Immunoglobin G4-related disease (IgG4-RD) is a rare fibro-inflammatory disease of unknown etiology that has been recently recognized. It can cause fibro-inflammatory masses in almost every organ of the body and is associated with dense lymphoplasmacytic infiltration of IgG4-postitive plasma cells, storiform fibrosis and elevated levels of serum IgG4.1 IgG4-RD is a systemic disease that may…
International Approvals for Guselkumab & Anakinra; Plus Adalimumab Biosimilar in Europe
In Japan the use of guselkumab to treat erythrodermic, plaque and pustular psoriasis is approved, and anakinra is now indicated for Still’s disease in the E.U…
Risankizumab Promising for Psoriasis; Plus Canada Approves Brodalumab
Two Phase 3 clinical trials show that risankizumab is effective for achieving skin clearance in patients with psoriasis…
The Latest Autoinflammatory Discoveries & Tips
SAN DIEGO—Since the identification of the autoinflammatory disease, familial Mediterranean fever (FMF), researchers have identified an alphabet soup of other autoinflammatory diseases with genetic underpinnings, from PAPA (pyogenic arthritis with pyoderma gangrenosum and acne) to NIAID (NLRP1-associated autoinflammation with arthritis and dyskeratosis) to TRAPS (TNF receptor-associated periodic syndrome). The torrent of discoveries, brought about largely…
An Amylodosis Warning & New Drug Hope
SAN DIEGO—Treatments do exist that can improve the prospects of a patient with the rare disease amyloidosis, but only if clinicians keep the disease in mind and treat the patient before it’s too late, an expert said at the 2017 ACR/ARHP Annual Meeting this past November. He also discussed research that may be close to…
Secukinumab Promising for Sustained Improvements in Palmoplantar & Nail Psoriasis
In clinical trials, secukinumab proved safe and effective as a long-term treatment for both palmoplantar and nail psoriasis…
Gene Signatures in IgG4-Related Disease
Research suggests prednisone treatment can selectively modulate the signatures of regulatory T (Treg) cells, eosinophils and neutrophils in patients with IgG4-related disease. The results show IgG4-related disease may result from an imbalance of immune and inflammatory cells…
Fibrotic Diseases of the Retroperitoneum & Skin, & Rare Scleroderma Mimics
SAN DIEGO—Fibrosis affects all organ systems, but isn’t always systemic sclerosis. Experts on less common forms discussed patient presentations, diagnosis and treatment at the 2017 ACR/ARHP Annual Meeting in San Diego on Nov. 6. Retroperitoneal Fibrosis Formerly called Ormond’s disease, retroperitoneal fibrosis (RPF) is usually an IgG4-related disease, but has some unique characteristics, said John…
Fellows’ Forum Case Report: Progressive Weakness and Debilitation with Skin Rash
The Presentation A pale, quiet woman—her mother—wheeled the girl into my clinic. It was a blistering Florida day, and the girl was shivering. She glanced up at me when I said hello and asked her name. “Hi,” she said, giving me a broad smile. Her smile was the only broad thing about her. Her elbows…
CCL2 Cytokine Serves as Biomarker in Interstitial Lung Disease
A recent prospective, observational cohort study of potential clinical biomarkers for progression to interstitial lung disease (ILD) in patients with early systemic sclerosis (SSc) found that higher levels of CCL2 circulating in their plasma predicted both faster ILD progression and poorer survival rates than in those with lower levels.1 CCL2, also known as monocyte chemoattractant…
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