: None declared, Vera Ortiz-Santamaria: None declared, Santos Castañeda: None declared, Carmen Bejerano: None declared, Ricardo Blanco Abbvie, Pfizer, Roche, Bristol-Myers-Squibb, Janssen, Lilly, Novartis, UCB, and MSD, Abbvie, MSD, Roche.Figure 1Evolution of C3, C4 and anti-dsDNA levels and activity and organ damage indices after starting anifrolumab. Table 1Clinical manifestations and treatments received before starting anifrolumabClinical manifestations before ANIN (%)Therapy before ANIN (%)Concomitant therapy with ANIN (%)articular87 (95.6%)oral steroids88 (96.7%)oral steroids78 (85.7%)cutaneous75 (82.4%)antimalarials88 (96.7%)antimalarials68 (74.7%)hematological57 (62.6%)BLM73 (80.2%)MMF23 (25.3%)oral ulcers47 (51.6%)MMF46 (50.5%)MTX13 (14.3%)alopecia46 (50.5%)AZA38 (41.7%)AZA5 (5.5%)renal30 (33%)RTX34 (37.3%)tacrolimus5 (5.5%)serositis28 (30.8%)MP boluses32 (35.1%)leflunomide2 (2.2%)neuropsychiatric12 (13.2%)CYM19 (20.9%)sulfones2 (2.2%)digestive6 (6.6%)tacrolimus9 (9.9%)RTX1 (1.1%)Abbreviations in alphabetical order: ANI: anifrolumab; AZA: azathioprine; BLM: belimumab; CYM: cyclophosphamide; MMF: mycophenolate mofetil; MP: methylprednisolone; MTX: Methotrexate; RTX: rituximabCYM1 (1.1%)anakinra1 (1.1%)

Background: The treatment armamentarium with immunosuppressive treatments (ISTs) for interstitial lung disease (ILD) in systemic sclerosis (SSc) has greatly expanded over the past decades. The implementation in clinical practice and the impact of IST on ILD progression is to date unclear. To understand this is of importance for the clinical management of patients and inclusion of patients into clinical trials. Objectives: Assess treatment regimens in SSc-ILD over time including initiation, switch and stop of ISTs and its impact on ILD progression and progression free survival. Methods: SSc patients registered in the EUSTAR database with presence of ILD on imaging, available FVC%, DLCO% predicted and ISTs at baseline and at 12 ± 3 months were included. We segregated treatment regimens into four periods based on patient’s first assessment: 1) ≤ 2006; 2) 2007-2011; 3) 2012-2016; 4) ≥ 2017 and assessed clinical characteristics and type of IST. Next, we evaluated the impact of ISTs across a 3-year follow-up period on the two outcomes, (I) progressive ILD events and (II) progression free survival. Absolute decline of FVC≥5% or DLCO≥10% over 12 ± 3 months was defined as a progressive event and progression free survival as survival in the absence of progression within 36 months. Next, we assessed the impact of ISTs on these two endpoints in patients with positive anti-topoisomerase I (ATA) to enrich for progressors and evaluated treatment changes after periods of progression. Non-parametric tests were used for multiple group comparisons. Results: In total, 1409 SSc–ILD patients were included with 236 (16.7%) in period 1; 558 (39.6%) in period 2; 338 (23.9%) in period 3 and 277 (19.7%) in period 4. The clinical characteristics were comparable across the periods (Table 1). The use of ISTs increased significantly from 13.6 % in period 1 to 57.4% in period 4 with a significant change of type of IST, and frequency of switches and combination in ISTs and time to treatment stop (Figure 1A). When assessing the impact of ISTs over the follow-up of mean 3 years (excluding period 1), we identified significantly fewer progressive events; with 115/540 (21.3%), 146/1061 (13.8%) and 160/1320 (12.1%) in period 2, 3 and 4, respectively (p <0.001). The treatment pattern after progression also differed among periods with increase in combination and switch in ISTs (Figure 1A). We identified improved progression free survival from period 2 to 3 and 4 with 46.3%, 64.9% and 55.0%; p = 0.007 (Figure 1B). In the ATA positive SSc-ILD patients subanalysis (136, 311, 185 and 154 in period 1-4), excluding period 1 we identified significantly fewer progressive events on IST with 73/318 (23.0%), 84/563 (14.9%) and 83/654 (12.7%) in period 2 compared to 3 and 4 (p = <0.001). Progression-free survival improved from 46.9%, to 67.1% and 57.6% in period 2, 3 and 4; p = 0.045 (Figure 1C).Table 1. Clinical and treatment features of SSc-ILD patients at first-time assessment across the 4 periods. Data are presented and numbers and (%). Conclusion: The majority of SSc-ILD patients are currently started on IST and over time there has been a significant change in treatments regimens with novel therapies being implemented in clinical practice especially in case of patients’ functional worsening. While this has reduced the number of observed progressive ILD events, progression-free survival is still unsatisfying with 45% surviving at 3 years. This represents a clear need for the development of novel treatment options and treatment regimens. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: Corrado Campochiaro Boehringer Ingelheim, Janssen, Novartis, Marie-Elise Truchetet AbbVie/Abbott, Boehringer-Ingelheim, Gilead, Merck/MSD, UCB, Madelon Vonk Boehringer Ingelheim, Corbus, Ferrer, Galapagos, Janssen, MSD, Giovanna Cuomo: None declared, Lidia P. Ananyeva: None declared, Eric Hachulla Bayer, CSL Behring, GlaxoSmithKlein(GSK), johnson&Johnson, Novartis, Otsuka, Roche-Chugai, sanofi-genzyme, Sobi, Vanessa Smith Boehringer Ingelheim, Support for travel, Galapagos, Janssen-Cilag, Ana Maria Gheorghiu AbbVie/Abbott, Boehringer-Ingelheim, Ewopharma, Sandoz, Radim Bečvář: None declared, Patricia Carreira: None declared, Nicolas Hunzelmann: None declared, Daniel Furst Amgen, Corbus, CSL Behring, Galapagos, Gilead, GSK, Horizon, Novartis, Pfizer, Roche, Vera Ortiz-Santamaria: None declared, Francesco Del Galdo AbbVie/Abbott, Arxx, AstraZeneca, Boehringer-Ingelheim, Capella, Chemomab, GlaxoSmithKlein(GSK), Janssen, Mitsubishi-Tanabe, Marco Matucci-Cerinic accelerong, actelion, bayer, biogen, Boehringer-Ingelheim, Chemomab, corbus, CSL Behring, Eli Lilly, galapagos, Inventiva, Janssen, Merck/MSD, Mitsubishi, Pfizer, regeneron, Roche, samsung, Anna-Maria Hoffmann-Vold Boehringer Ingelheim, Boehringer Ingelheim, Janssen, Medscape, Merck Sharp & Dohme, Novartis and Roche, ARXX, BMS, Boehringer Ingelheim, Genentech, Janssen, Medscape, Merck Sharp & Dohme and Roche, Boehringer Ingelheim, Janssen.Figure 1Panel A. ISTs and treatment regimens across the 4 periods. Panel B Progression-free survival of SSc-ILD patients across the 3 periods in the whole cohort, and Panel C in the enriched cohort.

