Primary central nervous system vasculitis with intracranial aneurysm.

Background: Unruptured intracranial aneurysms (UIAs) are rarely reported in primary central nervous system vasculitis (PCNSV). In this study we described the clinical findings, response to therapy, and outcomes of UIA in a large cohort of PCNSV patients.

Methods: We retrospectively studied 216 consecutive patients with PCNSV, selected by predetermined diagnostic criteria, who were seen during a 40-year period.

Unilateral Relapsing Primary CNS Vasculitis: Description of 3 Cases From a Single-Institutional Cohort of 216 Cases.

Objectives: To define the frequency and characteristics of patients with unilateral relapsing involvement in a cohort of patients with adult primary CNS vasculitis (PCNSV).

Methods: We retrospectively studied a cohort of 216 patients with PCNSV seen at the Mayo Clinic, Rochester, MN from 1983 to 2022. Twenty-five patients (19.

Takayasu arteritis and large-vessel giant cell arteritis in Italian population. Comprehensive analysis from a single institutional cohort of 184 cases.

Objective: To compare clinical and imaging characteristics in patients with Takayasu arteritis (TAK) and large vessel-giant cell arteritis (LV-GCA) in an Italian population.

Methods: We conducted a retrospective monocenter study comparing characteristics and outcomes of a cohort of 59 patients with TAK and a cohort of 127 patients with LV-GCA diagnosed between 1996 and 2016. Most of them (92%) were followed up for at least 24 months at Reggio Emilia Hospital (Italy).

Exploring Gene Expression Profiles in Primary Central Nervous System Vasculitis.

Objective: This study was undertaken to explore the gene expression profile of primary central nervous system vasculitis (PCNSV).

Methods: Brain specimens of 4 patients with granulomatous vasculitis (GV), 5 with lymphocytic vasculitis (LV), 4 with amyloid β-related angiitis (ABRA), and 4 normal controls were studied. RNA-sequencing was performed using the Illumina Hiseq-4,000 platform and the Illumina TruSeq Total-RNA library.

Long-term remission, relapses and maintenance therapy in adult primary central nervous system vasculitis: A single-center 35-year experience.

Objectives: To evaluate long-term treatment and outcomes of patients with primary central nervous system vasculitis (PCNSV).

Methods: In this cohort of 191 consecutive patients with PCNSV seen at Mayo Clinic, Rochester, MN, over 35 years with long-term follow-up we analyzed response to and duration of therapy, frequency of relapses, long-term remission, efficacy of maintenance therapy and initial intravenous glucocorticoid (GC) pulses, survival and degree of disability. We also compared the efficacy of initial IV and oral cyclophosphamide (CYC).

Cortisone in Popular Culture: Roueché, Ray, and Hench.

In this article, the authors offer a new perspective on how the administration of Compound E (ie, cortisone) to a volunteer Mayo Clinic patient with rheumatoid arthritis and the patient's subsequent miraculous improvement led not only to a major, successful clinical trial but also a Nobel Prize. The early and late side effects as an undesirable outcome of treatment of corticosteroids would soon follow. Corticosteroid side effects became known in popular culture, first through an indepth article in by medical journalist Berton Roueché, and later through a major fiction film, , directed by Nicholas Ray.

Rituximab therapy for primary central nervous system vasculitis: A 6 patient experience and review of the literature.

Objectives: To assess the efficacy and safety of Rituximab (RTX) in adult primary central nervous system vasculitis (PCNSV).

Methods: We retrospectively assessed the effect of RTX in 6 patients with PCNSV. Five of the 6 were refractory to high dose glucocorticoids (GCs) and/or conventional immunosuppressants (IS).

Primary central nervous system vasculitis mimicking brain tumor: Comprehensive analysis of 13 cases from a single institutional cohort of 191 cases.

Objective: To describe the clinical, laboratory, and imaging features and course of patients with primary central nervous system vasculitis (PCNSV) presenting with an intracranial tumor-like mass (TLM).

Methods: We retrospectively studied a cohort of 191 consecutive patients with PCNSV seen at the Mayo Clinic, Rochester, MN over a 35-year period (1982-2017). 13/191 patients presented with a TLM.

Primary central nervous system vasculitis associated with lymphoma.

Objectives: To record the clinical findings, response to therapy, and course of patients with primary CNS vasculitis (PCNSV) associated with lymphoma.

Patients And Methods: We reviewed the histories of 936 patients with a diagnosis of any type of vasculitis and lymphoma who were seen at the Mayo Clinic over a 32-year period. Ten patients with both PCNSV and lymphoma were identified.

Clinical and pathological evolution of giant cell arteritis: a prospective study of follow-up temporal artery biopsies in 40 treated patients.

