#EULAR2022
Contrasts in pathomechanisms and treatment targets between adult and paediatric inflammatory myositis
👁🗨 Awesome class on IIM pathogenesis
👁🗨The MSA and Phenotype Slide is 👌🏻👌🏻
#EULAR2022
Contrasts in pathomechanisms and treatment targets between adult and paediatric inflammatory myositis
👁🗨Summary of Mechanisms
👁🗨DM/JDM👉🏻 CD4 and TH17 mediated
👁🗨 Both Inflammatory and non inflammatory factors contribute
#EULAR2022
👁🗨 Type I,II IFN sig
👉🏻 DM/JDM>>ASS/PM>IBM>IMNM
👉🏻 Mi2/TIF/MDA5>>Jo1>>SRP/HMCGR
👁🗨⬆️INF levels 👉🏻⬆️ disease activity
👁🗨pDC and mDC mediated
👁🗨Mediate Muscle atrophy(perifasicular) and Capillary loss
#EULAR2022
Contrasts in pathomechanisms and treatment targets between adult and paediatric inflammatory myositis
👁🗨NETs & LDG⬆️ in Blood, Muscle, Lung & Skin
👁🗨MDA5 ⬆️NETs>>MJ,TIF,Jo1
👁🗨 Vasculopathy JDM>>>DM
👁🗨 Muscle damage in IIM us Multifactorial
👁🗨 DM 👉🏻 ⬆️INF, TNF, NF-KB
👁🗨IMNM👉🏻 ⬆️APR and ⬇️ Lymphocyte response
👁🗨ASS👉🏻 ⬆️APR, RNA binding ⬇️Muscle func, ubiquitination
#EULAR2022
👁🗨 Calcinosis JDM>>DM
👁🗨 CD3+ T Cells and Macrophages have a role
👁🗨 NETs engulf Ca crystals and Ca crystals cause NETOSIS
👁🗨Mitochondrial calcification
👁🗨 ⬆️ SIBLING protein expression
#EULAR2022
Contrasts in pathomechanisms and treatment targets between adult and paediatric inflammatory myositis
Summary Slides
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#ACR22#ACR2022
12S129. Purple Peripheries: Vasculopathy VS Vasculitis
Philip Seo is awesome as always
The most common cause is Cardiovascular followed by Rheumatic
More commonly vasculopathy than Vasculitis
#ACR22#ACR2022
12S129. Purple Peripheries: Vasculopathy VS Vasculitis
Algorithm for Digital ischemia in Rheum
Mx for Gangrene in SSc
#ACR22#ACR2022
12S129. Purple Peripheries: Vasculopathy VS Vasculitis
Large and Small vessel rarely cause Digital gangrene
#ACR2022#ACR22
12S130. Rheumatology Secrets and Pearls
Dr John Stone, at his best
🎯RA may present with asymmetric arthritis
🎯AAV might present with migratory oligoarthritis, which is steroid responsive
🎯Only a quarter of RA patients go into remission during pregnancy
#ACR2022#ACR22
12S130. Rheumatology Secrets and Pearls
🎯Following trends is more important in pregnancy that stat values (ESR/Complements)
🎯Proteinuria⬆️ creat ⬇️ due to ⬆️ GFR and maternal Blood volume in pregnancy
#ACR2022#ACR22
12S130. Rheumatology Secrets and Pearls
🎯Puffy fingers may be the first sign of SSc in RNApolIII SSc and No Raynaud's - May present with SRC
🎯ACEi are best kept reserved for SRC
#ACR2022#ACR22
12S130. Rheumatology Secrets and Pearls
Jason Kolfenbach, with his wonderful pearls 1. Vasculitis Pearls
🦪For Steroids ➡️ Less is more
🦪Be very careful in stopping Rx in AAV pts
🦪Don't give up on PLEX in suspected AAV Anti-GBM may have ANCA positivity.
#ACR2022#ACR22
12S130. Rheumatology Secrets and Pearls
🦪Not all Skin thickening = SSc
🦪Lipodermatosclerosis (with inverse 🍾 sign)
🦪Thyroid disease important mimics
🦪PPI can cause diarrhoea
🦪FVC/DLCO ratio can help diagnose ILD/PAH
#ACR2022#ACR22
12S130. Rheumatology Secrets and Pearls
🦪30% SLE pts have APS
🦪DLE uncommonly progresses to SLE
🦪PPIs, NSAIDs, Anti-HTN meds commonly cause SCLE
#ACR2022#ACR22
12S102. Year in Review 👌🏻
1st to be discussed was the Gloria Trial
Glucocorticoids effective but safety an issue
#ACR2022#ACR22
12S102. Year in Review 👌🏻
Next to the most discussed study of the year
Oral surveillance trial
The CV risk is higher in those with ASCVD - NNH 16😳
#ACR2022#ACR22
12S102. Year in Review 👌🏻
Next to the Proderm Study
Cost, thromboembolism a concern in the first positive phase 3 RCT in myositis
#EULAR2022
What is New (WIN)
IgG4
🔆IgG4 is a multi-organ Disease
🔆usually >50
🔆M:F - 3:1
🔆CD4+ cytotoxic cell implicated
🔆 Classification criteria known
#EULAR2022
What is New (WIN)
IgG4
🔆➕ve correlation with smoking (IgG4 retroperitoneal fibrosis)
🔆 blue Collar Jobs have ⬆️IgG4 (pancreato biliary)
🔆d/t toxic exposures 👉🏻 asbestosis, mineral dust
#EULAR2022
What is New (WIN)
IgG4
🔆Role of AutoAb suspected
🔆Anti-IL1RA👉🏻Mediates Inflammation and fibrosis
🔆Anti-Annexin A11👉🏻 damage biliary bicarbonate umbrella