EULAR 2025 Highlights – Other Connective Tissue Diseases and Vasculitis

EFFICACY AND SAFETY OF EFGARTIGIMOD PH20 SC IN ADULT PARTICIPANTS WITH ACTIVE IDIOPATHIC INFLAMMATORY MYOPATHY: PHASE 2 RESULTS FROM THE ALKIVIA STUDY

Abstract format and assignment number: Oral presentation OP0002

Date: Wednesday, 11 June, 16:40 – 16:50

Presenting author: H. Chinoy (UK)

In the Phase 2 ALKIVIA trial, 89 patients with active IIM received subcutaneous efgartigimod PH20 or placebo plus standard care. Patients treated with efgartigimod presented significantly higher mean Total Improvement Score as compared to the placebo group. Moreover, time to achieving clinical response was shorter. Safety profile was comparable to placebo with the most common AE to be injection site erythema, rash, bruising, and diarrhoea. These results support FcRn inhibition as an effective and safe option therapeutic strategy in IIM.

LONG-TERM TREATMENT WITH LOW-DOSE MEPOLIZUMAB FOR EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: FOLLOW-UP RESULTS FROM THE EUROPEAN EGPA STUDY GROUP

Abstract format and assignment number: Oral presentation OP0170

Date: Thursday, 12 June, 11:10 – 11:20 

Presenting author: A. Bettiol (Italy)

In this multicentre European study of 209 EGPA patients, long-term treatment with mepolizumab 100 mg/4 weeks showed sustained effectiveness, with up to 46% of patients achieving complete response at 60 months. Some patients required dose escalation to 300 mg due to poor disease control. Therapy was overall safe, with a few serious adverse events reported over five years. These findings support the low dose long-term use of mepolizumab in EGPA, with dose adjustment as needed for optimal disease control.

THE CLINICAL PHENOTYPE OF ANTI-Th/To+ PATIENTS IN SYSTEMIC SCLEROSIS: A CASE-CONTROL STUDY WITHIN THE EUROPEAN SCLERODERMA TRIALS AND RESEARCH (EUSTAR) COHORT

Abstract format and assignment number: Oral presentation OP0217

Date: Thursday, 12 June, 11:00 – 11:10 

Presenting author: L. Moschetti (Italy)

Multicentre study of 306 SSc patients from 28 EUSTAR units. Anti-Th/To+ individuals showed predominantly limited cutaneous involvement, frequent ILD but rare PAH. Compared to matched controls (anti-Th/To- patients), patients with anti-Th/To antibodies presented less severe organ damage, lower complication-related mortality, and slightly better long-term survival. Overall, those antibodies are associated with favourable outcome.

RISK FACTORS FOR CANCER IN SYSTEMIC SCLEROSIS, IMPACT ON DISEASE PHENOTYPE AND PROGNOSIS, AND PROPOSAL OF MACHINE LEARNING-BASED PERSONALIZED SCREENING STRATEGIES: INSIGHTS FROM AN EUSTAR STUDY

Abstract format and assignment number: Oral presentation OP0220

Date: Thursday, 12 June, 11:30 – 11:40

Presenting author: A. Tonutti (Italy)

A case-control study of 588 SSc patients  from 27 EUSTAR units identified smoking, diffuse SS, elevated CRP, anti-POLR3, PM/Scl, and RNP antibodies as risk factors for synchronous and interceptable cancers, while digital ulcers a were protective factor. Cyclophosphamide was associated with increased subsequent cancer risk, contrary to MMF. Cancer significantly reduced survival, especially when interceptable or subsequent. Machine learning models achieved high accuracy in predicting interceptable cancers.

IDENTIFICATION OF FIVE CLINICAL SUBTYPES IN PATIENTS WITH ANTI-SYNTHETASE SYNDROME-ASSOCIATED INTERSTITIAL LUNG DISEASE USING CLUSTER ANALYSIS: MULTICENTER MYKO COHORT STUDY

Abstract format and assignment number: Oral presentation OP0320

Date: Friday, 13 June, 11:20 – 11:30 

Presenting author: C. Akiyama (Japan)

In this multicentre Japanese study of 118 patients with ASyS-associated ILD, cluster analysis identified 5 distinct subgroups – cluster 1: patients with severe muscle involvement, cluster 2: youngest patients with prominent skin involvement with severe ILD, cluster 3: patients with skin and microvascular abnormality, cluster 4: associated with malignancy and severe ILD and cluster 5 – the eldest, male-dominant group with significant inflammation and the most severe ILD.  This study is a step towards personalised treatment in ASyS.

EFFICACY AND SAFETY OF NERANDOMILAST IN PATIENTS WITH AUTOIMMUNE DISEASE–RELATED PROGRESSIVE PULMONARY FIBROSIS: SUBGROUP ANALYSIS OF THE FIBRONEER-ILD TRIAL

Abstract format and assignment number: Oral presentation LB0003

Date: Saturday, 14 June, 10:50 – 11:00

Presenting author: A. M. Hoffmann-Vold (Norway)

Nerandomilast is a new antifibrotic drug with immunomodulatory properties. In the FIBRONEER-ILD trial, nerandomilast slowed FVC decline in patients with autoimmune-related ILD, showing consistent benefit with the overall population of patients with progressive pulmonary fibrosis. Nerandomilast reduced the risk of first acute exacerbation of ILD, hospitalisation for respiratory cause, or death. Safety profile was comparable to the placebo group and diarrhoea was the most common adverse events.

Aleksandra Opinc-Rosiak

Aleksandra is a trainee in rheumatology and a research and teaching assistant at the Department of Rheumatology, Medical University of Lodz. Her main clinical and research interests include idiopathic inflammatory myopathies, CTD-ILD and autoantibodies. She is a member of Polish Rheumatology Society, iMyoS, EEACI.

Aleksandra is a member of EMEUNET Newsletter Sub-committee and a current Country Liaison for Poland.

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