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Purpose of Review: The aim of this article was to review the recent contributions on the role of PET in assessing disease activity in patients with large-vessel vasculitis (giant cell arteritis and Takayasu arteritis)., Recent Findings: 18 FDG (fluorodeoxyglucose) vascular uptake in large-vessel vasculitis at PET shows moderate correlation with clinical indices, laboratory markers and signs of arterial involvement at morphological imaging. Limited data may suggest that 18 FDG (fluorodeoxyglucose) vascular uptake could predict relapses and (in Takayasu arteritis) the development of new angiographic vascular lesions. PET appears to be in general sensitive to change after treatment., Summary: While the role of PET in diagnosis large-vessel vasculitis is established, its role in evaluating disease activity is less clear-cut. PET may be used as an ancillary technique, but a comprehensive assessment, including clinical, laboratory and morphological imaging is still required to monitor patients with large-vessel vasculitis over time., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose of Review: Myositis, or idiopathic inflammatory myopathy, is an overarching concept that includes dermatomyositis, polymyositis, immune-mediated necrotizing myopathy and the antisynthetase syndrome. Glucocorticoids are still considered the mainstay of treatment of myositis but some patients require add-on immunosuppressive therapy because of insufficient response to glucocorticoids, relapses when glucocorticoids are tapered, or because they incur glucocorticoid-related side effects., Recent Findings: The goal of this article was to review (PubMed search from January 2019 through June 2020) the efficacy and safety of standard and novel agents used in adult dermatomyositis, polymyositis, immune-mediated necrotizing myopathy and the antisynthetase syndrome., Summary: Established therapies beyond glucocorticoids continue to have a major role in managing patients with myositis. In addition, novel agents are being tried for refractory manifestations of myositis.

Purpose of Review: Glucocorticoids are the mainstay of therapy for large-vessel vasculitis, but potential toxicity and frequent relapses led to studies with nonbiologic and biologic glucocorticoid-sparing agents. The aim of this review is to discuss the recent evidence for the management of giant cell arteritis (GCA) and Takayasu arteritis (TAK)., Recent Findings: Tocilizumab proved to be a powerful glucocorticoid-sparing agent for GCA in a randomized placebo-controlled trial, whereas the trials with tocilizumab and abatacept failed to show a significant difference from placebo in relapse-free survival rate in TAK. Further trials are awaiting for establishing the role of abatacept and ustekinumab for GCA, and rituximab and tumor necrosis factor inhibitors, including certolizumab for TAK, as well as nonbiologic agents for both indications., Summary: Despite recent randomized controlled trials with biologic agents, management of large-vessel vasculitis largely depends on observational studies. Well designed controlled trials using validated outcome measures in large number of patients, identification of biologic markers that could guide the choice of targeted treatments, and standardization of disease assessment including imaging modalities are unmet needs for the management of large-vessel vasculitis.

Purpose of Review: The aim of this article was to review the recent contributions to the scoring methods of PET in vasculitis as well as to its role in the diagnostic work-up., Recent Findings: Both visual and semiquantitative scoring methods can be used to interpret PET scans. PET has been shown to be both sensitive and specific in the diagnosis of large-vessel vasculitis. In addition, it also has a role in predicting vascular complications., Summary: There is a need to better standardize the scoring methods used to interpret PET scans. In clinical practice, PET is useful to diagnose untreated individuals with suspected large-vessel vasculitis and contributes to identify patients at risk for vascular complications.

