RWCS Highlights - Day 4 Save

As another year at RWCS in Maui wrapped up, I’m so thankful I had the opportunity to participate remotely.  Thank you to Drs. Kavanaugh and Martin for providing a needed reprieve from the day to day grind in order learn from some of the best! Here are my day 4 #RWCS2021 pearls.

Novel Therapies Year in Review (RA, SLE, OA) with Alvin Wells, MD, PhD and Roy Fleischmann, MD

• Voclosporin, modified tacrolimus = no dose adjustment, improved lipid profiling with reduction in DM, no impact on MPA (mycophenolic acid) when used in combo (auriniapharma.com)
• ADVOCATE trial: Avacopan + CTS or RTX: noninferior to GC at week 26 and superior in sustained remission at week 52. Study also showed significant increase in eGFR in renal disease patient with less CG-related toxicity. A draw back will be price compared to steroids. (Merkel P, et al. EULAR2020, OP0011)

Novel concepts in Pediatric Rheumatology with Susan Shenoi, MD

• PFAPA: (Soriano A et al. Frontiers in Immunology 2020) stands for:
  • P-eriodic  F-ever  A-phthous ulcers  P-haryngitis and  A-denitis  
  • Usually 1-5 years of age, feversevery 4-6 weeks lasting 1-3 days
  • Resolves by age 6, plus/minus 2 years after onset
  • Tends to cluster in families
  • Treated with prednisone, cimetidine, and tonsillectomy.
  • PFAPA in adults: Cantarini’s criteria:
    • over 16 yo after exclusion of infection, autoimmune, or neoplastic dz
    • Recurrent Fever accompanied by a)erythematous pharyngitis and/or cervical lymphadenitis
    • increased inflammatory markers during attacks
    • symptom-free intervals (Cantarini L et al. Frontiers in Immunology 2017)
  • PFAPA may be on Behcet’s spectrum (Manthiram K et al. PNAS. June 2020)
  • PFAPA associated with increased IL12p70 PFAPA monocytes thus, possible treatments: Apremilast – decreases IL-12p&) and INF-r in monocytes and ustekinumab – anti-IL-12/23 (Manthiram K et al PNAS June 2020)
• VEXAS: (Beck D et al. NEJM 2020) stands for:
  • V-acuoles   E-1 ubiquitin activating enzyme  X-linked  A-utoinflammatory  S-omatic mutation
  • Ferrada M et al, ACR 2020 #0509 showed some Relapsing Polychondritis patients met VEXAS criteria. Certain risk factors of RP nose/ears, Males > 50 yo, MCV >100, plts < 200 were 100% sensitive and 96% specific for disease
• Theres a reason for these febrile disorders: Ancient FMF mutations in pyrin that led to increased IL-1Beta gave a survival advantage during the Plague (Yesenia Pestis)

TeleRheumatology Update: Post-COVID, Pre-?? Alvin Wells, MD, PhD

• Check out https://www.imlcc.org/. One application, one medical license for 28 states. This can improve access for our patients.