Based on the classification system developed by the Chapel Hill Consensus Conference, anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis is defined as a necrotizing vasculitis involving small vessels that is associated with myeloperoxidase (MPO) ANCA or proteinase 3 (PR3) ANCA and displays minimal immune deposits. The mechanism behind the pathogenesis of ANCA-associated vasculitis is not fully…
The past five years have been busier than usual for the Churg-Strauss syndrome. It was renamed eosinophilic granulomatosis with polyangiitis (EGPA).1 Longitudinal cohorts totaling 484 patients—approximately as many as all previous series combined—were described.2,3 A proposal was advanced to remove and rename a subset in which vasculitis may not be present.4 And shortly after the…
A 43-year-old man with a past medical history of type 2 diabetes mellitus, bilateral inguinal hernia repair as a child and prior cholecystectomy woke from sleep with sudden-onset periumbilical abdominal pain. He was admitted to another hospital, but required transfer to our surgical intensive care unit after a recurrent episode of severe abdominal pain, during…
CHICAGO—High eosinophil counts that can’t be explained should concern rheumatologists and spark attempts to identify what’s happening, an expert said at the ACR State-of-the-Art Clinical Symposium in April. “Eosinophil counts over 1,500 (per microliter) should provoke worry,” said Bruce Bochner, MD, professor of medicine in allergy and immunology at Northwestern University Feinberg School of Medicine…
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…