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A new score to quantify GCA inflammation?
The treatment paradigm for Giant Cell Arteritis (GCA) has been a binary approach to the presence or absence of vasculitis. When GCA is present, we institute high doses of glucocorticoids for treatment of the inflammatory process. This approach is not well individualized to the patient as we do not account for the degree of inflammation that may or may not be present in the disease. Improved markers to stratify the extent of inflammation can help to tailor a more personalized approach to treatment.
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JAKs and Other Drugs in PMR
This has been an interesting ACR meeting in terms of PMR updates. I would argue that we are still far too wedded to glucocorticoids only in the management of PMR. Yes, some patients will do fine with just glucocorticoids but we persist far too long with a glucocorticoid only strategy in others who clearly need an alternative as glucocorticoid adverse events multiply.
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ANCA Vasculitis "Eye": Can rituximab help?
Dr. Janet Pope discusses abstract 0726 at #ACR24
https://t.co/KzYcmjon8D https://t.co/KDIi1vJE4P
Dr. John Cush RheumNow ( View Tweet)
Hypoxia seems to exacerbate neutrophil activation by ANCA
- enhanced F-Actin polymerization
- enhanced NET formation
Could hypoxia predilection in certain tissues (kidney, lung) lead to the AAV phenotypic disease patterns?
@RheumNow #ACR24 https://t.co/KxaeMvkd2t
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Brian Jaros, MD Dr_Brian_MD ( View Tweet)
Hydralazine - known culprit of drug-induced AAV
How does hydral (H) AAV differ from primary (1) AAV?
💥 High dual positivity MPO/PR3
💥 DAH / pleuritis more common in H-AAV
💥 ENT, constitutional, nervous, ILD in 1-AAV
💥 More 6mo remission in H-AAV
@rheumnow #ACR24 Abst 2496
Brian Jaros, MD Dr_Brian_MD ( View Tweet)
SELECT-GCA suggests JAKis may be the new kid on the block
Clinicians treating giant cell arteritis (GCA) have long had to contend with a disappointingly limited selection of drugs from which to select.
https://t.co/vtJzwFZMBq https://t.co/dikMRwjIZY
Dr. John Cush RheumNow ( View Tweet)
Risk stratification of GCA pts: who might lose vision?
Cranial MRI with vessel wall enhancement used to calculate additive score... # of vessels affected
Higher scores a/w ocular GCA > non-ocular GCA > no GCA and decreased w/ tx
@RheumNow #ACRbest #ACR24 https://t.co/knDVrl84Qs
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Brian Jaros, MD Dr_Brian_MD ( View Tweet)
Continuing or Stopping Low Dose Glucocorticoids in GPA
Dr. Mike Putman reports about plenary session abstract 0774 (the TAPIR study) about how and when patients can stop steroids in GPA. #ACR24
https://t.co/OWqN0jmkc1 https://t.co/foOaVRtGiV
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Dr. John Cush RheumNow ( View Tweet)
Yang et al. Cranial vessel wall MRI in GCA. Quantitated with PEG score. PEG score ocular GCA>non-ocular GCA>non-GCA. Decreases with treatment. @RheumNow #ACR24 Abstr#2650 https://t.co/e26urOpb7V https://t.co/eekQbzmKYm
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Richard Conway RichardPAConway ( View Tweet)
A#2651
Vasculitis w VEXAS
81 men, median age 67 - 27% had evid of vasculitis, variable size vessel
22% SVV- 17/18 cutaneous, one peritubular capillaritis on renal bx
2.5% MVV (2 cutan), LVV (2 carotid thickening, 1 had +TA bx), ANCA+
@RheumNow #ACR24 https://t.co/puablIBLCP
Eric Dein ericdeinmd ( View Tweet)
Sullivan et al. Vasculitis in VEXAS. 81 patients. 27% vasculitis. Cutaneous SVV 20%, AAV 2.5%, cutaneous MVV 2.5%, LVV 2.5%. @RheumNow #ACR24 Abstr#2651 https://t.co/AV3RcuSKpV https://t.co/xrax025TzP
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Richard Conway RichardPAConway ( View Tweet)
A#2648
PET for GCA
Delayed imaging at 180 min improve dx performance for pts on GCs
Optimal PET w/in 3 days of GC, most patients unable to do w/in that window
Delay has sensitivity 92% even on prednisone - may be good tool for pts unable to get early PET/CT
#ACR24 @RheumNow https://t.co/kxpNfRqkGN
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Eric Dein ericdeinmd ( View Tweet)
A#2650
MRI can show extent of vessel wall inflamm to guide severity of GCA
Brain & orbital MRI performed
Assessed 7 arteries bilaterally -> CAMRIS-GCA MRI score (0-10)
33 GCA (17 ocular), 41 not GCA
CAMRIS-GCA ass w/ ocular GCA, higher severity. Improved w Rx
@RheumNow #ACR24 https://t.co/19XCuZJXqF
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Eric Dein ericdeinmd ( View Tweet)
Disseminated acanthamoeba : pt with modular painful skin lesions on his palms and scalp— initially dx’d with a cutaneous vasculitis- he got worse with immunosuppression. Imaging and histology shown #ThievesMarket #ACR24 @RheumNow https://t.co/FjJ0Sk0Kxt
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TheDaoIndex KDAO2011 ( View Tweet)
A systematic review & meta-analysis by Dr. JBCorrea et al show that the ff immune-mediated dses are assocd w/⬆️ ocurrence risk after covid19 infection 👇
Behcets
SpA
SS
SLE
PMR
Pso
RA
Sjogrens
T1DM
Vasculitis
IBD
Ask prior covid infexn in pts history
@RheumNow #ACR24 abs2614 https://t.co/TIEglPrmvL
sheila RHEUMarampa ( View Tweet)
RIP to Glucocorticoids in GPA
For many years glucocorticoids were the mainstay of our treatment of granulomatosis with polyangiitis (GPA).
https://t.co/WqpO6ACqtu https://t.co/vb8qx3uHA0
Dr. John Cush RheumNow ( View Tweet)
Xia et al. Upadacitinib in refractory Behcets. Open label, 8 patients. Follow-up 4-14 months. Lower disease activity 2.5 vs 1.5. Lower pred, 11.9 vs 8.1. @RheumNow #ACR24 Abstr#2515 https://t.co/ZXsJkNRlTm https://t.co/S8LhpqZ8X3
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Richard Conway RichardPAConway ( View Tweet)
Does vasculitis flare with checkpoint inhibitors?
@MDAndersonNews experience:
17 stable pts
4 pts flared - all able to be controlled
(i.e. 13 didn't flare)
Encouragement that stable vasculitis pts that need ICI should get them!
#ACR24 ABST1986 @RheumNow https://t.co/jO4w3HWBMd
David Liew drdavidliew ( View Tweet)
Will be covering some TAK pearls and pitfalls from today’s session!
Renal dz stratified by location of vessel inflammation:
- TAK = proximal (off aorta)
- FMD = mid-section, beading
- PAN = distal arterial branches
@RheumNow #ACR24 https://t.co/zu6qJ6DXcF
Brian Jaros, MD Dr_Brian_MD ( View Tweet)
Imaging in TAK Diagnosis
@KQuinnRheum @RheumNow
⭐ Take homes ⭐
👉location of renal artery stenosis (RAS) key to making Dx with proximal RAS in TAK
👉FDG PET -focal uptake in TAK vs diffuse in GCA
👉MRI good surrogate for disease activity where PET unavailable
#ACR24 https://t.co/UWxTAZScIt
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Patricia Harkins DrTrishHarkins ( View Tweet)