To describe the characteristics of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), compare their presentation and evolution, and analyse possible complication predictors. Multicenter study. Data were retrieved from a hospital-based study of patients with a diagnosis or suspected diagnosis of sJIA or AOSD according to the responsible physician and followed-up for at least one year. Descriptive variables (classification criteria, clinical manifestations, complications, family, and personal history) were collected at disease onset and during follow-up. We present the clinical characteristics of 326 patients, 67% of whom had a diagnosis of sJIA and 33% of AOSD. Clinical manifestation frequencies were similar between the two groups, except for odynophagia, which was significantly more frequent in AOSD than in sJIA (78.4% vs. 25.5%; p < 0.0001). Among the complications, macrophage activation syndrome (MAS) was significantly more common in sJIA than in AOSD (24.4% vs. 9.5%; p = 0.002), to the extent that an sJIA diagnosis significantly increased the risk of MAS, together with serositis presence, and the need for biological therapy. Patients with sJIA and AOSD showed similar characteristics, supporting the idea that they are both part of Still's disease, but are expressed at different ages. Differences in manifestations and complications might be due to different management between diseases and immune response maturity.

Background Systemic sclerosis (SSc) is a heterogeneous disease with frequently associated interstitial lung disease (SSc-ILD). We aimed to determine the prognostic potential of phenotyping patients with SSc and SSc-ILD by inflammation and to describe disease trajectories stratified by inflammation and immunosuppressive treatment.Methods Patients from the European Scleroderma Trials and Research (EUSTAR) group cohort were allocated to persistent inflammatory, intermediate and non-inflammatory phenotypes if C-reactive protein (CRP) levels were >= 5 mg/L at >= 80%, at 20-80% and at <20% of visits, respectively. Cox regression models were used to analyse mortality risk and mixed effect models to describe trajectories of FVC and diffusing capacity for carbon monoxide (DLCO) %-predicted stratified by inflammation and immunosuppressive treatment.Results 2971 patients with SSc and 1171 patients with SSc-ILD had at least three CRP measurements available. Patients with SSc-ILD with a persistent inflammatory phenotype had a 6.7 times higher risk of mortality within 5 years compared with those with a persistent non-inflammatory phenotype (95% CI 3 to 15). In the inflammatory phenotype, FVC %-predicted was declining without (-1.11 (95% CI -2.14 to -0.08)/year), but stable with immunosuppressive treatment (-0.00 (95% CI -0.92 to 0.92)/year). In the non-inflammatory phenotype, patients with and without immunosuppressive treatment had a significant decline in FVC %-predicted, which was more pronounced in those with immunosuppressive treatment (-1.26 (95% CI -1.87 to -0.64) and -0.84 (95% CI -1.35 to -0.33)/year, respectively).Conclusions Phenotyping by persistent inflammation provides valuable prognostic information, independent of demographics, disease duration, cutaneous subtype, treatment and SSc-ILD severity. The findings from this study support early immunosuppressive treatment in patients with SSc-ILD with persistent inflammation.

Objective: To analyze the effectiveness and safety of intravenous immunoglobulin (IVIG) given in routine care to patients with systemic sclerosis (SSc). Methods: A retrospective multicenter observational study was conducted in SSc patients treated with IVIG. We collected data on epidemiological parameters and clinical outcomes. Firstly, we assessed changes in organ manifestations during IVIG treatment. Secondly, we analyzed the frequency of adverse effects. The following parameters were collected from baseline to the last follow-up: the patient's weight, modified Rodnan Skin Score (mRSS), modified manual muscle strength scale (MRC), laboratory test(creatine kinase(CK), hemoglobin and protein levels), The University of California Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 (UCLA GIT 2.0) questionnaire, pulmonary function tests, and echocardiography. Results: Data were collected on 78 patients (82% females; 59% with diffuse SSc). Inflammatory idiopathic myopathy was the most frequent concomitant overlap disease (41%). The time since Raynaud's phenomenon and SSc onset were 8.8 +/- 18 and 6.2 +/- 6.7 years respectively. The most frequent IVIG indication was myositis (38/ 78), followed by gastrointestinal (27/78) and cutaneous (17/78) involvement. The median number of cycles given were 5. 54, 53 and 9 patients have been treated previously with glucocorticoids, synthetic disease- modifying antirheumatic drugs and biologic therapies respectively. After IVIG use we found significant improvements in muscular involvement (MRC >= 3/5 92% IVIG, p = 0.001 and CK levels from 1149 +/- 2026 UI to 217 +/- 224 UI, p = 0.02), mRSS (15 +/- 12.4 to 13 +/- 12.5, p = 0.015) and improvement in total score of UCLA GIT 2.0 (p = 0.05). None Anti-RNA polymerase III patients showed an adequate response in gastrointestinal involvement (0/7) in comparison with other antibodies (0 vs. 25, p = 0,039). Cardiorespiratory involvement remained stable. A total of 12 adverse events were reported with only one withdrawn due to serious adverse effect. Conclusions: this study suggest that IVIG may improve myositis, gastrointestinal and skin involvement in SSc patients treated in routine care and seems to have a good safety profile.

