Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophil cytoplasmic antibody-associated vasculitis typically characterized by asthma, peripheral eosinophilia and medium- to small-vessel necrotizing vasculitis. Cutaneous manifestations in EGPA are diverse. Palpable purpura is the most common presentation, but urticaria, erythematous macules and papules, livedo reticularis, digital necrosis and cutaneous nodules have also been described.1 Non-hemorrhagic bullae…
The COVID-19 pandemic has filled hospitals with patients with rapidly progressive respiratory failure and diffuse bilateral opacities on chest X-ray.1 Additionally, many patients with severe COVID-19 develop acute kidney injury and require dialysis.2 Pulmonary-renal syndromes are also important to consider in this setting. Although alveolar hemorrhage is a cardinal feature of this syndrome, many patients…
A 44-year-old woman presented to the emergency department with bifrontal headaches that had started approximately one month earlier. She was diagnosed with migraines and discharged home. Three days later, the patient returned to the emergency department upon recurrence of her headaches, and this time she also reported abnormal leg movements. A computerized tomography (CT) scan…
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects medium-sized muscular arteries. The clinical subsets of PAN are idiopathic, generalized, secondary hepatitis B virus (HBV) associated and cutaneous PAN. These clinical subsets are important because of their therapeutic implications. Virtually any organ system can be affected in generalized PAN, but this vasculitis tends…
With both impressive sensitivity and specificity in capturing active large vessel vasculitis, a positron emission tomography (PET) scan is a potential tool for determining disease activity and predicting relapse in patients with large vessel vasculitis.
Hypereosinophilic syndromes and eosinophilic granulomatosis with polyangiitis (EGPA) represent a spectrum of eosinophilic disorders. Amy D. Klion, MD, provided a detailed overview of this spectrum during ACR Convergence 2021.
Granulomatosis with polyangiitis (GPA), is a type of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) that affects small- to medium-sized vessels.1 It can occur equally in both men and women, with a reported mean age at onset of 55 years.2 The classical presentation of GPA includes upper respiratory tract (i.e., chronic sinusitis, serous otitis and…
A soon-to-be published guideline from the ACR and the Vasculitis Foundation on Kawasaki disease underscores the importance of early diagnosis and intensified treatment for people with this serious condition.1 Intravenous immunoglobulin (IVIG) remains the treatment mainstay, and prompt, aggressive treatment may be able to reduce the risk of serious complications in some patients. The guideline…
During the ACR Convergence 2021 annual Review Course, Peter Merkel, MD, MPH, provided recent updates on vasculitis.
ACR CONVERGENCE 2021—Many of the effects of childhood-onset systemic lupus erythematosus (cSLE) and vasculitis carry into adulthood and present adult rheumatologists with key differences in managing these patients after their transition from a pediatric to an adult provider. “The young adult with childhood-onset lupus is similar in many ways to adults with lupus, but there…