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MDA-5 Antibodies and Juvenile Dermatomyositis

Jack Cush, MD

A multicentre, retrospective study examined MSA profiles (by immunoprecipitation) from 96 juvenile idiopathic inflammatory myopathies (JIIM) at nine pediatric rheumatology centres in Japan. MSA were found in 85 of 96 cases with over 90% of patients having one of the following three MSA

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Avacopan - Is it Really a Win for ANCA-Associated Vasculitis?

Jack Cush, MD

The NEJM has reported the results of a study showing that avacopan, a C5a receptor inhibitor, is as effective as steroids at treating patients with antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV).

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Update on Polymyalgia Rheumatica and Giant Cell Arteritis (Best of 2020)

Jack Cush, MD

JAMA has published a clinical update of recent studies on GCA. While PET/CT scanning are novel means of demonstrating activity, they have not changed the diagnostic approach to GCA or PMR and although there are no new proven treatment strategies for PMR beyond glucocorticoids, the FDA has approved tocilizumab as a first-line glucocorticoid-sparing agent. 

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Retrospective study of 162 ANCA-Assoc Vasculitis patients (63% GPA, 37% MPA) - risk of serious infx was 7.5 per 100Pt-Yrs & was higher with cyclophosphamide that RTX induction. Risks for SIE:plasmapheresis dialysis & high BVAS in 1st year https://t.co/kAlz9aGQup

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Biologic efficacy in 147 pts w/ eosinophilic granulomatosis with polyangiitis - compares RTX (63), mepolizumab (51) or omalizumab (31). Remission/partial rates were better in RTX (49%/24%) and MEPO (78%, 10%) vs OMA (15%/33%) https://t.co/ZsCFVnasIJ

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Retrospective review of 46 Rituximab treated ANCA-Assoc Vasculitis pts from 2 clinics (Mexico City, Lund) shows 24% had 18 severe infections (11.5/100 PY) - 72% within the 1st year. RTX was only independent SIE risk factor. 4 pts died, all from infection. https://t.co/8necVFP3AK

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Great full read NEJM review of Extrahepatic manifestations of Chronic HCV Infection; 1) mixed cryo vasculitis; 2)20-30% w/ HCV related Sicca (w/ SSA, SSB); 3) fatigue & FM; 4) AutoAbs: 70% RF+, <40% ANA+, 15% ACL Abs, 7% smooth muscle Abs https://t.co/YrIJbs8P8k

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Matched cohort study f142 Takayasu arteritis pts - incidence 0.8 per million/Yr. TAK had increased in All‐cause mortality (adj HR 1.88 ;1.29–2.76), ischemic heart dz, CVA, TIA, CVD, PVD - but not HTN, CHF or DM. Only ~50% were on statins or anti-Plt Rx https://t.co/rgNqkha6sq

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Population study from Southern Norway shows the incidence of giant cell arteritis is not increasing. Over 13yrs, from pop of 300k they identified 206 GCA pts (72%females). Incidence rate of 16.8/100k per year. Mortality was not increased https://t.co/UA16uIuwD1

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Avacopan - Is it Really a Win for ANCA-Associated Vasculitis? The NEJM has reported the results of a study showing that avacopan, a C5a receptor inhibitor, is as effective as steroids at treating patients with ANCA-AAV. https://t.co/vWmhEhX8Z3 https://t.co/omXpouPKoJ

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Among 335 AAV pts, 18% had venous thromboembolism (VTE). The incidence rate of AAV-related VTE was 2.4/100 PYs and was higher in the first 3 months post–AAV diagnosis (20.4/100 PY) and decreased over time. Age & BVAS were predictive of VTE.https://t.co/Arb2gk27B7

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Cutaneous manifestations of ANCA-assoc vasculitis studied in 1184 GPA, MPA, EGPA pts and found to assoc w/ more severe disease. Common were petechiae/purpura (15%); allergic Sxs such as pruritus, urticaria, were more common in EGPA https://t.co/cdoJPUnDiC

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Study of 147 Takayasu arteritis pts in UK (compared to 1,371 controls) shows all‐cause and CV mortality increased TAK (adj HR 1.88). While TAK had increased ischemic heart Dz, CVA, TIA, PVD, CVD; there was NO increase in HTN, CKD, CHF or diabetes. https://t.co/lanantCSXW

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Study of 52 pts w/ pericardial dz in GCA (44) & PMR (8) finds only 46% were >70 yrs. Manifest as pericardial effusion (85%), was initial Sx in 37%. 69% were symptomatic & classic cranial Sxs present in only 40%. Tamponade more likely w/ PMR https://t.co/B5IxBT9OO7

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Retrospective study of 171 GCA patients found that 32% had aoritis (by Sxs or AI murmur); hese pts were 6 fold more likely to have subsequent Aortic complications (aortic aneurysms or dissection) https://t.co/zRVzmnU7JH

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Study of 100 pts w/ Granulomatosis with polyangiitis finds 56% had elevated d-dimer levels and that d-dimer are associated with disease activity and inflammation rather than with the risk of venous thromboembolism. 23 had VTE not predicted by d-dimer https://t.co/zIYKXNl0oO

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#Bestof2020 Update on Polymyalgia Rheumatica and Giant Cell Arteritis https://t.co/8Kxts1X43H https://t.co/j06SFRxQ3m

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Plasmapheresis Indications : TTP, HUS, Cryoglobulinemia, Hyperviscosity, Myasthenia gravis, Goodpastures, Catastrophic APL Syn, Chr inflamm demyelinating polyneuropathy, Removal of drugs/toxins, Alveolar hemorrhage, ANCA vasculitis (questionable) https://t.co/8TBExIx0Kx

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#BestACR20Tweets RT @RichardPAConway : Really important study by @SattuiSEMD on high prevalence of frailty and its impact on QOL in PMR #ACR20 @rheumnow Abstr#422 https://t.co/QzDYGsqDeJ
Dr. John Cush RheumNow ( View Tweet)
Check out our latest article from Richard Conway ( @RichardPAConway) #ACR20 The tantalising prospect of glucocorticoid-free treatments for systemic vasculitis https://t.co/VG1ah3Nq5c https://t.co/js3l527VHg

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