Rare Diseases

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Rituximab is not Superior in EGPA

Jack Cush, MD

A cohort study of Eosinophilic Granulomatosis With Polyangiitis (EGPA) patients from the French Vasculitis Study Group failed to show superiority of rituximab compared to conventional strategy with glucocorticoids (GS) alone or in combination with cyclophosphamide.

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Emapalumab Approved for MAS in Still’s On June 27th, 2025, the FDA approved emapalumab for macrophage activation syndrome (MAS) in known or suspected Still’s disease (including both adult-onset Still’s disease (AOSD) and systemic juvenile idiopathic arthritis (sJIA)) with https://t.co/431PQlzBsV
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Fibrosis in IgG4-related disease due to the overexpression of early growth response-1 (EGR-1) by Epithelial cells that facilitates tissue fibrosis https://t.co/AEaoucfT13 https://t.co/7UVQpkj3cn https://t.co/PuCjh5LQc5

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CKD & Osteoporosis Rx Revisited (7.25.2025)

Jack Cush, MD

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

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Clinical Associations with Anti-RNA Polymerase III Antibodies A systematic review of anti-RNA polymerase III antibodies (ARA) in systemic sclerosis (SSc) patients finds ARA positivity in only 9% of SSc patients, but come with a higher risk of many potentially worrisome https://t.co/p1KVUFPJvN
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US CDC study of age-standardized mortality rate (ASMR) from 1990-2020, there were 39,496 deaths related to scleroderma (SSc) & and 5,163 from SSc w/ renal failure. Deaths lower in males (22%) than females w/ renal failure (78%). Mortality rate of SSc w? renal failure was https://t.co/OT3BHt3E85
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Fibrosis in IgG4-related disease due to the overexpression of early growth response-1 (EGR-1) by Epithelial cells that facilitates tissue fibrosis https://t.co/PBMqjr0MVV https://t.co/d9b7k2zwYE https://t.co/25aIjIrYm3

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Retrospective review of 457 Autoimmune liver disease pts found 42.5% with Polyautoimmunity. Predictors included ANA positivity (78 vs 47%), ANA titers, dsDNA (12 vs 2%), RF+ (25 vs 9%), disease duration, low albumin, high ESRs. https://t.co/Socl6XWU9a https://t.co/8qomVBh3KP

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Manifestations and Treatment in Relapsing Polychondritis A large multicenter cohort study of Relapsing Polychondritis (RP) describes the clinical manifestations and treatment approaches to this rare, heterogeneous, multisystem disease. https://t.co/Gz2qwtC4jZ https://t.co/llK9W45DlW

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SSB, > SSA, Abs are signif associated w/ abnormal DLCO (AUC 0.791) in Sjogrens syndrome, esp in assoc. w/ LIP- lymphocytic interstitial pneumonitis. These findings more likely in quadruple positive Sjogrens w/ RF/ANA/SSA/SSB positivity. https://t.co/s6IyRzfXiN https://t.co/rKDBovTOpl

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NHANES, Numbers, & Risk (7.18.2025)

Jack Cush, MD

Dr. Jack Cush reviews the news and journal reports from this past week on RheumNow.com along with interesting case questions from "Ask Cush Anything".

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Manifestations and Treatment in Relapsing Polychondritis A large multicenter cohort study of Relapsing Polychondritis (RP) describes the clinical manifestations and treatment approaches to this rare, heterogeneous, multisystem disease. https://t.co/Gz2qwtC4jZ https://t.co/ZLkjNQmbRC

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Dr. John Cush @RheumNow( View Tweet )
SSB, > SSA, Abs are signif associated w/ abnormal DLCO (AUC 0.791) in Sjogrens syndrome, esp in assoc. w/ LIP- lymphocytic interstitial pneumonitis. These findings more likely in quadruple positive Sjogrens w/ RF/ANA/SSA/SSB positivity. https://t.co/s6IyRzfXiN https://t.co/J3bMowsrUQ

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Dr. John Cush @RheumNow( View Tweet )
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1 in 5 Patients are Unaware of their Arthritis Diagnosis

Jack Cush, MD

Current CDC numbers suggest that arthritis (any type) affects an estimated 67.1 million (27.9% ) adults in the USA.  Interestingly, 1 in 5 do not know their arthritis subtype (21.6%, 14.4 million).