Purpose of Review: This article summarizes the current evidence on the management of primary and secondary central nervous system (CNS) vasculitis., Recent Findings: Two recent retrospective cohort studies have described the treatment and outcomes of patients with adult primary central nervous system vasculitis (PCNSV). Although the majority of the patients (>60%) responded to therapy with glucocorticoids alone or in conjunction with cyclophosphamide (CYC) and tended to improve during the follow-up period, an overall increased mortality was observed. The treatment response and the outcomes appeared primarily related to the size of the vessels involved in the inflammatory process. The involvement of small cortical/leptomeningeal vessels was associated with a more benign course, whereas the involvement of larger/proximal cerebral vessels was related to a less favorable prognosis and identified cases that should be treated more aggressively. Glucocorticoids combined with CYC are the mainstay of therapy for secondary CNS vasculitis. Observational studies have documented the efficacy of rituximab as induction therapy in patients with antineutrophil cytoplasm antibody-associated vasculitis (AAV) and CNS disease, while the role of antitumor necrosis factor (TNF) agents is more controversial. Case series have demonstrated the efficacy of anti-TNF agents in patients with neuro-Behçet's disease. Tocilizumab may also be effective in this condition., Summary: Recognition of findings at diagnosis that predict the course or outcomes of PCNSV may serve as guide for therapy. Biological agents may provide benefit to difficult-to-treat patients with CNS involvement secondary to AAV and Behçet's disease.

Purpose of Review: Chronic periaortitis is characterized by a fibro-inflammatory process spreading from the abdominal aorta and the iliac arteries. Originally, chronic periaortitis was considered a localized inflammatory response to severe aortic atherosclerosis. However, subsequent studies have shown that chronic periaortitis may also involve other arteries and present with features of auto-immune diseases. This article reviews the issue of large-vessel involvement in chronic periaortitis and its implications in the pathogenesis and nosography of the disease., Recent Findings: In many reports, chronic periaortitis has been shown to involve not only the aorto-iliac axis but also other vascular segments such as the thoracic aorta, the proximal epiaortic arteries, the coronary, renal, and mesenteric arteries. Thoracic aorta involvement may manifest as thoracic periaortitis with or without aneurysmal dilatation, or simply as thoracic aorta aneurysm. Thoracic periaortitis can also be a feature of the so-called IgG4-related systemic disease, with which chronic periaortitis may sometimes be associated. Histopathologic studies of chronic periaortitis show adventitial inflammation and fibrosis, vasculitis of vasa vasorum, and adventitial lymphoid follicles with germinal centers, suggesting that chronic periaortitis could be a primary aortitis. Genetic studies have demonstrated an association with HLA-DRB1*03, a marker of auto-immunity, and with the CCR5Δ32 polymorphism, which has been mapped to a Th2 response. Taken together, these findings support the notion of a primary inflammatory or immune-mediated disorder., Summary: Chronic periaortitis is an inflammatory or immune-mediated disorder characterized histopathologically by adventitial inflammation and clinically by variable involvement of different arteries, mainly of large caliber. These findings raise the issue of whether chronic periaortitis should be considered a large-vessel vasculitis.

Purpose of Review: Glucocorticoids remain the mainstay of treatment of giant cell arteritis. The aim of this review is to establish the optimal schedule of glucocorticoid administration, and to ascertain which other treatments may be used as glucocorticoid-sparing agents., Recent Findings: An initial dose of 40-60 mg/day of prednisone is usually adequate. Patients at risk of developing ischemic complications require dosages of around 1 mg/kg/day, whereas pulse glucocorticoid therapy is no more effective in preventing ischemic complications. In patients with longstanding disease or those at risk for glucocorticoid-related adverse events, methotrexate or azathioprine can be used as glucocorticoid-sparing drugs. Infliximab has been demonstrated to be efficacious in glucocorticoid-resistant disease in an open study, whereas a randomized controlled trial showed no efficacy in patients with recent-onset disease. Finally, two retrospective studies suggest that low-dose aspirin may decrease the rate of cranial ischemic complications secondary to giant cell arteritis., Summary: Glucocorticoids remain the cornerstone of therapy for giant cell arteritis. To achieve maximal efficacy but minimize glucocorticoid-related adverse reactions, dosage should be individually tailored. In patients with longstanding, recalcitrant disease, methotrexate, azathioprine or tumor necrosis factor-alpha inhibitors may be considered. Aspirin is recommended in all patients unless contraindicated. Osteoporosis prophylaxis should also be regularly implemented.