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RheumNow Podcast – Systemic Sclerosis Pearls (2.19.2021)

Feb 18, 2021 9:57 pm

Dr. Jack Cush reviews nearly a dozen news and journal reports from the past 2 weeks on, including the ACR's new COVID-19 vaccine guidance for patients with rheumatic disease.

  1. Very high levels of IL-18 IL-18 (> 10 000 pg/ml) is associated with Macrophage Activation syndrome (with systemic JIA) as well as primary and secondary Hemophagocytic lymphohistiocytosis (HLH) 
  2. Italian IBD clinic with 90 IBD Vedolizumab Tx pts, 8%(11) developed severe enthesitis (4 Multifocal, 6 unifocal). Sxs after mean 46 wks, CRP=5.1 mg/dl, majority HLA-B27 negative; confirmed by PD/UA in 7 - 4/7 developed post-inflam entheseal calcifications
  3. Common mucocutaneous findings in 35 kids w/ MIS-C & COVID: 1) conjunctival injection (21), palmoplantar erythema (18), lip hyperemia (17), periorbital erythema/edema (7), strawberry tongue (8), & malar erythema (6)
  4. Study of Systemic Sclerosis patients from the EUSTAR database: shows of the 144 with anti-PM/Scl+ Abs, there was an increased risk of muscle involvement, ILD, calcinosis and dermatomyositis; muscle involvement had a more severe disease phenotype.
  5. Johns Hopkins & UCSF Scleroderma pts tested for Abs against telomerase & shelterin (TERF1); while rare in RA, myositis, controls; TERF1 Abs seen in 9% of SSc & assoc w/ severe lung disease (OR 2.4) & shorter telomere length. (TERF1 found in 7% of IPF)
  6. Dr Uma Mahadevan presents results of the 14 yr registry study of pregnancy in IBD (Crohn's &UC) 1431 pts on with no Rx, thiopurines, biologics or thiop+biologics, showed no rush of fetal malformation, but did have incr in spont abortions @RWCSmtg @UmaMaha…
  7. Vaccinated COVID-19 pts appear less contagious; report from Israel - among 650,000 received two-doses of Pfizer vaccine, 2,897 later tested positive for COVID-19, but nasal swab samples showed 4-fold less virus - hinting protection
  8. Congrats to ACR Task Force who published guidelines on RMD drug use w/ COVID vaccines. My views on their recs: 1. I agree, no change most DMARD, biologics 2. I disagree; I would NOT hold Orencia, I would NOT hold JAKi 3. I disagree: I would not hold MTX   Watch the ACR Town Hall
  9. Danish Cohort study of 5310 cutaneous LE or SLE pts between 1995-2014 (7-8 yrs F/U and 40.724 PYs) shows an overall increased cancer risk for CLE (SIR 1.35)and SLE (1.45), esp a 3-4 fold increased risk for NHL, hematological, pancreatic and lung cancers
  10. MIRROR Study: Pegloticase With Methotrexate in Uncontrolled Gout
  11. High Risk Cancer Profiling with Inflammatory Myositis
The author has received compensation as an advisor or consultant on this subject

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