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The #scleroderma-like pattern on NVC is observed in pts with #dermatomyositis and MCTD
It consists of a combination of giant capillaries, some microhemorrhages, severe capillary loss and angiogenesis
@RheumNow #ACR24 @rheumarhyme https://t.co/WO9BOYn4ir
sheila RHEUMarampa ( View Tweet)
#Scleroderma #molecular #signatures in #skin predict long term outcomes
👇worst with inflammatory signature
This may have clinical and trial applicability
Nice work!
Abst0711 #ACR24 @RheumNow @ACRheum https://t.co/3CFxu6bACu
Janet Pope Janetbirdope ( View Tweet)
#PAH in #SSc still has a poor #prognosis
👎
Esp #ILD with PAH - a double whammy
But also
group 1 PAH
And
Some are unclassifiable ▶️also bad px
Need
Earlier detection
More interventions
?prevention
Abst#0678 #ACR24 @RheumNow @ACRheum @sclerodermaUM https://t.co/AEQsTC1pzQ
Links:
Janet Pope Janetbirdope ( View Tweet)
Lung ultrasound for ILD in SSc/IIM would be great, no radiation and by the bedside - but a lot of skill required to interpret in reality.
Maybe AI/computer vision can come to the rescue?
Some nice models @StanfordRheum - will be watching this space!
#ACR24 ABST1965 @RheumNow https://t.co/0uXBSVSE4c
David Liew drdavidliew ( View Tweet)
3D management of Sscl from @delgaldoFrances
-Time
-Severity
-Type of organ involvement
*Identify phenotypes: fibrotic vs vascular damage.
*Raynaud’s red flags: +ANA, puffy fingers, abn capillaroscopy.
*Early ttnt of of digital ulcers delays onset of PAH.
*High IFN-I =marker of… https://t.co/ByfuV0MJds https://t.co/GdRVwuRf4E
Links:
Adela Castro AdelaCastro222 ( View Tweet)
Interesting comparative effectivness study, TOFA vs calcineurin inhibitors in MDA5-dermato
Expansion of prior publication @NEJM that used historical controls: https://t.co/AzA8ybCqYH
Encouraging data for JAKs in this disease; need proper trials
#ACR24 @RheumNow Abstr#1736 https://t.co/BHVMZLu6Ip
Links:
Mike Putman EBRheum ( View Tweet)
A#1693
Change in FVC @ W12 or 24 has progn value @ w52 in AI-ILD
Study SENSCIS (SSc) & INBUILD (SARD) trial PBO gps
SENSCIS: OR for ILD progression 1.9 @ w12, 2.5 @ w24
INBUILD: OR 2.2 @ w12 & w24
Early PFTs predict outcomes @ 1 yr, short trials may be feasible
#ACR24 @RheumNow https://t.co/8cICAeG0nr
Links:
Eric Dein ericdeinmd ( View Tweet)
How do you manage skin in Sscl?
-RNA pol III + high risk for skin progression.
-Limited+ low risk + stable Sscl no need for immunesuppresors.
-Look for EF in pts with generalized morphea.
-Ttnt of skin depending on other organ involvements.
-If no Raynaud’s/hands spared think of… https://t.co/HBjO5eQfjT https://t.co/oS1ZmkanGA
Links:
Adela Castro AdelaCastro222 ( View Tweet)
A#1692
Single cell RNA-seq identifies double-negative-2 (DN2) B-cell population ass w progressive SSc-ILD
IgD- CD27- cluster is highest risk
CD21 low B cells are distinct subset associated with sclerosis, pHTN
DN2 B cells as potential biomarkers for SSc-ILD
@RheumNow #ACR24 https://t.co/IbnOGS8C3t
Eric Dein ericdeinmd ( View Tweet)
A#1690 fibroblast activation protein inhibitor (FAPI)-PET in SSc ILD
Introduces FAPI quantification algorithm
FAPI uptake predicts greater rate of FVC decline. Trend for DLCO, not statistically signif
FAPI volume & intensity both predict accel lung decline
#ACR24 @RheumNow https://t.co/YA2ptLDaMy
Links:
Eric Dein ericdeinmd ( View Tweet)
A#1691
Biomarkers for Rx response SSc ILD?
