PLEX continues to be OUT for DAH in AAV Retrospective review of ~1.4k AAV-DAH pt 375 who received PLEX compared to those who did not PLEX tx patients: - higher odds of death - higher sepsis - increased LOS - increased hospitalization cost @RheumNow #ACR25 Abst 2517

MAINRITSEG: RTX vs. AZA for maintenance in EGPA RTX not superior in primary remission outcome RTX favored in secondary outcome of remission with pred <4mg daily Limitation: only ~50% pt with FFS 1+ @RheumNow #ACR25 Abst 1765 https://t.co/dh5ZWCT12T

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Great debate on GCA ultrasound vs biopsy. Wolfgang Schmidt making the case for GCA US with the rise of its use in guidelines @RheumNow #ACR25 https://t.co/ORxzzG8xnF

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Mimics of ANCA-associated vasculitis: How can we tell? With increased disease awareness and more regular, accessible testing for anti-neutrophil cytoplasmic antibodies (ANCA), ANCA-associated vasculitis (AAV) has become an increasingly recognized clinical entity in rheumatologic https://t.co/LYRk0rHlzx

#1725 Functional NOTCH4 variants contribute to vasculopathy & fibrosis in SSc, particularly in African-American patients NOTCH4 hyperactivation disrupts angiogenesis & drives EndoMT; inhibition (including with FDA-approved Nirogacestat) restores vascular repair @RheumNow #ACR25

PET and histologic discordance in aortitis Of 5 aortitis patients who had PET/CT prior to aortic surgery with path, only 1 showed concordance (neg PET, neg biopsy) 4/5 patients had inflammatory biopsy with neg PET Small # but PET is not perfect! @RheumNow #ACR25 Abst 1620

PMR patients need healthcare professionals who know a bit about their disease. Survey of US HCPs, amongst responders: - 68% were not aware/unsure about PMR - of those who were aware, knowledge was… not great (see below) PMR needs everyone’s respect #ACR25 ABST1617 @RheumNow https://t.co/SpyEnAW8lI

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ANCA status does not inform IL-5 agent response in EGPA patients Terrier et al. analyzed the OLE of MANDARA and compared ANCA+ and ANCA- subgroups: - similar rates of remission - similar rates of GC withdrawal - comparable relapse rates @RheumNow #ACR25 Abst 1594

#ACR25 Abstr#1608 10! 10! Across the board. 2-yr data from RCT + Open Label showed frequency of both airway and non-airway features of #EGPA improved with Benralizumab/Mepolizumab. Provides assurance to use more in real-world @RheumNow https://t.co/i78xXojGja

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56 pts w/tracheobronchial stenosis in GPA, highly frustrating disease manifestation Not surprising to see severe dx & prior intralesional tx assoc with incidence (ie necessity) of tracheal dilation Leflunomide protective? Have others seen this? #ACR @RheumNow Abstr#1596 https://t.co/djJXnAkxnv

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Case series of 5 patients with binutuzumab (CD20) in AAV Short story short; it worked. Of course it worked. Obviously it worked. Everyone knew it would work. Why aren't we doing more TRIALS with new/better B cell inhibitors in AAV? #ACR25 @RheumNow Abstr#1601 https://t.co/H6Zz3Tf3xo

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