Polyarteritis nodosa (PAN) is a necrotizing vasculitis, predominantly involving medium-sized arteries, that causes systemic disease, and, less commonly, cutaneous-limited disease. The population prevalence for PAN ranges from 2 to 33 per million.1-3 Estimates vary due to the increased recognition and classification of other forms of vasculitides over time and variation in the regional prevalence of…
In 2021, the ACR—in concert with the Vasculitis Foundation (VF)—released four new vasculitis guidelines, one each on: 1) anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis, 2) giant cell arteritis (GCA) and Takayasu arteritis, 3) polyarteritis nodosa and 4) Kawasaki disease. The guideline development process is complex. For the vasculitis guidelines, this process kicked off in June…
Brendan Antiochos, MD, provided a timely update on research and treatments related to vasculitis, addressing treatments for ANCA-associated vasculitis and giant cell arteritis, COVID-19 vaccination and more.
In this EULAR 2022 session, new & revised treatment recommendations for ANCA-associated vasculitis, axial spondyloarthritis & rheumatoid arthritis were presented.
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.