Autoinflammatory

Blau syndrome -a rare autoinflammatory Dz w/ triad of granulomatous dermatitis, arthritis, & uveitis (may cause blindness); from a NOD2 gain-of-function mutations. Onset age 2-4 yrs. Early biologics (esp TNFi) may prevent joint complications; but metanalysis shows no preferred https://t.co/IIJbJ7x2WB
Dr. John Cush @RheumNow( View Tweet )
A retrospective VEXAS cohort study of 59 pts receiving 71 targeted Rx - 98%male, mean 71 yrs & 46% had myelodysplastic syndr. Treatments included tocilizumab(19), anakinra (13), azacitidine(13), baricitinib (11), & pred only (10). Best responses w/ azacitidine & tocilizumab https://t.co/jqDmKinPDt
Dr. John Cush @RheumNow( View Tweet )
A retrospective VEXAS cohort study of 59 pts receiving 71 targeted Rx - 98%male, mean 71 yrs & 46% had myelodysplastic syndr. Treatments included tocilizumab(19), anakinra (13), azacitidine(13), baricitinib (11), & pred only (10). Best responses w/ azacitidine & tocilizumab https://t.co/fBdfrrZt1M
Dr. John Cush @RheumNow( View Tweet )
Neutrophil alkaline phosphatase (NAP) elevated in active AOSD. 141 AOSD compared to Bcell lymphoma, SLE, aPL & controls. NAP pos. correl w/ Systemic Dz activity score (R=0.71), & 0.59 for CRP/ANC/ferritin. NAP scores higher in pre-MAS, but lower w/ MAS (w/lower WBC). NAP responds https://t.co/YbYPaJUEbz
Dr. John Cush @RheumNow( View Tweet )
Emerging therapies targeting the NLRP3-inflammasome (active in Autoinflammatory Dz, Gout, neurodegenerative Dz/Alzheimers). Currently no FDA approved drugs, but many in trials. eg, Oxidized DNA (DAMP) activates NLRP3 & may be blocked by repurposed glycosylase inhibitors to reduce https://t.co/CJv50LVKhy
Dr. John Cush @RheumNow( View Tweet )
A brief history of familial Mediterranean fever - FMF is most common periodic fever syndr. an inherited polyserositis mainly affects Arabs, Armenians, Jews, & Turks. Hx may date back to 1802 & Heberden. Osler described 11 cases in 1895. 1st clear report 1945 by Siegal 10 pts. https://t.co/m20uO3bOoj
Dr. John Cush @RheumNow( View Tweet )
A brief history of familial Mediterranean fever - FMF is most common periodic fever syndr. an inherited polyserositis mainly affects Arabs, Armenians, Jews, & Turks. Hx may date back to 1802 & Heberden. Osler described 11 cases in 1895. 1st clear report 1945 by Siegal 10 pts. https://t.co/61WOxt9fPy
Dr. John Cush @RheumNow( View Tweet )
2024 Management of Familial Mediterranean Fever Recommendations A EULAR/PReS combined task force has developed recommendations for the management of Familial Mediterranean fever (FMF), the most common monogenic autoinflammatory disease worldwide. These evidence-based https://t.co/jZxCdXrDi6
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Neglected adult PFAPA syndrome case ub 69 yoF mis-Dx as pharyngitis or lymphadenitis unresponsive to Abx. Could've been Rx w/ daily colchicine. (PFAPA = Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis) https://t.co/0Nx5uFlwSx https://t.co/ZAUILEsH8E

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Dr. John Cush @RheumNow( View Tweet )
Neglected adult PFAPA syndrome case ub 69 yoF mis-Dx as pharyngitis or lymphadenitis unresponsive to Abx. Could've been Rx w/ daily colchicine. (PFAPA = Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis) https://t.co/0Nx5uFlwSx https://t.co/5pv6qGI8IE

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Dr. John Cush @RheumNow( View Tweet )
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2024 Management of Familial Mediterranean Fever Recommendations

Jack Cush, MD

A EULAR/PReS combined task force has developed recommendations for the management of Familial Mediterranean fever (FMF), the most common monogenic autoinflammatory disease worldwide. These evidence-based recommendations update the 2016 recommendations developed for rheumatologists and other HCPs

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Stills,continuum

Unmet Needs in Still’s disease

Jack Cush, MD

In February 2023, an expert consensus panel of pediatric and adult rheumatologists met to address challenges in Still’s disease (SD), including systemic juvenile idiopathic arthritis and adult-onset SD. They addressed four main topics: (1) early recognition and diagnosis of SD; (2) pathogenetic

