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Vasculitis

Trial Data Reveals the Limitations of Steroids in Giant Cell Arteritis Therapy

Thomas R. Collins  |  September 20, 2018

AMSTERDAM—Just how seldom prednisone is successful at inducing remission in giant cell arteritis (GCA), despite such a long history of use for the disease, is one of the many lessons to emerge from the data in the GiACTA trial, said the principal investigator of the trial, which is the largest ever in GCA and is…

New Study Aimed at Better Predicting Large Vessel Vasculitis Relapse

Elizabeth Hofheinz, MPH, MEd  |  September 20, 2018

Imagine telling a patient “You’re in remission!” and finding out it’s not true. The last thing you want to do is get it wrong clinically and put your patient on an emotional rollercoaster. With large vessel vasculitis (LVV) in particular, physicians struggle to be accurate, to determine if indeed the disease has gone away or…

Advocate for Prompt Vasculitis Diagnosis & Treatment

Carina Stanton  |  August 28, 2018

Rheumatologists can act as advocates for patients and their families in a way that supports targeting vasculitis early. Here’s how…

Study Finds Tocilizumab Could Be Treatment Option for Takayasu Arteritis

Carina Stanton  |  August 17, 2018

For patients with refractory Takayasu arteritis (TAK), glucocorticoids (GCs) are often provided as the initial therapy for treatment. However, GCs are often associated with adverse effects for long-term use; relapse also occurs frequently during GC tapering.1 TAK involves interleukin (IL) 6. Tocilizumab—a recombinant, humanized, anti-IL-6 receptor (IL-6R) monoclonal antibody—was first reported by Nishimoto et al….

Figure 2: Renal Biopsy

The Classification & Diagnosis of Granulomatosis with Polyangiitis

Harry E. Subramanian, Ravi Sutaria, MD, & Fotios Koumpouras, MD  |  August 16, 2018

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 Latest on Eosinophilic Granulomatosis with Polyangiitis

Paul A. Monach, MD, PhD  |  July 19, 2018

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…

Case Report: Ehlers-Danlos Syndrome Mimicking Vasculitis

Case Report: Ehlers-Danlos Syndrome Mimicking Vasculitis

Catherine (Katie) Donnelly, MB, BCh, BAO, & Surabhi Khanna, MD  |  July 19, 2018

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…

Eosinophilia & Hypereosinophilia Diagnosis & Treatment Tips

Thomas R. Collins  |  June 21, 2018

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…

Submassive pulmonary embolism. Filling defects seen in the pulmonary artery.

Case Report Illustrates Hypercoagulability in Behçet’s Disease

Aiza Tariq, MD, & Jasim Alidina, MD  |  June 21, 2018

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…

Figure 1. A nasal biopsy shows intimal infiltration of the small blood vessels (black arrow).

Case Illustrates the Difficulty Diagnosing Granulomatosis with Polyangiitis

Diana Girnita, MD, PhD, & Vishnuteja Devalla, MD  |  June 21, 2018

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…

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