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Objective: Optic nerve sheath enhancement on magnetic resonance imaging has been reported in patients with giant cell arteritis (GCA), with or without visual manifestations. Whether similar findings can be documented on ultrasound is unknown. Optic nerve ultrasound is a point‐of‐care, easy to learn, rapid, and noninvasive technique. This study aims to investigate whether optic nerve sheath diameter (ONSD) measured on ultrasound is useful in the diagnosis of active, new‐onset GCA. Methods: A single‐center, diagnostic accuracy study was performed from June to November 2022 on consecutive eligible patients referred for suspected GCA. Optic nerve ultrasound was performed on both eyes. The ONSD (includes the optic nerve and its sheath) and optic nerve diameter (OND) were measured 3 mm behind the ocular globe. The presence or absence of GCA was confirmed clinically 6 months later. Multivariable linear regression, adjusting for age and sex, was used to determine the association between optic nerve ultrasound measures and final GCA diagnosis. Results: Thirty participants were enrolled, including nine participants with a final diagnosis of GCA. Mean ± SD ONSD was 5.98 ± 1.17 mm in patients with GCA and 4.02 ± 0.99 mm in patients without GCA. Mean ONSD was greater by 1.26 mm in patients with GCA (95% confidence interval 0.30–2.21 mm, P = 0.01) compared with those without GCA, adjusting for age and sex. Mean ± SD OND was 2.97 ± 0.46 mm in patients with GCA and 2.47 ± 0.58 mm in patients without GCA. There was no evidence of an association between GCA diagnosis and OND. Conclusion: Patients with GCA had a significantly greater ONSD on ultrasound than patients without GCA. Optic nerve ultrasound may represent a novel, rapid, bedside diagnostic test for GCA. A large prospective study is required to confirm these findings and evaluate whether ONSD can be used as a disease activity biomarker in GCA. [ABSTRACT FROM AUTHOR]

Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener’s granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg - Strauss syndrome). Renal-limited AAV can be considered a fourth entity. Despite their rarity and still unknown cause(s), research into AAV has been very active over the past decades and has allowed for the development of new therapeutic regimens. The pathogenesis is a complex process of immune dysregulations with genetic and environmental influences. Recent genome-wide association studies have identified multiple genetic predisposing variants, especially at the major histocompatibility complex region. The pathogenic role of antimyeloperoxidase ANCA (MPO-ANCA) is well supported by several animal models, but that of antiproteinase 3 ANCA (PR3-ANCA) is not as strongly demonstrated. B cells likely play a major role in the pathogenesis because they produce ANCAs, as do neutrophil abnormalities, imbalances in T-cell subtypes, and/or cytokine - chemokine networks. The role of the alternative complement pathway was established more recently, and studies of the antagonist of human C5a receptor (avacopan) in AAV have just been completed, with promising results. The current standard management of severe AAV still consists of remission induction therapy with glucocorticoids combined with rituximab or, less often now, cyclophosphamide. Several studies showed that reduced-dose regimens of glucocorticoids are noninferior to the previously used heavier regimens, for therefore less cumulative exposure to glucocorticoids. Avacopan use may even lead to new steroid-free therapeutic approaches, at least for some selected patients. Several trials and studies have now shown the superiority of rituximab over azathioprine or methotrexate as maintenance therapy. However, the optimal dosing regimen and duration for maintenance remain to be better defined, at the individual patient level. Many changes have occurred in the standard of care for AAV over the past decades, and more are expected soon, including with use of avacopan, but also, likely, a few other agents under investigation or development [ABSTRACT FROM AUTHOR]

Objectives This study describes the spectrum and initial impact of pulmonary manifestations in the primary systemic vasculitides. Methods Description and comparison of pulmonary manifestations in adults with Takayasu's arteritis (TAK), GCA, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic GPA (EGPA), polyarteritis nodosa (PAN) and IgA vasculitis (IgAV), using data collected within the Diagnostic and Classification Criteria in Vasculitis study. Results Data from 1952 patients with primary vasculitides were included: 170 TAK, 657 GCA, 555 GPA, 223 MPA, 146 EGPA, 153 IgAV and 48 PAN. Pulmonary manifestations were observed in patients with TAK (21.8%), GCA (15.8%), GPA (64.5%), MPA (65.9%), EGPA (89.0%), PAN (27.1%) and IgAV (5.9%). Dyspnoea occurred in patients with TAK (14.7%), GCA (7.8%), GPA (41.8%), MPA (43.5%), EGPA (65.8%), PAN (18.8%) and IgAV (2.6%). Cough was reported in TAK (7.6%), GCA (9.3%), GPA (34.8%), MPA (37.7%), EGPA (55.5%), PAN (16.7%) and IgAV (3.3%). Haemoptysis occurred mainly in patients with ANCA-associated vasculitis (AAV). Fibrosis on imaging at diagnosis was documented in GPA (1.9%), MPA (24.9%) and EGPA (6.3%). Only patients with AAV (GPA 2.7%, MPA 2.7% and EGPA 3.4%) required mechanical ventilation. At 6 months, the presence of at least one pulmonary item in the Vasculitis Damage Index was observed in TAK (4.1%), GCA (3.3%), GPA (15.4%), MPA (28.7%), EGPA (52.7%), PAN (6.2%) and IgAV (1.3%). Conclusion Pulmonary manifestations can occur in all primary systemic vasculitides, but are more frequent and more often associated with permanent damage in AAV. [ABSTRACT FROM AUTHOR]