Although clinical signs and symptoms of giant cell arteritis improve promptly after starting glucocorticoid therapy, reports have suggested that the vascular inflammation may persist. To assess the duration and quality of histopathologic changes in treated patients, we prospectively obtained second temporal artery biopsies in patients treated for 3 to 12 months after their first diagnostic biopsy. Forty patients (28 women, 12 men, median age 77 years) agreed to have a second temporal artery biopsy randomly assigned to 3, 6, 9, or 12 months subsequent to the first.

Predictors of Dissection in Aortic Aneurysms From Giant Cell Arteritis.

Background: Factors associated with dissection from inflammatory aortic aneurysms may be different from those in the general population.

Objective: The aim of this study was to evaluate the risk factors for aortic dissection/rupture in patients with giant cell arteritis (GCA) and aortic aneurysms.

Methods: A population-based incident cohort of patients with a diagnosis of GCA from 1950 to 2004 was used.

Imaging Findings of Cerebral Amyloid Angiopathy, Aβ-Related Angiitis (ABRA), and Cerebral Amyloid Angiopathy-Related Inflammation: A Single-Institution 25-Year Experience.

Vascular inflammation is present in a subset of patients with cerebral amyloid angiopathy (CAA) and has a major influence in determining the disease manifestations. Radiological characterization of this subset is particularly important to achieve early recognition and treatment. We conducted this study to investigate the role of imaging in differentiating CAA with and without inflammation.

Management of primary and secondary central nervous system vasculitis.

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.

An update of the Mayo Clinic cohort of patients with adult primary central nervous system vasculitis: description of 163 patients.

Primary central nervous system vasculitis (PCNSV) is an uncommon condition in which lesions are limited to vessels of the brain and spinal cord. Because the clinical manifestations are not specific, the diagnosis is often difficult, and permanent disability and death are frequent outcomes. This study is based on a cohort of 163 consecutive patients with PCNSV who were examined at the Mayo Clinic over a 29-year period from 1983 to 2011.

Mycophenolate mofetil in primary central nervous system vasculitis.

Objective: To evaluate the efficacy and safety of mycophenolate mofetil (MMF) in adult primary central nervous system vasculitis (PCNSV).

Methods: We studied a cohort of 163 patients with PCNSV who were seen at the Mayo Clinic from 1983 to 2011. We compared patients treated with MMF and those receiving other therapies.

Adult primary central nervous system vasculitis treatment and course: analysis of one hundred sixty-three patients.

Objective: To describe the treatment and outcomes of patients with primary central nervous system (CNS) vasculitis.

Methods: We retrospectively studied a cohort of 163 consecutive patients with primary CNS vasculitis who were seen at the Mayo Clinic over a 29-year period. We analyzed treatments, treatment responses, and factors predictive of outcomes.

Catastrophic primary central nervous system vasculitis.

Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and the spinal cord. It is heterogeneous in presenting characteristics and outcomes. We report a patient with a catastrophic rapidly progressive course refractory to intensive treatment with pulses of methylprednisolone and iv cyclophosphamide.

Treatment of primary CNS vasculitis with rituximab: case report.

Primary CNS vasculitis (PCNSV) is an uncommon disorder of unknown cause that is restricted to brain and spinal cord. Glucocorticoids alone or in combination with cyclophosphamide achieve a favorable response in most patients.(1,2) However, some patients are intolerant or respond poorly to cyclophosphamide; therefore, there is the need for new treatment options.

Discontinuation of therapies in polymyalgia rheumatica and giant cell arteritis.

Glucocorticoids are highly effective in treating polymyalgia rheumatica and giant cell arteritis, but their use is associated with numerous adverse events. Therefore, it is important to use them for the shortest period of time possible. The published evidence suggests that discontinuation of GC is feasible in a substantial number of patients with polymyalgia rheumatica and giant cell arteritis after an adequate period of treatment, provided that glucocorticoids are tapered gradually.

Aβ-related angiitis: comparison with CAA without inflammation and primary CNS vasculitis.

Objective: To analyze the clinical findings, response to therapy, and outcomes of patients with cerebral vascular amyloid-β (Aβ) deposition with and without inflammatory vascular infiltration.

Methods: We report 78 consecutive patients with cerebral vascular Aβ deposition examined at Mayo Clinic Rochester over 25 years (1987 through 2011). Specimens reviewed by a neuropathologist showed 40 with vascular Aβ peptide without inflammation (cerebral amyloid angiopathy [CAA]), 28 with granulomatous vasculitis (Aβ-related angiitis or ABRA), and 10 with perivascular CAA-related inflammation.