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Simon Says “Don’t Do This” (1.27.2022)
Dr. Jack Cush reviews this week’s journal articles, focuses on studies that should not have been done and responds to viewer questions in Ask Cush Anything.
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JAK Inhibitor Potential in Systemic Sclerosis-Associated ILD
The occurrence of interstitial lung disease (ILD) in Systemic sclerosis portends serious morbid and mortal outcomes for those affected. This review examines the available clinical literature on the potential benefits and outcomes of JAK inhibitor use in SSc=ILD.
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NOVESA trial ziritaxestat (an autotaxin inhibitor) in 33 early diffuse systemic sclerosis pts. After 24 wks, ziritaxestat showed signif reduction in mRSS (–8.9 vs. –6.0 PBO; P=0.04). It was well tolerated & showed biologic effect w/ reduced LPA C18:2 https://t.co/GKC2TKOQhE https://t.co/AUaminOyiH
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Systemic Sclerosis cohort study of 506 SSc pts with saw 5.3 with Degos-like lesions (89% in fingers). Degos lesions more assoc w/ diffuseSSc & acro-osteolysis, digital ulcers, and calcinosis (but not renal crisis, ILD, or pulmonary HTN
https://t.co/eCumzZvEmO https://t.co/B41NLt4RYF
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Early ph II Pilot trial of 30 Systemic Sclerosis Pts using a new antifibrotic FT011 400mg vs 200mg v PBO x 12 wks showed CRISS score improved by 60% (400mg; (p=0.02 ) or 20% (200mg) at 12wks. An OL extension is planned for 9 mos @400 mg/d. https://t.co/8R51VLUbvX https://t.co/vc3JJKXfk3
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Subanalysis of Scleroderma Lung Study II (MMP vs CTX in SSc-ILD) showed pt reported GI reflux (~moderate) was independently associated with the change ILD and fibrosis over two years (but not so for esophageal dilation) https://t.co/5Gz2vfOock https://t.co/N2FsvNe24M
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Dr. John Cush RheumNow ( View Tweet)
AutoAb analysis of 140 systemic sclerosis shows low sensitivity (<33%), high specificity (>88%). Scl-70+ signif assoc w sine scleroderma, digital ulcers, Raynauds as 1st onset sign. SSc-ILD more common Scl-70 and PAH with CENP A/B Abs https://t.co/ZQksWVauMZ https://t.co/RKSWOItdxu
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Dr. John Cush RheumNow ( View Tweet)
AutoAb analysis of 140 systemic sclerosis shows low sensitivity (<33%), high specificity (>88%). Scl-70+ signif assoc w sine scleroderma, digital ulcers, Raynauds as 1st onset sign. SSc-ILD more common Scl-70 and PAH with CENP A/B Abs https://t.co/1zvkte4drJ https://t.co/FaSmshUvhY
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Dr. John Cush RheumNow ( View Tweet)
Remibrutinib in Sjogren's Syndrome
Dr. Michael Putman reports on abstract 1113 presented at ACR22. Abstract 1113: Remibrutinib (LOU064) in Sjögren’s Syndrome: Safety and Efficacy Results from a 24‑Week Placebo-controlled Proof-of-Concept Study
https://t.co/4UAxMq2z0X https://t.co/kzinfgDGZr
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Dr. John Cush RheumNow ( View Tweet)
Drugs causing Scleroderma (DASSc) - Anticancer rx made up 42% DASSc & 62.3% case reports. Included: taxane-based agents, bleomycin, vinblastine, imatinib, dacarbazine, pembrolizumab pemetrexed; also HRT romiplostim & eculizumab https://t.co/jPNIuaYdnJ https://t.co/efZG5kG2Yd
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2 open-label reports of JAK inhibitors in Systemic sclerosis (SSc) - 59 SSc pts: significant decr in mRSS in 88%. 28/29 w/ ILD had no progression. Overall responses better in treatment naïve SSc patients. 20% had side-effects. More studies needed https://t.co/zjxZtTFnlS https://t.co/XTwip2wv1n
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Rituximab Efficacy in Systemic Sclerosis
The DESIRES trial studied rituximab (RTX) in patients with systemic sclerosis (SSc) and showed clinically significant improvement in skin and lung outcomes after a subsequent 24-week open-label extension phase.
