Rare Diseases

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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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Unmet Needs in Still’s disease 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 (sJIA) and adult-onset SD (AOSD). They addressed four main https://t.co/h6FNkQyoKj
Dr. John Cush @RheumNow( View Tweet )
Post-treatment Lyme Syndrome Symptoms that persist long after Lyme disease is treated are not uncommon — a 2022 study found that 14% of patients who were diagnosed and treated early with antibiotic therapy would still develop Post Treatment Lyme Disease (PTLD). https://t.co/mmwGCnARY8
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Prevalence of ANCA Assoc Vasculitis (especially GPA) in Alaskan Natives (AN/AI) is high. GCA prevalence is lower than White populations, but higher than many others. AN/AI peoples w/ AAV & GCA may present at younger ages with more severe disease https://t.co/dhiu6sPGBk https://t.co/rNCo25HGcn

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Rheumatology: Believe It or Not (4/25/2025)

Jack Cush, MD

Dr. Jack Cush reviews the news and journal reports from the past week on RheumNow.com. Should we believe the reviews and metanalyses?

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Castleman disease (CD) is a rare, non-clonal lymphoproliferative disorder w/ systemic sxs & maybe linked w/ herpesvirus 8, POEMS or TAFRO syndrome. Mimickers include lymphoma, sarcoma, IgG4-RD, Infections (EBV, HHV8, HIV, CMV, TB), Autoimmune Dz (RA, SLE, Stills) https://t.co/29M0jePXHd
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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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Castleman disease (CD) is a rare, non-clonal lymphoproliferative disorder w/ systemic sxs & maybe linked w/ herpesvirus 8, POEMS or TAFRO syndrome. Mimickers include lymphoma, sarcoma, IgG4-RD, Infections (EBV, HHV8, HIV, CMV, TB), Autoimmune Dz (RA, SLE, Stills) https://t.co/70sROoHusy
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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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Enzyme Porcupine inhibition of Wnt signaling is a promising treatment forsclerosteosis - rare Dz caused by SOST mutations & loss of functional sclerostin. Results in high bone mass & skeletal overgrowth (Sxs of facial paralysis, hearing loss, incr intracranial pressure https://t.co/Bgr5zoHyMG
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Genetic Sequencing in Rheumatology Genetic sequencing as a research and diagnostic tool has expanded in medicine and rheumatology. An interesting review in Arthritis and Rheumatology suggests that despite currently modest use, there will be an expanded role for genetic

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Genetic Analysis of Asymptomatic Antinuclear Antibody Positive Patients A large scale genomic population study found antinuclear antibodies (ANA) in up to 14% of the population; most with ANA are asymptomatic, and ANA production is not associated with significant genetic risk. https://t.co/Ltqf1yaaoy
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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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Enzyme Porcupine inhibition of Wnt signaling is a promising treatment forsclerosteosis - rare Dz caused by SOST mutations & loss of functional sclerostin. Results in high bone mass & skeletal overgrowth (Sxs of facial paralysis, hearing loss, incr intracranial pressure https://t.co/9DYO82quiC
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Phase 3 trial shows inebilizumab reduces flares in IgG4-related disease (10% vs 60% in placebo; HR 0.13, p<0.001) and boosts remission rates without background immunosuppressants. CD19+ B-cell depletion shows promise as a new treatment. #Rheum @RheumNow https://t.co/7iSyPfnMV0 https://t.co/M51b0dipMs

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Natasha Salman, DO @DrNatashaSalman( View Tweet )
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Is High Dose Aspirin Needed in Kawasaki Disease?

Jack Cush, MD

Does aspirin add any advantage to intravenous immunoglobulin (IVIG) alone in children with Kawasaki disease (KD)? A randomized clinical trial IVIG alone was not inferior to IVIG plus aspirin, suggesting that high-dose aspirin during initial IVIG treatment did not reduce coronary artery

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Prof Ian Giles will be covering a range of topics at this year's Rheumapreg Conference such as how immunologic changes and placental dysfunction cause adverse outcomes in rheumatic disease pregnancies. View the full prgramme and learn more: https://t.co/tjiycjKJ8i https://t.co/FugHvtm8fY

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BSR @RheumatologyUK( View Tweet )
Sjogrens is a hot area in drug development: “Sjogren’s Syndrome Pipeline Insight 2025” report provides comprehensive insights about 20+ companies and 22+ pipeline drugs, including: nipocalimab VAY736 (ianalumab) HZN-1116 AMG 329 R-2487 https://t.co/sVbyPLi2Um https://t.co/IlvthM7qyG

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EMR Messaging Woes (4.11.2025)

Jack Cush, MD

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

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Systemic sclerosis pts in the PRESS & STRIKE cohorts - High type I interferon (IFN) scores in diffuse SSc pts correlated w/ high disease activity & worse 12-month prognosis and overall survival. https://t.co/pdPjoVfQNH https://t.co/RB7li2ZIxX

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Dr. John Cush @RheumNow( View Tweet )
Inebilizumab, an anti-CD19+ (B cell)mAb was tested in myasthenia gravis in phase 3, 238 pt trial and shows to signif. improv function and reduce disease severity in acetylcholine recpt Ab+ or muscle-specific kinase Ab+ myasthenia gravis pts. https://t.co/vBBjf8AUug https://t.co/cUbK4PJEvf

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FDA announced approval of Inebilizumab (Uplizna; Amgen), 1st approved Rx for IgG4-related disease (targets CD19+ B cells). IG4-related Dz is estimated to affect 20,000 in the USA. List price is $140,248.50 per dose, After two initial loading doses it is given q 6 mos. https://t.co/aD9yBVJukQ
Dr. John Cush @RheumNow( View Tweet )
FDA announced approval of Inebilizumab (Uplizna; Amgen), 1st approved Rx for IgG4-related disease (targets CD19+ B cells). IG4-related Dz is estimated to affect 20,000 in the USA. List price is $140,248.50 per dose, After two initial loading doses it is given q 6 mos. https://t.co/zHD2Yv3PjI
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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Cancer Survival with TNF Inhibitors (3.28.2025)

Jack Cush, MD

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

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Recent Blogs

Choice Overload (and the Way Out)

Jack Cush, MD
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I recently overheard a very good practicing rheumatologist complain, “why do I need yet another drug for psoriatic arthritis? I have more treatment choices than I have actual PsA patients who need a new or better drug”. Read Post

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

Jack Cush, MD
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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
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