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Scleroderma/Raynauds

28 biomarkers studied in 259 SSc-ILD, 179 SSc-NO ILD, 172 IPF & 30 healthy controls. Composite biomarker index (w/ SP-D [surfactant prot. D], Ca15-3 [MUC1], & ICAM-1) strongly correlated w/ SSc-ILD (adj OR 12.7); independent of age, sex, smoking, PFTs https://t.co/F0iKFqcZoY https://t.co/Ao25N1DFIx

Big Time Vasculitis (3.10.2023)

Mar 10, 2023

Dr. Jack Cush Reviews the News and Journal articles from the past week on RheumNow. This weeks highlights includes insights on scleroderma, myositis, vasculitis and answers the question - does aggressive biologic treatment of psoriasis prevent future psoriatic arthritis?




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Systemic sclerosis current therapy: - early dcSSc: MMF, CTX, RTX, TCZ - rapidly progressive dcSSc - ?Stem cell transpl? - SSc-ILD: MMF>CTX, possibly nintedanib, perfinidone - Raynauds w/ digital ulcers: CA channel block, PDE5 inhib, iloprost, Bosentan https://t.co/87giuQR4YZ https://t.co/z2TPlJnu5e
Long-Term Benefit of Rituximab in Systemic Sclerosis A small cohort trial of rituximab (RTX) in 29 systemic sclerosis patients showed significantly improved skin sclerosis and lung function after a follow-up of 96 weeks. https://t.co/f68wc6lQGH https://t.co/G4PKPnJ0LJ

Long-Term Benefit of Rituximab in Systemic Sclerosis

Mar 06, 2023

A small cohort trial of rituximab (RTX) in 29 systemic sclerosis patients showed significantly improved skin sclerosis and lung function after a follow-up of 96 weeks.

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Steroids assisting options - CTX, LEF or RTX @anisha_dua @RWCSmtg @RheumNow #RWCS23 https://t.co/gVw2tGKYNG https://t.co/ItIKB8MFTI

Half the Experts are Wrong (2.17.2023)

Feb 17, 2023

Dr. Jack Cush reviews the news and tries to stump the expert panel this week on the podcast.

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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
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
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
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

Simon Says “Don’t Do This” (1.27.2022)

Jan 27, 2023

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.




  1. "Dry Synovitis" exist in juvenile arthritis pts? Study of 12 DS pts, says its different from Poly #JIA; onset 6.1 yrs

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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
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
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
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
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
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
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

JAK Inhibitor Potential in Systemic Sclerosis-Associated ILD

Dec 15, 2022

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.



Overall the authors

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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
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
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
#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
☝️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
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