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Rare Diseases

Best of 2025: ERS/EULAR guidelines for CTD-related interstitial lung disease

The European Respiratory Society (ERS) and European Alliance of Associations for Rheumatology (EULAR) have published clinical practice guidelines for the evaluation and management of connective tissue diseases (CTD) associated interstitial lung disease (ILD) in two simultaneous publications in

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Best of 2025: 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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Disease Activity Criteria for Adult-Onset Still's Disease: EULAR Points to Consider EULAR has published a "points to consider" (PtCs) guidance on the development of criteria for the assessment of the disease activity in adult-onset Still’s disease (AOSD). https://t.co/3FikFiGgxM
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Nerandomilast FDA Approved for Progressive Pulmonary Fibrosis

The FDA has approved nerandomilast (Jascayd), a PDE4b inhibitor, to treat adults with progressive pulmonary fibrosis (PPF). Nerandomilast was approved to treat idiopathic pulmonary fibrosis (IFP) in October 2025.  

PPF is a a life-threatening, progressive lung condition, leading to

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Best of 2025: Overview of the VEXAS Syndrome

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

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Telling You Where to Go (12.19.2025)

Dr. Jack Cush reviews the news and journal articles from the past week on RheumNow.com. Who would've thunk - a PsA FMF connection? Labs to diagnose SSc-ILD? IgA-RF may be important or RZV may not work in JAK inhibitor treated patients?

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Disease Activity Criteria for Adult-Onset Still's Disease: EULAR Points to Consider

EULAR has published a "points to consider" (PtCs) guidance on the development of criteria for the assessment of the disease activity in adult-onset Still’s disease (AOSD).

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Reefer Madness (12.12.2025)

Dr. Jack Cush reviews the news, journal and regulatory reports from this past week on RheumNow.com.  B cell drugs in SLE and ITP, biomarkers in GCA & PSS and great videos by APPs.

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B Cell Targeting in Idiopathic Thrombocytopenic Purpura

EurekAlert!

The New England Journal of Medicine  has published a phase III clinical trial showing that a B cell targeting monoclonal antibody, ianalumab, was effective in more than half of the patients with primary immune

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Hematologic Malignancies Increased in Systemic Sclerosis Swedish national study found that among a cohort of systemic sclerosis (SSc) patients a two-fold increase risk of hematological malignancies was seen. https://t.co/K1WuMC0mXI https://t.co/MGkvwESqIe
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APPs in the News (12.5.2025)

Dr. Jack Cush reviews the news and journal reports from RheumNow.com, including info on scleroderma, dermatomyositis and malignancy, rheumatologist and APP salary concerns.

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Hematologic Malignancies Increased in Systemic Sclerosis Swedish national study found that among a cohort of systemic sclerosis (SSc) patients a two-fold increase risk of hematological malignancies was seen. https://t.co/BypsCRUthe https://t.co/lfLJhSqn9e
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10 yr European retrospective study of haemophagocytic lymphohistiocytosis found 3345 deaths due HLH from 29 countries. Crude mortality rate = 5.3 per 10million PYs (range: 1.1-11.3). From 2011 to 2021, increasing from 3.9 to 6.6/10millPYs, varying by country, sex, age. https://t.co/58gCQ72saF
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Hematologic Malignancies Increased in Systemic Sclerosis

Swedish national study found that among a cohort of systemic sclerosis (SSc) patients a two-fold increase risk of hematological malignancies was seen.

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A New Disease Activity Score for Antiphospholipid Syndrome? APS is a systemic autoimmune disorder. It is characterised by the presence of antiphospholipid antibodies and a broad spectrum of clinical features, causing obstetric as well as thrombotic, microvascular and https://t.co/G2xGtOPmwb
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Coccyx and Sacrococcygeal Joint Disorders

Pain from coccygeal or sacrococcygeal disorders is rare, often presenting as a diagnostic challenge. Coccygeal pain is often mistaken for lumbosacral, pelvic, or gastrointestinal disorders. This review presents the epidemiology, anatomy, biomechanics, presentations, diagnostic evaluation,

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Treating Refractory Still's Disease

A full read systematic review article examined treatment options for difficult to manage systemic juvenile idiopathic arthritis, or Still’s disease. It classified these refractory disease patients as those with either (1) persistent arthritis, (2) recurrent or difficult-to-treat

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ILD in Patients with Connective Tissue Disease Dr. Janet Pope reports on abstract 0154 (Incidence and Prevalence of Connective Tissue Diseases with Interstitial Lung Disease (CTD-ILD) in the United States) at #ACR25. https://t.co/WrJhsMizwV https://t.co/rkFny5ZjZQ
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Organ Involvement with Behçet’s A review of patient data from the International AutoInflammatory Disease Alliance (AIDA) Network registry identifying those with mucocutaneous Behçet’s disease (BD) may progres to major organ involvement (MOI)m especially at later stages. https://t.co/jqM4IZuobe
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A New Disease Activity Score for Antiphospholipid Syndrome? APS is a systemic autoimmune disorder. It is characterised by the presence of antiphospholipid antibodies and a broad spectrum of clinical features, causing obstetric as well as thrombotic, microvascular and https://t.co/eouynDPLUX
Dr. John Cush @RheumNow( View Tweet )
Myelodysplastic & chr myelomonocytic leukemia pts rarely get lupus. Review of 19 w/ SLE & 5 w/ CLE; these were older (65 yrs), more male (15M/9F), w/ less renal [10%] & articular [36%] Dz w/ less dsDNA [32%]. Thought to be clonal inflammatory, & not autoinflammatory, process. https://t.co/EAvkJm6GQs
Dr. John Cush @RheumNow( View Tweet )

Organ Involvement with Behçet’s

A review of patient data from the International AutoInflammatory Disease Alliance (AIDA) Network registry identifying those with mucocutaneous Behçet’s disease (BD) may progres to major organ involvement (MOI)m especially at later stages.

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Pulse Steroids and Mycophenolate in Juvenile Dermatomyositis

JAMA Dermatology has published a pilot study demonstrating the safety and efficacy of intermittent intravenous methylprednisolone pulse (IVMP) therapy plus mycophenolate in 28 patients with juvenile dermatomyositis (JDM).

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20-40% of Autoimmune hepatitis (AIH) is associated with other autoimmune diseases - lead by autoimmune thyroiditis, type 1 DM, ulcerative colitis, Crohn disease, & celiac (but less so for RA, PBC, SLE, Sjögren syndrome, vitiligo). AIH Dx Abs include ANA, ASMA, LKM1. https://t.co/FphWZwwohx
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Beyond malignancy, look out for LICATS with CAR-T CD19 chimeric antigen receptor (CAR) T cell therapy took the centre stage in 2022 where data pertaining to a successful case series of 5 patients with refractory SLE were published by Prof Schett’s group. Since then, this area https://t.co/JbTB033gti
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