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An official publication of the ACR and the ARP serving rheumatologists and rheumatology professionals

Other Rheumatic Conditions

The Latest Autoinflammatory Discoveries & Tips

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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

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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…

Gene Signatures in IgG4-Related Disease

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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

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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

Usman T. Malik, MBBS  |  

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

Larry Beresford  |  

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…

Laurent/Yakou / Science Source

How to Recognize, Diagnose Periodic Fever Syndromes in Adults

Rick Brasington, MD  |  

A number of autoinflammatory syndromes that result from genetic mutations have been described recently. The vast majority occur in children. However, three periodic fever syndromes are important for rheumatologists who treat adults to know about. The goal of this review is to provide a concise description of each condition, and to help the clinician understand…

Fellows’ Forum Case Report: Necrotizing Autoimmune Myopathy

Shivani Garg, MD, Suzana Alex John, MD, & Frehiywot Ayele, MD  |  

Necrotizing autoimmune myopathy (NAM) is a relatively recently discovered subgroup of inflammatory myopathies. NAM is characterized by predominant muscle fiber necrosis and regeneration with little or no inflammation.1 One subgroup of NAM is 3-hydroxy-3-methylglutaryl-CoA reductase antibody (HMGCR Ab)-related immune-mediated necrotizing myopathy (IMNM), which occurs (rarely) after statin exposure, with a rough incidence of two per…