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Rare Diseases

RA Extra-articular Manifestations Declining

Sep 12, 2023

A Mayo Clinic population based cohort study shows the extraarticular manifestations of rheumatoid arthritis (ExRA) have declined with time; but the increased mortality risk of RA remains unchanged.

They defined their cohort as adult patients with incident RA (ACR Criteria positive)

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RheumNow Podcast – with Special Guest Dr. Janet Pope (9.8.2023)

Sep 08, 2023

This week, Dr. Jack Cush reviews the news with special guest, Dr. Janet Pope. They discuss "Not-non-Inferior", large scale genetic screening, Zoster risk w/ newer lupus drugs, tapering, RA referral problems and the TNR webinar session on "ORAL Surveillance Revisited".

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2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria

Aug 29, 2023

The American College of Rheumatology (ACR) and EULAR have combined efforts to establish an international multidisciplinary Steering Committee to develop classification criteria for the new antiphospholipid syndrome (APS) to be applied in observational studies and clinical trials.

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Cardiovascular Disease Precedes ANCA-associated Vasculitis

Aug 21, 2023

A nested case–control study from the Danish Nationwide Registries finds an increased risk of cardiovascular (CV) disease in the months preceding diagnosis of ANCA-associated vasculitis (AAV).

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Manifestations of Systemic Sclerosis Sine Scleroderma

Aug 21, 2023

Systemic sclerosis (SSc) sine scleroderma is a unique and rare subset of SSc defined by the absence of skin fibrosis. An analysis of the EUSTAR database suggests systemic disease can be a problem, including interstitial lung disease (>40%) and SSc renal crisis (almost 3%). 

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Pizza and Rheumatoid Arthritis (8.11.2023)

Aug 11, 2023

Dr. Jack Cush reviews the news and journal reports from the wonderful wide world of Rheumatology. This week:

  1. Swedish study of 325 #AAV pts saw CVA in 35 pts (8%); CVA incidence=11.3/1000 PYs - this was higher than gen population [SIR 1.85). AAV CVA risk factors included age

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Bifunctional B-cell Targeted Therapy in IgG4-related Disease

Aug 02, 2023

A novel B-cell depletion trial, using obexelimab (a bifunctional, monoclonal antibody that binds CD19 and Fc gamma receptor IIb), has shown efficacy and safety in patients with active IgG4-related disease.

Lancet Rheumatology has published the results of an open-label, single

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Tofacitinib in Polymyalgia Rheumatica (EAST PMR Study)

Aug 01, 2023

An open-label, uncontrolled pilot trial has shown that JAK signaling is involved in the pathogenesis of PMR and that tofacitinib is as effective as glucocorticoids (GC) in patients with polymyalgia rheumatica (PMR).

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EULAR/ACR Guidance on Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome

Jul 26, 2023

A EULAR/American College of Rheumatology task force has established evidence based, up-to-date guidance and expert opinion on the evaluation, management and monitoring of patients with Haemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS), with the primary intent to 

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ICYMI: LAVLI - A New Autoinflammatory Disorder

Jul 06, 2023

NIH researchers have have described a novel autoinflammatory disorder called "Lyn kinase-associated vasculopathy and liver fibrosis" (LAVLI), based on a mutation in the LYN gene (that encodes the Lyn kinase protein). They discovered that increased Lyn kinase activity promotes systemic

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Scleroderma – Thick and Thin (6.30.2023)

Jun 30, 2023

Dr. Jack Cush reviews the news, journal articles and regulatory decisions from the past week from This week focuses on parodoxical psoriasis, scleroderma outcomes and subsets, and alopecia areata therapy.

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Scary Outcomes with Systemic Sclerosis Sine Scleroderma

Jun 29, 2023

The hallmark of systemic sclerosis is scleroderma, but less than 10% of SSc patients have sine scleroderma (ssSSc). A EUSTAR database review compared the manifestations and outcomes of ssSSC to limited cutaneous SSc and diffuse cutaneous SSc.

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Predictors of Cardiovascular Events in ANCA-Associated Vasculitis

Jun 28, 2023

A large, retrospective, multinational study of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) shows they may be at risk for cardiovascular events (CVEs).

A total of 2286 AAV patients from the European Union, China, Turkey, Russia

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TitAIN Study: Secukinumab Efficacy in Giant Cell Arteritis

Jun 23, 2023

A phase 2 trial has demonstrated the efficacy of secukinumab, an anti-interleukin-17A monoclonal antibody, in patients with active giant cell arteritis (GCA).

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What is Colchicine Worth? (6.23.2023)

Jun 22, 2023

Dr. Jack Cush reviews the news and reports from the past week on This week a new colchicine FDA approval, rising rate of IgG4 related disease and what's the safest biologic?

  1. An RCT of 1136 newly COVID test positive pts compared long term outcomes if immediately

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How Often Do You Monitor MTX? (6.16.2023)

Jun 15, 2023

Dr. Jack Cush reviews the news and journal reports from this past week on, including new drug approval, a new SpA variant and new rules for methotrexate (MTX) monitoring and depression screening. 

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Higher Genetic Loads Linked to Worse Systemic Lupus

Jun 15, 2023

A Korean study of a large systemic lupus erythematosus (SLE) cohort shows by genomic analysis, that higher weighted genetic risk scores (wGRS) is associated with earlier SLE onset, higher anti–Sm antibody positivity, lupus nephritis and more diverse lupus manifestations.

Genotyping of

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Diagnostic Yield of Cranial and Large Vessel Imaging in GCA

Jun 13, 2023

Combined cranial and large vessel ultrasound and PET/CT provided excellent accuracy for the diagnosis of giant cell arteritis (GCA).

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EULAR 2023 – Day 2 Report

Jun 01, 2023

Day 2 at the international EULAR Congress meeting is always bigger and busier. There were sessions on JIA therapies, crystal arthritis, novel drugs for SLE and Sjogren's, early RA and preclinical RA interventions, IgG4 disease, osteoporosis and the growth and application of artificial

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AOSD: Young vs. Elderly Onset

Adult onset still's disease (AOSD) is an autoinflammatory condition characterized by fevers, arthritis, and rash. It is considered an orphan disease because of its low prevalence - it is reported anywhere between 16 to 40 per ten million. Traditionally, a bimodal distribution by age has been

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Belimumab in Lupus Nephritis

May 29, 2023

Belimumab is a B-lymphocyte stimulator (BLyS)-specific inhibitor that is FDA approved for the treatment of active lupus and lupus nephritis, based on a few pivotal trials; and now a metanalysis supports its use in active lupus nephritis.

A meta-analysis and systematic review of six

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How Age of Onset Affects Giant Cell Arteritis

MedPage Today
May 23, 2023

Giant cell arteritis (GCA) has a different phenotype when diagnosed late in life compared with earlier onset, a large Italian cohort study indicated.

Features of what might be called "senior GCA" include increased rates of cranial symptoms, a markedly higher risk for aortic aneurysm

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International Consensus on ANCA-Associated Vasculitis with Glomerulonephritis

May 22, 2023

Nephrology Dialysis Transplantation has published an overview of the management of anti-neutrophil cytoplasmic antibody associated vasculitis with glomerulonephritis based on several international guidelines, including those frorm the ACR 2021, EULAR 2022 and KDIGO 2021 (Kidney Disease:

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Allergic Drug-Induced Arthritis (5.19.2023)

May 19, 2023

Dr. Jack Cush reviews the news and journal reports from this past week on  This week we delve into some odd associations, repeat drug warnings and the consequences of antirheumatic therapies.

  1. Subanalysis of the tofacitinib ORAL Surveillance study shows the JAK

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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.