Off-Label Use of JAK Inhibitors in Orphan Diseases and Other Conditions?
When should you consider off-label use of JAKi?
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Nailfold video-capillaroscopy (NVC) study of 76 dermatomyositis (DM) pts - 74% had anomalous NVC patterns (diminished density, abnl morphology). NVC abnl pts had more Raynaud's, and cluster 1 were more likely to develop ILD.https://t.co/OC7jNHdFMn https://t.co/cCCO70Xzoh
Dr. John Cush @RheumNow( View Tweet )
What Lies Beneath: Understanding Nailfold Capillaroscopy
Capillaroscopy is a non-invasive imaging technique used for the in vivo assessment of the microcirculation. kin capillaries at the nailfold are evaluated because the fingers are involved in pathological processes such as Raynaud’s phenomenon. Several abstracts and sessions on nailfold
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⏩Lecture by Prof Takeuchi on Sjogren Syndrome. Anti-centromere ab is seen in 10% of primary Sjogren syndrome. more likely to have sclerodactyly. https://t.co/mVry2OaNmq @IRACON24Blr https://t.co/SZlWQDpx8B
Links:
Dr. Dinesh Khanna @sclerodermaUM( View Tweet )
ICYMI: Precision medicine in Ssc ILD? Yes!
-Watch and wait approach can cause irreversible lung damage.
-⬆️CRP: biomarker of response (predicts mortality)
-⬆️KL-6: biomarker of severity (predicts progression of fibrosis)
-FAPI-PET: can eval disease activity vs response to ttx.… https://t.co/bTepwrsuNN https://t.co/e6xjtuquka
Links:
Adela Castro @AdelaCastro222( View Tweet )
Are we under-monitoring in scleroderma ILD?
Dr. Janet Pope discusses abstract 0678, Patients with Interstitial Lung Disease Due to Systemic Sclerosis or Rheumatoid Arthritis Need Monitoring More Frequently Than Annually, and abstract 0706, Single Center Prospective Cohort of… https://t.co/1y1VXGvqjp https://t.co/4ofh45cYwq
Dr. John Cush @RheumNow( View Tweet )
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 )
Recent Blogs
Will the results of two recent large scleroderma lung studies finally inform us on how to manage interstitial lung disease in systemic sclerosis?
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