Contexte : La simulation est une méthode d'enseignement utilisée dans plusieurs programmes de médecine ; cependant, il existe peu d'études standardisées portant sur l'utilisation de la simulation dans l'évaluation de la progression des apprentissages cliniques. Objectifs : Comparer la progression des étudiants dans l'apprentissage de l'auscultation cardio-pulmonaire avec un test de type questions à choix multiples (QCM) composé de la reconnaissance de sons cardio-pulmonaires simulés isolés, ou de ces mêmes sons intégrés dans des vignettes cliniques. Méthodes : Un examen QCM est composé en utilisant 20 sons d'auscultation du simulateur cardio-pulmonaire Harvey® et du ventriloscope Lescat®. L'examen est divisé en deux parties : en première partie, 20 sons cardio-pulmonaires doivent être identifiés dans une liste de 14 auscultations cardiaques et 12 bruits pulmonaires ; en deuxième partie, ces mêmes 20 auscultations sont intégrées à des vignettes cliniques et les étudiants doivent choisir parmi 5 décisions cliniques pour chaque situation. Les scores totaux et individuels de chaque partie sont comparés pour chaque groupe d'étudiants faisant le même examen deux années consécutives. Résultats : 1 063 étudiants ont participé au même examen en 2011 et en 2012. Le coefficient alpha Cronbach est de 0,68, 0,44, et 0,79 pour l'examen entier, la première partie et la deuxième partie respectivement. Les étudiants de la première année montrent une progression pour le score total, ainsi que pour le score de la première et deuxième partie de l'examen (p < 0,001). Les étudiants de deuxième et de troisième année montrent une légère progression pour le score total (p < 0,05). La progression dans la reconnaissance isolée des sons simulés n'est pas significative ; en revanche pour ces étudiants, une progression significative est observée quand les sons sont associés à un contexte clinique (p < 0,001). Conclusion : Intégrer des sons simulés d'une auscultation cardio-pulmonaire à des vignettes cliniques semble permettre de mieux mettre en évidence la progression des étudiants dans leur capacité de poser un diagnostic clinique par rapport à l'identification simple des sons. [ABSTRACT FROM AUTHOR]

Objectives: This study describes the spectrum and initial impact of pulmonary manifestations in the primary systemic vasculitides., Methods: Description and comparison of pulmonary manifestations in adults with Takayasu's arteritis (TAK), GCA, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic GPA (EGPA), polyarteritis nodosa (PAN) and IgA vasculitis (IgAV), using data collected within the Diagnostic and Classification Criteria in Vasculitis study., Results: Data from 1952 patients with primary vasculitides were included: 170 TAK, 657 GCA, 555 GPA, 223 MPA, 146 EGPA, 153 IgAV and 48 PAN. Pulmonary manifestations were observed in patients with TAK (21.8%), GCA (15.8%), GPA (64.5%), MPA (65.9%), EGPA (89.0%), PAN (27.1%) and IgAV (5.9%). Dyspnoea occurred in patients with TAK (14.7%), GCA (7.8%), GPA (41.8%), MPA (43.5%), EGPA (65.8%), PAN (18.8%) and IgAV (2.6%). Cough was reported in TAK (7.6%), GCA (9.3%), GPA (34.8%), MPA (37.7%), EGPA (55.5%), PAN (16.7%) and IgAV (3.3%). Haemoptysis occurred mainly in patients with ANCA-associated vasculitis (AAV). Fibrosis on imaging at diagnosis was documented in GPA (1.9%), MPA (24.9%) and EGPA (6.3%). Only patients with AAV (GPA 2.7%, MPA 2.7% and EGPA 3.4%) required mechanical ventilation. At 6 months, the presence of at least one pulmonary item in the Vasculitis Damage Index was observed in TAK (4.1%), GCA (3.3%), GPA (15.4%), MPA (28.7%), EGPA (52.7%), PAN (6.2%) and IgAV (1.3%)., Conclusion: Pulmonary manifestations can occur in all primary systemic vasculitides, but are more frequent and more often associated with permanent damage in AAV., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)