Behçet’s disease is a chronic, relapsing and remitting vasculitis with multisystem involvement. Commonly referred to as the Silk Road disease due to its prevalence in the Asian and Mediterranean region of the traditional Silk Road, Behçet’s was first described by Hippocrates as a triad of symptoms—genital and oral ulcers with uveitis—and attributed to links with…
Granulomatosis with polyangiitis (GPA) was first described in the British Medical Journal in 1897 by Scottish otolaryngologist Peter McBride.1 GPA is a relatively rare, systemic necrotizing vasculitis that can make diagnosis challenging. The incidence has been estimated anywhere between two and 12 cases per million.2 GPA mainly affects adults between the ages of 45 and…
NEW YORK (Reuters Health)—Cryoglobulinemic vasculitis associated with hepatitis C virus (HCV) infection resolves after effective treatment with direct-acting antivirals (DAAs), with most patients remaining in remission for two or more years, researchers from Spain report. “Most clinical manifestations of the disease improve over time, but some patients may have a clinical recurrence of their disease…
Baltimore—Rheumatologists and meteorologists share more in common than you might suspect, said Dr. Rebecca Manno, assistant professor of medicine for the Division of Rheumatology at Johns Hopkins University, Baltimore, and assistant director of the Johns Hopkins Vasculitis Center. At a March 8 meeting of the Maryland Society for the Rheumatic Diseases, Dr. Manno used the…
Eosinophilia is usually defined as an eosinophil count of more than 500/microL in peripheral blood.1 An eosinophil count of more than 1,500 is referred to as hypereosinophilia (HE); hypereosinophilic syndrome (HES) is defined as HE associated with organ dysfunction attributable to eosinophilia.2 Eosinophilia can occur due to infectious, malignancy, autoimmune or allergic etiologies. However, a…
A proposed model to predict the risk of giant cell arteritis (GCA) prior to a temporal artery biopsy could help triage patients and guide decision making about the need for biopsy or monitoring (see Figure 1). There’s no specific biomarker for GCA, and GCA can be a “diagnostic conundrum, especially when it presents in an…
In 1866, Adolf Kussmaul, an internist, and Rudolf Maier, a pathologist, published the classic characterization of what eventually became known as polyarteritis nodosa.1 It was the first scientific clinical characterization of a noninfectious vasculitis. As such, it became a paradigmatic point of contrast to other types of vasculitides that were later described. Their description also…
SAN DIEGO—Recent research tells us more about giant cell arteritis (GCA) to help rheumatologists more accurately diagnose and effectively treat patients with this type of vasculitis. On Nov. 6 at the ACR/ARHP Annual Meeting, three experts explored the latest findings on GCA pathogenesis, diagnostic approaches, imaging modalities and growing treatment options. GCA: What’s Really Happening?…
SAN DIEGO—At the 2017 ACR/ARHP Annual Meeting this past November, three researchers discussed the latest ANCA-associated vasculitis (AAV) research, including studies on AAV pathogenesis, therapies and remission maintenance. In the Philip Hench, MD, Memorial Lecture, J. Charles Jennette, MD, chair in pathology and laboratory medicine at the University of Carolina at Chapel Hill in Chapel…
SAN DIEGO—An increasing number of patients is being identified with deficiency of adenosine deaminase type 2 (DADA2); fortunately, researchers and clinicians continue to better understand the genetic disease as well, experts said in a session at the 2017 ACR/ARHP Annual Meeting Nov. 3–8. The childhood-onset disease involves loss-of-function mutations to the CECR1 gene (i.e., cat…