Many a rheumatology consult has centered on a perplexing question: Does this patient have central nervous system (CNS) vasculitis? At the 2021 ACR State-of-the-Art Clinical Symposium, Rula Hajj-Ali, MD, FACP, professor of medicine and associate director of vasculitis care and research, Cleveland Clinic Lerner College of Medicine, discussed this topic in detail, providing a series…
Calciphylaxis, or calcific uremic arteriolopathy, is a rare disease characterized by calcification of the arterioles and capillaries in the dermis and subcutaneous tissue, resulting in thrombus formation and subsequent skin ischemia and necrosis.1 This serious condition most commonly occurs in patients with end-stage renal disease (ESRD) requiring dialysis or in kidney transplant recipients. In rare…
A phase 3 trial described in The New England Journal of Medicine (NEJM) highlights the potential of a C5a receptor inhibitor, avacopan, for anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis.1 Avacopan may potentially offer a steroid-sparing option for the treatment of this serious disease. Current Treatment of ANCA-Associated Vasculitis Morbidity and mortality from ANCA-associated vasculitis have…
The ACR, in concert with the Vasculitis Foundation, released three new vasculitis guidelines online first in July, with a fourth slated for publication by the end of the year. These are the first ever to be produced and endorsed by the ACR and the Vasculitis Foundation. Although most of the recommendations are conditional, due to…
We report on a 77-year-old patient who was diagnosed with giant cell arteritis (GCA) on histopathology of the submandibular gland and eventually went on to develop classic signs and symptoms of GCA. This observation highlights neck swelling as a rare initial presentation of GCA. Case Presentation A 77-year-old previously healthy man presented to his primary…
Drug-induced lupus erythematosus and ANCA-associated vasculitis (AAV) are both autoimmune conditions associated with the use of hydralazine, a commonly prescribed drug for hypertension and congestive heart failure. Although the pathogenesis is unknown, it is believed that hydralazine alters neutrophil and lymphocyte function and promotes exposure of antigens, leading to the development of anti-neutrophil antibodies (ANCA)…
Hydralazine has been in use as a treatment for hypertension, most notably in heart failure patients, since 1951.1 The drug is a known cause of autoimmune disease, most specifically hydralazine-induced lupus. Hydralazine-induced lupus occurs in 7–13% of those taking the medication.2-4 It often presents with constitutional symptoms, arthritis/arthralgias, cutaneous lesions, serositis, myalgias and/or hepatomegaly. Features…
It is an exciting time in the world of vasculitis research. More clinical studies and trials are being conducted now than at any time in history. In the past ten years, four drugs have been approved by the U.S. Food & Drug Administration (FDA) and other regulatory agencies specifically for the treatment of vasculitis: Rituximab…
The ACR Review Course featured eight talks providing a practical review of issues encountered by rheumatologists.
In this ACR Convergence session, Jason R. Kolfenbach, MD, & John H. Stone, MD, MPH, shared clinical insights about rheumatic diseases.