PNL, number of escalations, number of de-escalations, time to first escalation, number of mild-to-moderate flares, number of severe flares and change in damage index) and cluster were examined using analysis of variance (ANOVA) and Tukeys HSD. Results A total of 210 HDAS periods (104 patients) were identified. Of the HDAS periods, patients were classified as treatment naïve (10%), HCQ inadequate response (20%), IS inadequate response (68%), and combination IS inadequate response (2%). The most commonly used IS was mycophenolate (23% of all HDAS periods). The trajectories were categorized into 3 final clusters: Cluster A (42/210) had more escalations than Cluster B (132/210) and Cluster C (36/210), see figure 1. There was no difference between clusters in the duration of time spent in HDAS, but a trend for higher cumulative PNL in Cluster A and they had significantly more and earlier escalations than Cluster B and C. Damage accrual appeared to be highest in Cluster C (the de-escalators) although not statistically significant. There was no difference between the distribution of the baseline treatment groups in each cluster. Conclusions Treatment trajectories can be described using clustering that examines treatment escalations and de-escalations. This pilot study showed that treatment trajectories appear to have an effect on clinical outcomes. Further studies are planned to explore the relationship of patient characteristics or physician treatment decisions have on these clusters. Funding Source(s): nil 219 EFFICACY AND SAFETY OF BELIMUMAB IN EXTENSION STUDIES OF 74 PATIENTS WITH ACTIVE SEROPOSITIVE SYSTEMIC LUPUS ERYTHEMATOSUS. RESULTS OF A SINGLE BRAZILIAN CENTER

Introduction Double-stranded-DNA antibodies (antiDNAds) are the most frequently detected serological markers in patients with Systemic Lupus Erythematosus (SLE). In clinical practice, it is usually determined by the ELISA technique with a specificity of 91–96%. There is another technique with a specificity of 98–100%, which is performed by immunofluorescence (IF) using Crithidia lucilae (CL) parasite. Purpose To determine anti-DNA by CL in patients with ANA and positive anti-DNAds by ELISA. To analyse whether there is a relationship between the patients who meet the 2019-ACR-EULAR classification criteria for SLE, and the positivity of anti-DNA by CL. Methods Bicentric retrospective observational study (Hospital Universitari Germans Trias i Pujol and Hospital General de Granollers). Patients with ANA ≥1/320 and DNA by ELISA >100IU/mL between 2018–2019 were collected. All underwent the IF CL test. The classification criteria for SLE ACR-EULAR 2019 were applied. Results Conclusions Anti-DNA CL test could be useful to discriminate patients with anti-DNA positive test by ELISA and low suspicion of SLE. In our series 95.7% of the patients with CL negative test did not meet SLE criteria. SLE in elderly behaves different than in younger patients. In our population: ⋄ Age over 65 years: anti-DNA CL test does not allow discrimination between those who meet the SLE criteria and those who don't. ⋄ Age under 65 years: anti-DNA CL test presents a high correlation with the clinical and analytical data of the patients. This test could be very useful in clinical practice to complement diagnostic criteria in SLE patients <65 years of age.