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A multi-center, retrospective chart review of 82 Still's disease pts (63% female; mean Dx age 6.4 ± 4 yrs; F/U ~2.8 yrs). When analyzing Dz course, 34% were monophasic, 46% polyphasic (higher-than-expected), 19.5% persistent Dz. non-monophasic predicted by active 3 mos from Dx https://t.co/qtONtpeA22
B cell Targeted CAR-T Therapy for Autoimmune Diseases The review by Qi Li and colleagues provides a comprehensive examination of the progression, clinical applications, challenges, and future directions of B-cell-targeted CAR-T therapy across multiple autoimmune conditions. https://t.co/b1SmrOBbBU
Spectrum of skin Sxs in 518 pts w/ Stills dz from AutoInflammatory Dz Alliance (AIDA) Network Registry dedicated to Still’s disease includes Salmon evanescent rash (64%), macules (7.7%), urticaria (6%), persistent pruritic papules & plaques (PPPP) (4.8%) https://t.co/DW9NoaxNPP https://t.co/U5GrCutYT0
🧬 PAPA Syndrome: When Sterile Inflammation Mimics Infection (Pyogenic Arthritis, Pyoderma gangrenosum, Acne) •A rare monogenic autoinflammatory disease •Caused by gain-of-function mutations in PSTPIP1 🧠 Pathophysiology •Mutant PSTPIP1 → increased interaction with pyrin https://t.co/tJ4INdgfky
This week’s RheumNow Live pre-Learn spotlight: Vasculitis & Autoimmune disease. We’re diving into this important topic with expert-led talks from Drs. Nancy Olsen, Matthew Baker, and Clay Cockerell. To help you get the most out of their sessions, our speakers have shared key https://t.co/zLKY52Fi17
Rheumatologists can’t ignore hypereosinopilic syndrome anymore, not when it borders on our diseases and therapies. Great takeaways on subtypes and therapies from Giacomo Emmi in an elegant talk #EULAR2025 https://t.co/yzQVt1fLKW https://t.co/hrbEeppZhW
Hyperferritinemia is common in Hyperinflammatory syndromes but is a hallmark of Macrophage activation syndrome and Sepsis; and is a poor prognostic sign too. MAS is excessive activation of T-lymphocytes & macrophages leading to cytokine storm & multi-organ damage. https://t.co/PTJPhcvnzC
10 yr European retrospective study of haemophagocytic lymphohistiocytosis found 3345 deaths due HLH from 29 countries. Crude mortality rate = 5.3 per 10million PYs (range: 1.1-11.3). From 2011 to 2021, increasing from 3.9 to 6.6/10millPYs, varying by country, sex, age. https://t.co/58gCQ72saF
Treating Refractory Still's Disease A full read systematic review article examined treatment options for difficult to manage systemic juvenile idiopathic arthritis, or Still’s disease. It classified these refractory disease patients as those with either (1) persistent https://t.co/uDeIkIek1d
Organ Involvement with Behçet’s A review of patient data from the International AutoInflammatory Disease Alliance (AIDA) Network registry identifying those with mucocutaneous Behçet’s disease (BD) may progres to major organ involvement (MOI)m especially at later stages. https://t.co/jqM4IZuobe
Retrospective review of 113 AOSD pts, median age 45yrs, ferritin 4626 (IQR 1169–14,239). Advanced Rx (IVIG, IL-1, IL-6) required in 15 (13.3%), NOT predicted by ferritin levels, either measured continuously or by quartiles. Ferritin is not a reliable biomarker in AOSD. https://t.co/pHEPeIf9u2
Robinson et al has published that Epstein-Barr virus infects and reprograms autoreactive B cells to drive the systemic autoimmune response in SLE https://t.co/vjpvILrJOt https://t.co/HepyZrYW5L
20-40% of Autoimmune hepatitis (AIH) is associated with other autoimmune diseases - lead by autoimmune thyroiditis, type 1 DM, ulcerative colitis, Crohn disease, & celiac (but less so for RA, PBC, SLE, Sjögren syndrome, vitiligo). AIH Dx Abs include ANA, ASMA, LKM1. https://t.co/I3ZPHiRgqZ
Hospitalization in Stills: Is it "Still" an infection? Dr. Janet Pope discusses abstract 0163, "Trends in hospitalization and outcomes among patients admitted with Adult-onset Still’s disease in the United States: INSIGHTS FROM THE NATIONAL INPATIENT SAMPLE (2016-2022)," https://t.co/3Z0U9XN3nx
@CarolineDiorio @ChildrensPhila @RheumNow Indeed, there are a lot of familiar HLH/MAS friends in the post-CAR-T CRS mix... (indeed, rheum friends from many places) @CarolineDiorio @ChildrensPhila in the #ACR25 BCRC pre-meeting @RheumNow https://t.co/OzWu3MV1FA
I've always (very) simplistically considered post-CAR-T CRS and HLH/MAS in the same bucket, and there certainly are substantial similarities. It raises the obvious question - can we borrow therapies? @CarolineDiorio @ChildrensPhila in the #ACR25 BCRC pre-meeting @RheumNow https://t.co/2HkHxpl89u