This most recent analysis of the prevalence and distribution of arthritis subtypes

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Interstitial Lung Disease and Lung Cancer Risk A large prospective cohort study found that interstitial lung disease (ILD) is associated with an increased risk of lung cancer. https://t.co/SdOT28qVfr https://t.co/6KhAijhfHw

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Dr. John Cush @RheumNow( View Tweet )
Martinque, Afro-Caribbean population (n 354 800) study(2004-2022) of 33 AOSD pts (71%F; 79% systemic; 21% chr articular); 91% met Yamaguchi criteria; features as expected but more Pulmonary Dz(51%; 94% w/ a systemic pattern). Half Dx < age 35 yrs. Prevalence 7.6 /100 000 mean https://t.co/QWyfNuvhkl
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Manifestations and Treatment in Relapsing Polychondritis

Jack Cush, MD

A large multicenter cohort study of Relapsing Polychondritis (RP) describes the clinical manifestations and treatment approaches to this rare, heterogeneous, multisystem disease

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Interstitial Lung Disease and Lung Cancer Risk

Jack Cush, MD

A large prospective cohort study found that interstitial lung disease (ILD) is associated with an increased risk of lung cancer.

This cohort study of 5,425,976 used a a sibling-controlled design to account for genetic factors. Data was taken from the Swedish Total Population Register

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Overview of the VEXAS Syndrome A current systematic review in Rheumatology addresses the clinical features seen in the VEXAS syndrome. https://t.co/ZjuOO08sKx https://t.co/rNVBFxgjZY

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Study of 74 systemic sclerosis pts shows nailfold videocapillaroscopy (capillary disorganization, late pattern changes) predicts ILD progression, worsening PFTs & mRSS in SSc over 2 yrs. KL-6 correlates w/ NVC findings & PFT decline. ILD incr from 38 to 51% in 2yrs https://t.co/A3LiG6Nn0m
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Is the term polymyositis obsolete?.. now being replaced by dermatomyositis, necrotizing myopathy, inclusion body myositis, antisynthetase syndrome & overlap myositis. Full read overview of inflammatory myositis & new treatments from Joint Bone & Spine https://t.co/X46sptp1Ax https://t.co/smqCd8Rm2o

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EUSTAR Study of 893 SSc-ILD pts - 94 (10.5%) died (mean F/U 39 mos). Predictors of mortality: FVC deterioration >10% w/in 12 mos (HR 3.81) & >10% FVC decline or mRSS worsening (HR 2.82); but not changes in DLCO, mRSS or digital ulcer burden alone. https://t.co/28wSQc1dUS https://t.co/dourQ6WGDq

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Dr. John Cush @RheumNow( View Tweet )
Is the term polymyositis obsolete?.. now being replaced by dermatomyositis, necrotizing myopathy, inclusion body myositis, antisynthetase syndrome & overlap myositis. Full read overview of inflammatory myositis & new treatments from Joint Bone & Spine https://t.co/X46sptp1Ax https://t.co/QqyMv6fkVS

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Dr. John Cush @RheumNow( View Tweet )
Referral of 304 suspected autoinflammatory Dz (SAID) to a UK national centre for primarily adult SAID in 2022, 121 (39.5%) were diagnosed with SAID, w/ half of these having monogenic Dz. Median Dx delay was 2 to 6 yrs, worse in late-onset SAID. https://t.co/x8VOFyjuVA https://t.co/3R2HdYUlSg

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Vexas

Overview of the VEXAS Syndrome

Jack Cush, MD

A current systematic review in Rheumatology addresses the clinical features seen in the VEXAS syndrome.

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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
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
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