ACR Convergence 2024: Do Not Miss – Rare and autoinflammatory diseases

Guillermo Carvajal Alegria
Guillermo is an associate professor of rheumatology at the University of Tours and in the rheumatology department of the Tours University Hospital, France. His research interest are polymyalgia rheumatica and giant cell arteritis, ultrasonography and procedures for clinical research and administration of biopharmaceuticals for scientific research. He is a member of the board of directors of the French Rheumatology Society and of steering committee of the French College of Teaching Rheumatologists. He is a member of the EMEUNET Visibility & Global Affairs sub-committee.

Poster 0289 | Saturday, 16th November, 2024 10:30
Miscellaneous Rheumatic & Inflammatory Diseases Poster I
Presenting author: Lopez-Gutierrez, F (Spain)
Title: Effectiveness of Rituximab in IgG4 Related Disease

In this retrospective, multicentric, cohort the authors describe the characteristics of 54 patients with IgG4 related disease and treated with rituximab.  Complete clinical improvement was observed in 52.5% and 64.5% of the patients at 12 months and 24 months respectively. Rituximab 1g twice (two weeks apart) was the most frequent regimen.
Oral 0858 | Saturday, 16th November, 2024 03:00
Miscellaneous Rheumatic & Inflammatory Diseases I
Presenting author: Devauchelle, V (France)
Title: Baricitinib in Early Polymyalgia Rheumatica (BACHELOR Study)

In this proof-of-concept, randomized, controlled trial, the authors report the efficacy of baricitinib, a JAK inhibitor, in patients with early polymyalgia rheumatica and without any glucocorticoids. This study is a first step towards a more in-depth evaluation of the benefit-risk ratio of JAK inhibitors compared to glucocorticoids in polymyalgia rheumatica.
Oral 0856 | Saturday, 16th November, 2024 03:00
Miscellaneous Rheumatic & Inflammatory Diseases II
Presenting author: Brooks, L (USA)
Title: Comparative Effectiveness of Disease Modifying Antirheumatic Drugs for Cardiac Sarcoidosis

In this retrospective cohort study based on health records database, the risk of developing a cardiac complication (for patients with cardiac involvement at baseline) or having a cardiac involvement during follow-up (for patients without cardiac involvement at baseline) was the same in the methotrexate, the mycophenolate mofetil and the azathioprine groups. The tolerance of methotrexate was better with less infections.
Poster 2030 | Monday, 18th November, 2024 10:30
Miscellaneous Rheumatic & Inflammatory Diseases Poster III
Presenting author: Ruscitti, P (Italy)
Title: The Development of the EULAR Score for the Definition of Disease Activity in Adult-onset Still’s Disease; The “DAVID” Project

The DAVID score has been developed after a systematic literature review and the evaluation of the disease activity of 187 patients with adult-onset Still’s disease. The binary (yes/no) criteria selected in the score are fever, skin rash, arthritis, patient global assessment > 5cm, CRP > 10mg/l. Remission, low disease activity and active disease were defined using these criteria.
Oral 2660 | Tuesday, 19th November, 2024 11:00
Miscellaneous Rheumatic & Inflammatory Diseases II 
Presenting author: Montes, D (USA)
Title: Developing Predictive Models for the Diagnosis of VEXAS Syndrome

Using the clinical and laboratory from 144 patients with suspected VEXAS (77 positive for UBA1 mutation), the authors developed two models for diagnostic prediction. The second model includes only 5 factors (mean corpuscular volume, auricular chondritis, inflammatory skin rash, dysphonia, and pulmonary disease) and has a positive predictive value of 0.84.

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