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Juvenile and Adult-onset Scleroderma Differ
A retrospecitve cohort comparision of juvenile (jSSc) and adult-onset (aSSc) systemic sclerosis (SSc) are both rare but present differently.
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British Society of Rheumatology Plans Revision of Systemic Sclerosis Guideline
British Society for Rheumatology (BSR) last published its guideline for systemic sclerosis (SSc) in 2016. Given the many advances in the field the BSR has outlined their plans to update the management of SSc.
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Big Time Vasculitis (3.10.2023)
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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A shame. Pts with #scleroderma are dying waiting for organ transplant- we should list and work them up for Tx earlier. PAH Pts May do worse @eular_org #EULAR2023 @RheumNow OP0241 https://t.co/8ZlHJmETGA
Janet Pope Janetbirdope ( View Tweet)
Should we use #immunesuppression EARLY in #scleroderma to prevent #ILD? Answer from a study comparing Y vs N immune suppression- there was no difference in FVC decline. Maybe not a long enough study, ? Chanelling bias scleroderma #lung #disease OP0239 @RheumNow #EULAR2023
Janet Pope Janetbirdope ( View Tweet)
Should immunosuppression be used to improve #PAH in #SSc added to PAH Rx (85%) Morbidity or mortality outcomes >700 pts - half had a DMARD after dx RheumNow #EULAR2023 OP0238 Targeted Rx improved esp RTX\TCZ findings V interesting results #GameChanger May change my Rx practice!
Janet Pope Janetbirdope ( View Tweet)
No new predictors for decline in #SSc lung function in ILD Progression of #scleroderma Lung disease is common - risks male, dcSSc, low FVC, Topo1 @RheumNow #EULAR2023, OP0236 @eular_org https://t.co/bTxTyLE8dZ
Janet Pope Janetbirdope ( View Tweet)
Risks for #ILD #SSc lung progression. FVC decline >5% is the gold standard but there are other criteria. OP0236 #EULAR2023 @RheumNow @eular_org #scleroderma Some criteria are stricter - larger PFT changes and/or worsening symptoms or fibrosis, so depends on def’n for % def’n https://t.co/TCuX2KY64w
Janet Pope Janetbirdope ( View Tweet)
#Scleroderma activity index #SCTC has an initial validated tool that is associated with death and organ damage with moderate correlations, so it has some degree of face validity @RheumNow #EULAR2023 @eular_org #OP0235 https://t.co/RDekhr7d2e
Janet Pope Janetbirdope ( View Tweet)
Scleroderma has an activity index that is associated w future morbidity & mortality. I wonder re the weightings of diff clinical manifestations as scleroderma renal crisis could weigh similarly to ex elevated CRP & a less severe manifestation. OP0235 @RheumNow #EULAR2023 https://t.co/KkimHBDVpg
Janet Pope Janetbirdope ( View Tweet)
#ClinicalPearl Updated #scleroderma Rx recommendations OP0234 New recommendations included ILD, early dcSSc 20 vs prior 16 guidelines. And they recommend ??! Won’t release yet due to importance of wording which will be finalized soon EULAR2023 @RheumNow @eular_org https://t.co/lES6pqkgeT
Janet Pope Janetbirdope ( View Tweet)
Early Rx with immunosuppressives doesn’t seem to prevent ILD in #scleroderma ? Chanelling bias as dcSSc get more ILD, esp if active disease but in this side it didn’t make a difference #EULAR2023 @RheumNow OP0239 https://t.co/xt0mtpOpYl
Janet Pope Janetbirdope ( View Tweet)
#EULAR2023 #POS0627 Researchers in Manchester identified several proteins associated with subclinical cardiovascular abnormalities on Cardiac MRI in Scleroderma using proteomics. Need validation. Could be useful for stratification and identify therapeutic targets @RheumNow https://t.co/55eNGhMu5f
Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
Is Interferon Type1 Impt in #scleroderma? #EULAR2023 OP#0114 Interferon accelerated skin fibrosis in mouse model- 61 pts 1/3 dcSSc, 2/3 lcSSc had markers of INF-1 in blood vessels &skin - more in #diffuse #SSc ?Rx opportunity w available drugs? RCT needed @RheumNow @eular_org https://t.co/vZWjSY8jKg
Janet Pope Janetbirdope ( View Tweet)
#EULAR2023 #OP0007 What are the recent trends of autoimmune disease (AID)? A database study (22m) in UK showed:
- 1 in 10 people had AID in the last 20yrs
- socioeconomic, seasonal & regional disparities cd influence pathogenesis
- CTD (SLE,pSS,SSc) tended to co-occur @RheumNow https://t.co/3DDPR2K5NC
Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
Juvenile and Adult-onset Scleroderma Differ
A retrospecitve cohort comparision of juvenile (jSSc) and adult-onset (aSSc) systemic sclerosis (SSc) are both rare but present differently.
https://t.co/57OrgaUNqj https://t.co/Y2puN18ux0
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Dr. John Cush RheumNow ( View Tweet)
Lit review of 2236 articles found only 28 w/ Post-COVID autoimmune Dx (64% F; age 51 yrs) - that included 11 (39) IIM (w/ 4 DM); 7 (25%) SLE; 4 (14%) anti-synthetase syndrome; 4 (14%) SSc; 1 lupus/MCTD overlap. Mean onset 23.7 d post COVID https://t.co/jtRaqIBGH1 https://t.co/WilcRFNAL9
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Dr. John Cush RheumNow ( View Tweet)
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
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Dr. John Cush RheumNow ( View Tweet)
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
Dr. John Cush RheumNow ( View Tweet)
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
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Dr. John Cush RheumNow ( View Tweet)