https://t.co/cqlVo104Y9 https://t.co/dYgBsj65BJ
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Dr. John Cush RheumNow ( View Tweet)
Nailfold Capillaroscopy doesnt have to be expensive: use handheld Carson Microbrite (~$10) 10x or 20 x handheld microscope (w/ KY gel) to evaluate #Raynauds #scleroderma https://t.co/aJ6K6JeVCp https://t.co/0aUBnqxhic https://t.co/54s66sWvyv #RWCS2022
#BestOf2022
Dr. John Cush RheumNow ( View Tweet)
3388 pts in J Hopkins Scleroderma study found 29% w/ calcinosis (13.5% these w/ heavy burden calcinosis/HBC). Calcinosis assoc w/ diffuse SSc, disease severity (PH, ILD, CVD, GI, renal crisis, myopathy)(OR 2.9- 6.9) & PM/Scl Abs (OR 17.31) https://t.co/VsRDg5Utik https://t.co/AMqHUveJ6e
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Dr. John Cush RheumNow ( View Tweet)
3388 pts in J Hopkins Scleroderma study found 29% w/ calcinosis (13.5% these w/ heavy burden calcinosis/HBC). Calcinosis assoc w/ diffuse SSc, disease severity (PH, ILD, CVD, GI, renal crisis, myopathy)(OR 2.9- 6.9) & PM/Scl Abs (OR 17.31) https://t.co/ksdPcH9W0O https://t.co/OvI9wytf5H
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Dr. John Cush RheumNow ( View Tweet)
Comparison of 227 Systemic Sclerosis pts w/ & without ILD shows SSc-ILD to have more muscle Dz [OR 2.55], LVDD [OR 2.36], PAH [OR 9.13], dcSSc [OR 2.86], PLR [OR 1.005], elevated KL-6 [OR 2.03], and anti-Scl-70 [OR 3.1]. https://t.co/Pzx9N5rPcF https://t.co/F8mBQ1u5iL
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Dr. John Cush RheumNow ( View Tweet)
#ACR22 Highlights. ILD in CTDs #rituximab = #cyclophoshamide at 6 mo for CTD ILD & ritux better on skin score in #scleroderma RECITAL RCT abst0003 but #pirfenidone added to #MMF not better than MMF alone in SLSIII RCT—ILD w #SSc abst#520 vs in SENCIS #nintedanib is + @RheumNow
Janet Pope Janetbirdope ( View Tweet)
☝️Treatment is the 3rd reason why we need to order a BL HRCT in pts with ILD; "We can't treat SSc-ILD if we are not able to detect SSc-ILD" - Dr. Elana Bernstein
#ACR @RheumNow #rheumatology https://t.co/ooZVSbt3Xf
sheila RHEUMarampa ( View Tweet)
Ab1672 #ACR22 SSc on Women's Health
100 pts - 75% lSSc
59% sexuality aff by SSc (vag dryness 67%, dig ulcers 37%, GI 37%, dyspnea 20%)
48 pts dx during repro ys - 73% never discuss contraception, 30% didn't reach family plan
22 pt - 43 pregn, 33 birth: C-sxn, low bth wt
@Rheumnow https://t.co/JRkyCVEXLB
Eric Dein ericdeinmd ( View Tweet)
Mihai et al. Immunosuppressive treatment in SSc assoc lower GI symptom score. @RheumNow #ACR22 Abstr#2174 https://t.co/Y1gyRe8ron https://t.co/tz9cIESzqo
Richard Conway RichardPAConway ( View Tweet)