92 SSc-ILD pts, 19 had progressive pulm fibrosis (PPF)
KL-6 - incr in PPF, decr in those w/o PPF
CXCL-4 decr across cohort - non-signif trend for more w/o PPF
Specifically in MMF: CRP, KL6, CXCL4 are predictive
#ACR24 @RheumNow https://t.co/9fG6atcLmK
Links:
Eric Dein ericdeinmd ( View Tweet)
#1689: race-neutral 🫁 function equations in SSc improve equity:
🔹 Race-specific equations underestimate disease severity in Black patients.
🔹 Switching to race-neutral reclassified 45% of Black patients to more severe, 16% of White patients to less severe
@RheumNow #acr24 https://t.co/BfK2PsCMX5
Caoilfhionn Connolly CaoilfhionnMD ( View Tweet)
171 SSc pts w/ hand Xrays and US found 20% w/ Joint involvement. Active Synovitis & tenosynovitis predicted by CRP>10mg/L (p = 0.013), fibrotic tenosynovitis on US (p = 0.005), anti-RNA polymerase III antibodies (p = 0.043) https://t.co/DZrM5aQpJ7 https://t.co/CkGP6H8J6x
Dr. John Cush RheumNow ( View Tweet)
How often should #scleroderma #ILD pts have #PFTs? 🤷♂️
Data from SENCIS RCT of SSc-ILD
25% worsened FVC by 6/12
Are we UNDER a ordering PFTs in an ILD #SSc population
👇
Because if worsening we would consider a change in background #immunesuppression
@RheumNow #ACR24 #678 https://t.co/a9ybO3FwDd
Janet Pope Janetbirdope ( View Tweet)
De Lorenzis et al. MMF in LcSSc. Target Trial Emulation. 1435 patients. Incidence vasodilator treatment escalation over 41 months, 1/100py in MMF vs 7.3/100py in controls. @RheumNow #ACR24 Abstr#0688 https://t.co/D3qTUHyIIc https://t.co/6azvaS0ElL
Links:
Richard Conway RichardPAConway ( View Tweet)
Diagnostic work up for sarcoidosis. Think of CVID as an important differential @RheumNow #ACR2024 #sarcoidosis https://t.co/DvJzkN3ZjY
Antoni Chan MD (Prof) synovialjoints ( View Tweet)
Pathophysiology of calcinosis. Antonia Valenzuela at Review Course @RheumNow #ACR24 https://t.co/gSJSa2lGXw
Antoni Chan MD (Prof) synovialjoints ( View Tweet)
Risk factors and crystal composition of in calcinosis. In SSc - calcinosis is assc with vasculopathy, dz duration #ACR24 #ACRReview https://t.co/LJvrdVG1jg
TheDaoIndex KDAO2011 ( View Tweet)
171 SSc pts w/ hand Xrays and US found 20% w/ Joint involvement. Active Synovitis & tenosynovitis predicted by CRP>10mg/L (p = 0.013), fibrotic tenosynovitis on US (p = 0.005), anti-RNA polymerase III antibodies (p = 0.043) https://t.co/OIornA9sAA https://t.co/yXyX5k2yqD
Dr. John Cush RheumNow ( View Tweet)
Scleroderma registry enrolled SSc pts w/o ILD. Incident ILD occurred in 199/969 (21%) pts over median 2.4yrs. Late onset SSc-ILD (>7yr from Raynauds) occurs 32% less. These pts were more White, w/ arthritis & RNA-polymerase III Abs, but have similar ILD Progression (vs… https://t.co/zdL4OPEPhi https://t.co/wl9XmsCHIE
Dr. John Cush RheumNow ( View Tweet)
