GCA is the most common form of primary systemic vasculitis affecting older people. It is considered a clinical emergency because it can lead to irreversible blindness in around 20% of untreated cases. High doses of glucocorticoids should be initiated promptly to prevent disease-related complications; however, glucocorticoids therapy usually results in significant toxicity. Therefore, correct diagnosis is crucial. For many years, temporal artery biopsy has been considered the diagnostic ‘gold standard’ for GCA, but it has many limitations (including low sensitivity). US has proven to be effective for diagnosing GCA and can reliably replace temporal artery biopsy in particular clinical settings. In cases of suspected GCA with large-vessel involvement, other imaging modalities can be used for diagnosis (e.g. CT and PET). Here we review the current evidence for each diagnostic modality and propose an algorithm to diagnose cranial-GCA in a setting with rapid access to high quality US.

Purpose of Review To provide an updated review on epidemiology, clinical manifestations, diagnostic assessment, treatment, and prognosis of localized vasculitis, following the 2012 Revised International Chapel Hill Consensus Conference Nomenclature on single-organ vasculitis. Recent Findings Localized, single-organ vasculitides encompass a group of rare conditions in which there is no evidence of concomitant systemic vasculitis. Most data on this topic derives from case reports and small case series. Although some aspects of these diseases, such as clinical manifestations and histologic findings, have already been extensively investigated, there is still a lack of robust data concerning the pathogenesis, epidemiology, and treatment. Summary Localized vasculitides may have a wide range of clinical features depending on the organ affected. The inflammatory process may have a multifocal/diffuse or unifocal distribution. Diagnosis is usually based on histopathology findings and exclusion of systemic vasculitis, which may frequently pose a challenge. Further research on treatment is warranted. Keywords Single-organ vasculitis . Cutaneous leukocytoclastic angiitis . Cutaneous arteritis . Isolated aortitis This article is part of the Topical Collection on Vasculitis

In the past decade, unprecedented progress has been made in understanding the pathogenesis, diagnosis, assessment, and treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs). International collaborations and input from several fields (e.g. immunology, rheumatology, and nephrology) have been critical for analyzing demographics, disease manifestations, and outcomes in clinical research studies. Such efforts opened new avenues for generating novel questions and rationale to design better clinical trials. In addition, clinical research has been a source of several biological discoveries and the starting point for knowledge seeking on the pathophysiology of AAV. Interestingly, the blending of clinical and basic research provides a platform for personalized medicine. Despite recent revisions on AAV classification, the incorporation of new findings on disease genetics and immunologic responses may soon result in changes in clinical practice. These advances will enhance the selection of more specific and targeted therapies. However, current unmet needs in the management of AAV are still sizable and heavily impact long-term survival. Especially, frequent relapses, damage accrual, and high morbidity contribute to poor outcomes. Finally, the lack of defined biomarkers for disease activity and the prognosis is a permanent challenge in AAV research. Our work provides an overview of the current state of the art in AAV literature and suggests bridges for the remaining knowledge gaps. It offers potential future directions for the clinical assessment, management, and research in the field toward a more personalized medicine approach.

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