All News
A&R reviews the inflammasome in autoimmune diseases. Pattern recognition recpt induce inflammasome activation, pro-caspase-1 converts to caspase-1 convertin pro-IL-1β and pro-IL-18 active IL-1β and IL-18; seen in RA, SLE, gout, Sjogrens https://t.co/JGadiEWQWf
Links:
Dr. John Cush RheumNow ( View Tweet)
Analysis of 128 Pediatric Rheum pts (JIA, FMF, etc) analzyed for reactions to 8 different biologics during 32,494 infusions/injections - the frequency of anaphylaxis (from TCZ or RTX) was 3.9% in children. https://t.co/GzvcR0R7Bv
Links:
Dr. John Cush RheumNow ( View Tweet)
Are you an Expert in Still's disease, Periodic Fevers, FMF or Autoinflammatory Dz? Please let me know ASAP - I want to add you to the list! https://t.co/UwKkco8q1N
Are you an Expert in Dx & management of Still's disease, Periodic Fevers, FMF or Autoinflammatory Dz? If so -- please Direct Message me and tell me where you are and who you want to see (Im developing an EXPERT Referral list). OR TELL ME who is person to see in your town! https://t.co/BGBRbn4yko
Increasing Lung Disease in Systemic JIA
A single-center cohort analysis shows that lung disease is increasingly seen in children with systemic juvenile idiopathic arthritis (SJIA), especially those complicated by macrophage activation syndrome.
Prior to 2013, reports of pulmonary disease in SJIA were rare, but since there have been increasing reports of alveolar hypertension, interstitial lung disease and pulmonary alveolar proteinosis; often with a high mortality rate.
As such, since 2014, the Cincinnati Children’s Hospital Medical Center has seen an increase in lung disease and severe lung disease in SJIA.
RheumNow Podcast – Boiling Hot MAS (7.12.19)
Dr. Jack Cush reviews this week's news and journal reports featured on RheumNow.com. PsA v. non-PsA pregnancies, Still's disease, lupus, nutritional supplements and dietary interventions, use of cannabis, tanezumab in OA, CBD oils and more.
Read Article
Study of 182 hospitalized adult-onset Still's disease patients shows predictive factors for developing macrophage activation syndrome to include: 1. Splenomegaly (OR 5.745), 2. pericarditis (OR 6.5), and 3. ferritin >2000 (OR 4.7). https://t.co/oNsaCL6O92
Links:
Dr. John Cush RheumNow ( View Tweet)
A study examined whether EGCG suppresses the activation of the NLRP3 inflammasome, thereby effectively preventing gouty #inflammation. It was found that EGCG reduces inflammation in mouse foot tissue making it a promising treatment option for #gout https://t.co/X9O2mbJx6A https://t.co/wgUYz0BmeY
First Line IL-1 Inhibition in Systemic JIA
First-line treatment of systemic juvenile idiopathic arthritis (JIA) with anakinra (Kineret) was highly effective as monotherapy, minimizing the need for glucocorticoids, a single-center prospective study found.
Read Article
Key features in distinguishing Adult onset Stills disease from Fever of unknown origin: Arthralgia, rash, sore throat, neutrophilia, ferritin >5xULN, LDH based on comparison study of 69 AOSD & 87 FUO pts. 3 or more fever peaks daily makes AOSD less likely. https://t.co/49X4GGludn
Links:
Dr. John Cush RheumNow ( View Tweet)
Impressive Survival of Interleukin-1 Inhibitors in Systemic Juvenile Idiopathic Arthritis
The drug retention rate of interleukin-1 inhibitors (IL-1) used to treat systemic juvenile idiopathic arthritis (JIA) appears to be quite high according to a new study.
Read Article
QD Clinic - Febrile syndromes; How many days of fever? | from RheumNow - https://t.co/E9TJL3eZaD
Links:
Dr. John Cush RheumNow ( View Tweet)
RheumNow Podcast – TNFs and the Inflammasome (1.25.19)
Dr Jack Cush reviews the news from the past week at RheumNow.com.
Read Article
Using FMF-KI knockout mice (high TNF levels) researchers find TNF signaling drives pyrin & inflammasome activation; TNF is a critical modulator of pyrin of pyrin-inflammasomopathies like FMF. This is why TNF inhib sometimes work in inflammasomopathies. https://t.co/0EvuTI57tn
Links:
Dr. John Cush RheumNow ( View Tweet)
Pulmonary arterial hypertension is a very uncommon complication of Adult onset Stills disease - 4.8% of 41 AOSD pts developed PAH - often severe, with a mortality risk. https://t.co/XjyVArMNBl
Links:
Dr. John Cush RheumNow ( View Tweet)
Genetic Diagnosis for Previously Undiagnosed Disorders
The NEJM has reported the NIH's Undiagnosed Diseases Network (UDN) study results of genetically identifying new diseases from prospectively followed persons with undiagnosed disorders.
The UDN was formed in 2014 as a network of seven clinical sites, two sequencing cores, a coordinating center, central biorepository, a metabolomics core, and a model organisms screening center. It was established to apply a multidisciplinary model in the evaluation of the most challenging cases and to identify the biologic characteristics of newly discovered diseases.
Vasculitis may complicate autoinflammatory Dz - in FMF (IgA vasculitis, PAN), vasculitis may occur w/ CAPS, TRAPS, HIDS, DIRA, PAPA & Behcets syndromes. Vasculitis is part of DAD2 deficiency (STING-assoc vasculopathy w/ onset in infancy-SAVI (PAN-like) https://t.co/7MKo05pOa3
Links:
Dr. John Cush RheumNow ( View Tweet)
IL-6 for Adult Still's: A New Option?
Some clinical benefits were seen among patients with adult-onset Still's disease treated with tocilizumab (Actemra) in a small clinical trial, but the study's primary endpoint was not met, Japanese researchers reported.
Read Article
Long-Term Efficacy of Canakinumab in Systemic Juvenile Idiopathic Arthritis
Only inhibitors of IL-1 (canakinumab) and IL-6 (tocilizumab) are FDA approved for use in children with systemic onset juvenile idiopathic arthritis (sJIA). Now, long-term data from the extension studies from two phase III studies shows that canakinumab yields long-term improvements with reduced glucocorticoid dosing with no new safety findings with long-term use.
Read Article
Treatment Preferences in Still’s Disease
In July 2018, RheumNow launched a “Live Vote” survey of US and non-US rheumatologists that asked how they diagnose and treat systemic juvenile idiopathic arthritis (sJIA), also known as “Still’s disease”. It appears that many prefer to start therapy with an IL-1 inhibitor, after a course of steroids and MTX/DMARD. Yet, these findings suggest there are significant unmet needs in the diagnosis and management of sJIA patients.
Read Article