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Rare Diseases

Telitacicept, a BLyS (BAFF)/APRIL dual-target fusion drug, announced it has met the primary endpoint in its Phase III clinical trial in treating primary Sjögren's syndrome (pSS) in China. Primary endpoint was the change in ESSDAI score at Week 24. https://t.co/afOgpGLdV1 https://t.co/9EKbbrCgTL
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Antibiotics are not linked to Autoimmune disease in kids

EurekAlert!

The global incidence of autoimmune diseases among children has increased over the past few decades. A study published August 21st in the open-access journal PLOS Medicine by Ju-Young Shin at Sungkyunkwan University, Republic of Korea, and colleagues suggests that early

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Sjögren's Graduates (8.22.2025)

Dr. Jack Cush reviews the news, journal reports, FDA approvals, drug safety, and more from this week.

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Cardiovascular Sequelae in Adults After Kawasaki Disease

A long-term cohort study of children diagnosed with Kawasaki disease (KD) shows evidence of future adult cardiovascular complications and hospitalizations. These severe cardiovascular events clustered during the young KD adults and later, a second surge in their late 30s. 

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Glossary for Giant Cell Arteritis Dejaco and colleagues have published a glossary of terms often used in conjunction with giant cell arteritis. The glossary of definitions for 23 signs and symptoms of GCA was developed through a consensus process involving international https://t.co/cIA3UvNcP6
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TNF Inhibitors in Takayasu's Arteritis

A large, retrospective, multicenter study demonstrated the efficacy of intravenous infliximab and  subcutaneous adalimumab in patients with Takayasu arteritis.

This multicentre study enrolled patients from Takayasu's referral centers in France, Italy, Spain, Armenia,

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Guidance on aromatase inhibitor-associated bone loss with hormone-sensitive breast cancer

EurekAlert!

An updated joint position statement on the management of aromatase inhibitor-associated bone loss (AIBL) addresses the heightened fracture risk in women undergoing endocrine therapy for estrogen-responsive breast cancer, providing practical guidance for both osteoporosis specialists

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Nailfold videocapillaroscopy (NVC) in Dx of systemic sclerosis - study of 258 NVCs found giant capillaries (specificity 98%) & capillary density to be best at defining a scleroderma pattern (39%). Density ≤8 capillaries/mm best defined dropout (AUC 0.911); having both = 91% https://t.co/WQx6fbQiAB
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The Patient Journey with Rheumatic Disease

Oton and colleagues have reviewed the "patient journey" - from symptoms to specialty referral in those diagnosed with rheumatic and musculoskeletal diseases (RMDs).
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Retrospective UK study of CPRD dataset (2012-2023) found 22 829 incident CTD Dx (81% F). Over time incidence SLE & Systemic Sclerosis fell, Sjogrens & MCTD stable, & IIM rose 3.23–4.31/100K. IIM had highest mortality (28/1000 py), followed by SSc, SLE, MCTD; lowest SjD (9.7)

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2025 ACR Guidance on Diagnosis and Management of VEXAS

The ACR has published a formal international consensus guidance on VEXAS as a resource for clinicians seeking to understand the disease and its management. 

VEXAS is a rare genetic disorder; the acronym stands for Vacuoles E1 enzyme X-linked

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DID YOU KNOW? >30% of Systemic Sclerosis pts present without Raynauds!. Data from 2 large US cohorts, of SSc dx with puffy fingers/hands or other symptoms. Such patients presented with more severe skin and musculoskeletal disease. https://t.co/NP3KeUL5iX https://t.co/XWiAIljujL
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Repurposing & Pipeline (8.8.2025)

Dr. Jack Cush reviews the news, reports, pipeline and drug repurposing on this week’s podcast.

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Dermatomyositis Reviewed

Researchers from the University of Pennsylvania have published an open-access review of dermatomyositis (DM) - its manifestations, hallmark cutaneous features, etiopathogenesis and treatment options.

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Retrospective study of 63 pts w/ Cryoglobulinemia (essential mixed [EM], CTD-related) vasculitis (CryoVas) in remission post RTX induction. W/ 58 mos F/U Relapse rates were 23%, 42%, 71% (@ 1yr, 2yrs, 5yrs). Relapse risk w/ purpura [HR 2.2] & prior CryoVas flare (HR 1.9). https://t.co/6zGgSN8Wfe
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Pipeline of drugs being developed for Scleroderma by these companies - Bayer, Novartis, GSK, Boehringer Ingelheim, Celgene Corp., Johnson & Johnson, Prometic, Cytori arGentis - either as fusion proteins, peptides, small molecule, mAb, Polymern& Gene therapy https://t.co/dWRcQ1a3GU
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Juvenile Idiopathic Arthritis - NEJM Review

Sandborg et al. has published an overview of juvenile idiopathic arthritis (JIA) - its classification, biology, genetics, , clinical presentations and treatment advances in the New England Journal of Medicine (NEJM).

JIA is the most common form of inflammatory arthritis in the pediatric

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🧵 Vasculitis is Not One Disease – It’s a Spectrum It’s not a diagnosis. It’s a mechanism. From small-vessel purpura to large-vessel aneurysms, here’s how to recognize and reason through the vasculitis spectrum👇 @Amansharmapgi @DurgaPrasannaM1 @12VRavindran @Kanjivellam https://t.co/eEalTpDTGD
Aravind Palraj @Rheumat_Aravind( View Tweet )

2025 BSR Recommendations for ANCA-associated Vasculitis

The British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) have published a guideline for the management of adults with ANCA-associated vasculitis (AAV); specifically three conditions: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA)

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Rituximab is not Superior in EGPA

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)

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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