Distinctive clinical features in biopsy-proven nerve large-arteriole vasculitis and microvasculitis.

Vasculitic neuropathy is caused by inflammatory destruction of nerve blood vessels resulting in nerve ischemia. Nerve vasculitis can be divided into two categories based on vessel size - large arteriole vasculitis (≥75 µm) and microvasculitis (<75 µm). Herein, we characterize the clinical features of nerve large-arteriole vasculitis compared to nerve microvasculitis.

Expert Perspective: Management of Relapses in Giant Cell Arteritis.

Giant cell arteritis (GCA) is a relapsing large-vessel vasculitis with risk of serious ischemic manifestations including vision loss and vascular damage in the form of large-artery stenosis, aneurysms and dissections. Approximately 50% of patients treated with glucocorticoid (GC) monotherapy and 30% of patients receiving adjunctive therapy with tocilizumab experience disease relapses, often during the first 2 years after diagnosis. Although most relapses in GCA do not involve life- or organ-threatening presentations and can be controlled successfully, frequent relapses may lead to increased use of GC and consequent treatment-related morbidity, in addition to risk of further vascular damage.

Treatment trends of systemic lupus erythematosus from 2007 to 2023 in the USA.

Objective: To characterise the changing trends in the pharmacological management of SLE in the USA between 2007 and 2023 as new treatment options emerged.

Methods: In a retrospective cohort study using data from OptumLabs Data Warehouse, we characterised the annual prevalent (ie, all) and incident (ie, new) use of antimalarials, glucocorticoids and immunosuppressive medications among patients with SLE from 2007 to 2023 and assessed for changing trends over time.

Results: We identified 19 122 adults with SLE; they were 51.

Vessel wall MRI in giant cell arteritis: standardized protocol and scoring approach developed by an international working group.

Objectives: There are an increasing number of centers performing research on high-resolution vessel wall magnetic resonance imaging (VW-MRI) in giant cell arteritis (GCA). However, harmonized approaches to VW-MRI in GCA are lacking and are essential to performing multicentre studies. Using a data-driven, consensus-based approach, an international expert group developed a standardized MRI protocol and scoring system to advance multi-centered research in cranial GCA.

Etiologies and Outcomes of Granulomatosis With Polyangiitis-Associated Optic Neuropathy: A Case Series and Review of the Literature.

Background: Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare autoimmune disease characterized by inflammation of small- to medium-sized blood vessels (vasculitis). We described the 3 causes of GPA-associated optic neuropathy (compressive, inflammatory, or ischemic) and analyzed initial and final visual acuities (VAs) in each group, which could potentially help prognosticate visual outcomes depending on the etiology of optic neuropathy.

Methods: This was a retrospective chart review of patients who were diagnosed with GPA-associated optic neuropathy and were seen in the Department of Ophthalmology at Mayo Clinic in Rochester, Minnesota.

Treatment of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis With Diffuse Alveolar Hemorrhage With Avacopan.

Objective: Avacopan, an activated complement factor 5 receptor antagonist, has been approved as adjunct therapy for severe active antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Current evidence supports the management of AAV presenting with diffuse alveolar hemorrhage (DAH) by administering glucocorticoids combined with either rituximab or cyclophosphamide in addition to supportive care. The role of avacopan in patients with DAH as a primary severe disease manifestation of AAV has not been well established.

Subclinical Aortic Inflammation in Patients with Polymyalgia Rheumatica.

Objectives: To examine the clinicopathologic features of patients with polymyalgia rheumatica (PMR) who had thoracic aorta repair surgery. Findings were compared with those of a cohort of patients with giant cell arteritis (GCA) requiring thoracic aorta repair.

Methods: All patients evaluated at Mayo Clinic in Rochester, MN, with Current Procedural Terminology (CPT) codes for thoracic aorta repair surgery between 2000- 2021 were identified.

Current management of giant cell arteritis and its complications.

Purpose Of Review: This review provides an update on current management strategies for giant cell arteritis (GCA), emphasizing the need for alternative therapies to reduce disease relapses and mitigate glucocorticoid (GC)-related morbidity.

Recent Findings: The standard of care for GCA has traditionally involved prolonged use of GC, and recent studies are exploring faster GC tapering regimens in an effort to reduce adverse effects while maintaining disease control. Randomized clinical trials have highlighted the efficacy of tocilizumab (TCZ), an interleukin-6 receptor inhibitor, in reducing disease flares and sparing GCs.

Imaging Findings in Giant Cell Arteritis: Don't Turn a Blind Eye to the Obvious!