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Just published, a review of Inflammasomopathies: mechanisms and disease signatures - these are Gain-of-function mutations in inflammasome receptors leading to chronic inflammatory conditions. https://t.co/kDebFdXlx9 https://t.co/NeJrgI9eRV

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Dr. John Cush @RheumNow( View Tweet )
Bullhead Sign in #SAPHO Syndrome ▶️Synovitis, ▶️Acne ▶️Pustulosis ▶️Hyperostosis ▶️Osteitis 👉🏽https://t.co/ownI4Q543w via @MayoProceedings https://t.co/FXlTtmsDDX

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Dr Ai Lyn Tan @DrAiLynTan( View Tweet )
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Osteoarthritis Risky Business (4.18.2025)

Jack Cush, MD

Dr. Jack Cush reviews the news and Journal reports from this week on RheumNow.com.  Osteoarthritis patients have unique risks and synovial fluid WBC numbers can tell you when to worry about septic arthritis in gout and pseudogout patients.

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Patient info on Sweets Syndrome: acute febrile neutrophilic dermatosis https://t.co/0q93q2NVc9 https://t.co/cE3LA8LaRQ

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1st Line Biologics vs. csDMARDs in Adult Still's Disease A German multicentre, retrospective study assessed the first-line efficacy of biologics and conventional synthetic DMARD therapy in patients with adult-onset Still's disease (AOSD) and found biologic agents were https://t.co/NaxACbmyxl
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An Epidural Letdown (4.4.2025)

Jack Cush, MD

Dr. Jack Cush reviews the news and journal reports from this past week on RheumNow.com.

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1st Line Biologics vs. csDMARDs in Adult Still's Disease A German multicentre, retrospective study assessed the first-line efficacy of biologics and conventional synthetic DMARD therapy in patients with adult-onset Still's disease (AOSD) and found biologic agents were https://t.co/EeSuBiYx5Q
Dr. John Cush @RheumNow( View Tweet )
Stillsnow, Stills, podcast

1st Line Biologics vs csDMARDs in Adult Still's Disease

Jack Cush, MD

A German multicentre, retrospective study assessed the first-line efficacy of biologics and conventional synthetic DMARD therapy in patients with adult-onset Still's disease and found biologic agents were significantly better with sustained, event-free remissions and fewer complications.

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If COVID was responsible for causing autoimmune Dz (AD), it is possible that Molecular mimicry was in play. Study shows 10nsequence homology between SARS-CoV-2 & AD epitopes; 2) similar MHC binding; 3) of high empirical immunogenicity. https://t.co/WO3gjxhVBw https://t.co/QdqtQMHcpL

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Dr. John Cush @RheumNow( View Tweet )
A comprehensive examination of the pathogenesis and recent advancements in Familial Mediterranean Fever (FMF). This condition results from autosomal recessive mutations in the MEFV gene, which codes for Pyrin, a crucial component of the NLRP3 inflammasome. These genetic https://t.co/XNV89LW893
Dr. John Cush @RheumNow( View Tweet )
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ERA, APPs, & Alpha GAL (3.21.2025)

Jack Cush, MD

Dr. Jack Cush reviews the news and journal reports from this past week on RheumNow.com.  Listen in for 2 new case questions - Ask Cush Anything.

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Sobi announced it has submitted to FDA a supplemental (sBLA) for Gamifant (emapalumab-Izsg) for use in adult & kids w/ hemophagocytic lymphohistiocytosis (HLH)/macrophage activation syndrome (MAS) in Still’s disease with an inadequate response glucocorticoids, or with recurrent… https://t.co/a3I1fzHLdl https://t.co/WTFAs5PLWF
Dr. John Cush @RheumNow( View Tweet )
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World Changers (3.14.2025)

Jack Cush, MD

Dr Jack Cush and his podcast friends are out to change the world. Here is his weekly review of the news and journal reports from the past week on RheumNow.

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Recent Blogs

Best of 2022: Five Mistakes When Diagnosing Still’s Disease

Jack Cush, MD
Thermometer
Adult-onset Still's presents an interesting and diagnostic challenge when encountered. Here are 5 tips to improve your diagnostic acumen for this febrile disorder. Read Post

Best of 2022: Myths & Misconceptions with Febrile Disorders

Rachel Tate, DO
Myth vs reality
Still’s disease and autoinflammatory disorders are rare conditions unified by recurrent fevers and inflammation of skin, joints, and other organs. While labs often support the diagnosis, these conditions are best diagnosed by genetic testing, or by specific criteria (in the absence of a genetic test). The following is a table that lists common misconceptions (myths) and their clarifying explanations. Read Post
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