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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.
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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.
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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.
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RheumNow Podcast – TNFs and the Inflammasome (1.25.19)
Dr Jack Cush reviews the news from the past week at RheumNow.com.
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What temp is a fever? Great article from @WSJ that discusses our @feverprints app! https://t.co/Kixwp1JLB8 #rheum https://t.co/54BmZF5PhC
I often wonder why my FMF pts are almost all young... At Cerrahpaşa in Istanbul, 180/≈5000 (3.6%) are >40yo, attacks get less frequent/severe after 40 but more comorbidities to deal with. Onset >40yo possible but milder dx (table⬇️) @egeli_bugra #ACR19 ABST1251/1256 @RheumNow https://t.co/JedUmF7KlR
They're both anti-IL-1β agents, but failure of anakinra in adult-onset Still's Disease doesn't mean canakinumab might not work (and work quickly) - see row 7-10 below. Anyone else have that experience? Corrado @Giacomo97811458, San Raffaele Hosp, Milan #ACR19 ABST1245 @RheumNow https://t.co/2qQK08apnT
#ACR19 @rheumnow ABS 1237 OLT1177, oral NLRP3 inflammasome inhibitor- a potential drug for Rx of gout flares: ⬇️ systemic inflamm by ⬇️ WBC count & circulating IL-1β& IL-6 in all3 doses (2000mg, 1000mg or 300mg). ⬇️ ex vivo IL-1β & IL-6 production by cultured PBMCs days 3, 7& 14
Olga Petryna DrPetryna ( View Tweet)
The questions that Dr. Amanda Ombrello (@NIH) asks when evaluating patients with recurrent fevers: -Age of onset -Frequency -Duration -Regularity -Height of fever -Prodromal symptoms #ACR19 @rheumnow
Jonathan Hausmann MD hausmannMD ( View Tweet)
IL-1 blockade beneficial in MAS. Ferritin to ESR ratio useful for identifying these patients. Cytokines Storm Syndrome is one way of thinking of the spectrum of conditions like MAS, HLH, auto inflammatory disease which respond to IL-1 and IL-6 blockade. Cron R #ACR19 @RheumNow
Dr. Antoni Chan synovialjoints ( View Tweet)
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
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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
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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
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
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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
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
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Dr. John Cush RheumNow ( View Tweet)
QD Clinic - Febrile syndromes; How many days of fever? | from RheumNow - https://t.co/E9TJL3eZaD
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Dr. John Cush RheumNow ( View Tweet)
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
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Dr. John Cush RheumNow ( View Tweet)