Giant cell arteritis (GCA) is the most common primary large vessel systemic vasculitis in the Western World. Even though the involvement of scalp and intracranial vessels has received much attention in the neuroradiology literature, GCA, being a systemic vasculitis, can involve multiple other larger vessels including the aorta and its major head and neck branches. Herein, the authors present a pictorial review of the various cranial, extracranial, and orbital manifestations of GCA.

Assessment of Damage in Takayasu's Arteritis.

Objectives: To evaluate damage and clinical characteristics associated with damage in Takayasu's arteritis (TAK).

Methods: Patients with TAK enrolled in a multicentre, prospective, observational study underwent standardized damage assessment every 6 months using the Vasculitis Damage Index (VDI) and the Large-Vessel Vasculitis Index of Damage (LVVID).

Results: The study included 236 patients with TAK: 92% female, 81% Caucasian; median (25th, 75th percentile) disease duration = 2.

Predictive Factors of Renal Recovery and Progression to End-Stage Kidney Disease in Patients With Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis With Severe Kidney Disease.

Introduction: A significant number of patients with antineutrophil cytoplasmic antibodies (ANCA)- associated vasculitis (AAV) with glomerulonephritis (AAV-GN) still progress to end-stage kidney disease (ESKD, estimated glomerular filtration rate [eGFR] <15 ml/min per 1.73 m) despite advances in remission-induction treatment.

Methods: This is a retrospective cohort study on myeloperoxidase (MPO)-ANCA or proteinase 3 (PR3)-ANCA positive patients with AAV (microscopic polyangiitis, MPA; or granulomatosis with polyangiitis, GPA) and eGFR <15 ml/min per 1.

Giant cell arteritis in patients with systemic sclerosis: a case series.

Objectives: Giant cell arteritis (GCA) in patients with systemic sclerosis (SSc) is rare, and optimal treatment strategies for this group of patients have not been defined. We aim to describe the first case series of GCA/SSc overlap.

Methods: A single-institution retrospective study was performed reviewing all patients that had diagnosis codes for both SSc and GCA between January 1, 1996, and December 31, 2020.

Giant cell arteritis associated with intravenous zoledronic acid administration.

Bisphosphonates frequently provoke a cytokine-driven acute clinical response (ACR) characterized by fever, chills, arthralgias, and myalgias. More rarely, an association between aminobisphosphonates, such as alendronate and zoledronic acid, and rheumatologic and/or immune-mediated syndromes (RIMS) has been described. Herein we report 2 patients, one with a prior history of rheumatic disease and one without, who developed giant cell arteritis meeting the American College of Rheumatology 2022 criteria following zoledronic acid infusion.

Comprehensive morphologic characterization of bone marrow biopsy findings in a large cohort of patients with VEXAS syndrome: A single-institution longitudinal study of 111 bone marrow samples from 52 patients.

Objectives: VEXAS syndrome is an adult-onset autoinflammatory disease caused by a somatic pathogenic mutation in the UBA1 (ubiquitin-like modifier activating enzyme 1) gene. Patients present with rheumatologic manifestations and cytopenias and may have an increased predisposition to myelodysplastic syndrome (MDS) and plasma cell neoplasms. Prior studies have reported on the peripheral blood and bone marrow findings in patients with VEXAS syndrome.

Association between sinusitis and incident rheumatic diseases: a population-based study.

Objectives: To determine whether antecedent sinusitis is associated with incident rheumatic disease.

Methods: This population-based case-control study included all individuals meeting classification criteria for rheumatic diseases between 1995 and 2014. We matched three controls to each case on age, sex and length of prior electronic health record history.

Reappraisal of large artery involvement in giant cell arteritis: a population-based cohort over 70 years.

Objective: To evaluate the incidence and outcomes of large artery (LA) involvement among patients with giant cell arteritis (GCA) and to compare LA involvement to non-GCA patients.

Methods: The study included Olmsted County, Minnesota, USA residents with incident GCA between 1950 and 2016 with follow-up through 31 December 2020, death or migration. A population-based age-matched/sex-matched comparator cohort without GCA was assembled.

Associations between plasma metabolism associated proteins and future development of giant cell arteritis: results from a prospective study.

Objective: To investigate the relation between biomarkers associated with metabolism and subsequent development of giant cell arteritis (GCA).

Method: Participants in the population-based Malmö Diet Cancer Study (MDCS; N = 30447), who were subsequently diagnosed with GCA, were identified in a structured process. Matched GCA-free controls were selected from the study cohort.