The 19th Annual Johns Hopkins Advances in the Diagnosis and Treatment of Rheumatic Diseases Symposium session titled, Updates on Vasculitis provided an overview of the latest information on vasculitis diagnosis and treatment.
Case Report: GPA or IgG4-Related Disease in an 11-Year-Old Girl?
Granulomatosis with polyangiitis (GPA) is a type of anti-neutrophil cytoplasmic antibody (ANCA) associated small vessel vasculitis that typically affects the kidneys, lungs and sinuses.1 Due to an overlap in signs and symptoms, GPA may initially be difficult to distinguish from IgG4-related disease, another condition that can affect multiple tissues and has variable presentations. Further complicating…
GPA & Relapsing Polychondritis Discussed
Challenges related to diagnosing chondritis are discussed.
FDA Approves Rituximab for Children with GPA & MPA
Intravenous rituximab can now be used to treat pediatric patients with GPA and MPA as young as two years old…
Mortality from Anti-neutrophil Cytoplasmic Autoantibody-Associated Vasculitis Falling Slightly
NEW YORK (Reuters Health)—Mortality rates in patients with antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV) decreased from 1999 to 2017, according to records from the Centers for Disease Control and Prevention (CDC). “The mortality attributed to several rare diseases [e.g., hypersensitivity pneumonitis] has increased in recent years,” Alexander W. Steinberg, MD, from Saint Joseph Hospital, Denver, tells…
Rituximab Receives Label Update for ANCA-Associated Vasculitis
The FDA has approved an update for rituximab’s label, which will include safety and efficacy information for treating ANCA-associated vasculitis…
The Classification & Diagnosis of Granulomatosis with Polyangiitis
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…
Case Illustrates the Difficulty Diagnosing Granulomatosis with Polyangiitis
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…
ANCA-Associated Vasculitis Pathogenesis & Treatment Updates
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…
GPA Patient Carries NORD Banner to Top of Mt. Everest
On May 23, 2010, Cindy Abbott was standing on top of the world. She had spent the past 51 days climbing Mount Everest, the tallest mountain in the world, and had finally reached the summit. “I was very anxious to get off and get back down,” she says, adding that the summit is about the…