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Vasculitis

Untangling the Diagnosis & Management of GCA

Jason Liebowitz, MD, FACR  |  June 28, 2021

Vision loss, implications of treatment and more—Sarah Mackie, BMBCh, PhD, MRCP, discussed the latest research on the evaluation and management of giant cell arteritis.

Case Report: Child Develops Coronary Artery Aneurysms with GPA

Tryphina Adel Mikhail & Mary Bratovich Toth, MD  |  June 14, 2021

In September 2019, a previously healthy, 9-year-old white girl presented to the emergency department following two months of sinusitis and unexplained fever responsive to ibuprofen. She presented with anorexia; a 9 lb. weight loss; intermittent, nonbilious, nonbloody emesis; and occasional epistaxis with digital manipulation of the nose. Six weeks prior to admission, she had presented to…

7 Key Insights Into the Evaluation of Central Nervous System Vasculitis

Jason Liebowitz, MD, FACR  |  May 13, 2021

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…

Case Report: Warfarin-Induced Non-Uremic Calciphylaxis Mimicking Vasculitis

Marie Dardeno, DO, Joshua D. Sparling, MD, & William Monaco, MD  |  May 13, 2021

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…

Study: Can Avacopan Replace Steroids in ANCA-Associated Vasculitis?

Ruth Jessen Hickman, MD  |  May 13, 2021

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…

Alisusha; Trueffelpix / shutterstock.com

3 New Vasculitis Guidelines Discussed

Ruth Jessen Hickman, MD  |  May 13, 2021

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…

Case Report: A Patient with Submandibular Swelling

Julia A. Ford, MD, Robert Padera, MD, PhD, & Lindsey A. MacFarlane, MD, MPH  |  March 15, 2021

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…

Case Report: Drug-Induced Lupus & ANCA-Associated Vasculitis Overlap

Mohammad A. Ursani, MD, RhMSUS, Ojas Naik, MD, Rohaan Khan & William F. Glass II, MD, PhD  |  February 16, 2021

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)…

Case Report: Hydralazine-Induced ANCA-Associated Vasculitis

William J. Scheuing, MD, Nitasha Kumar, MD, William Davis, MD, & Robert Quinet, MD  |  February 16, 2021

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…

ilusmedical / shutterstock.com

The State of Clinical Research in Vasculitis: 2021

Shubhasree Banerjee, MD, & Peter A. Merkel, MD, MPH  |  January 19, 2021

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…

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