Frequency and characteristics of severe relapse in giant cell arteritis.

Objectives: To assess the frequency and characteristics of severe relapse in patients with giant cell arteritis (GCA) in a real-life setting.

Methods: In a monocentric database of 530 patients, we retrospectively analysed patients who experienced at least one relapse and distinguished severe from nonsevere relapses. Severe relapse was defined by the occurrence of an ischaemic event (ophthalmologic, neurologic, digestive, limb ischaemia), the occurrence of an aortic complication (i.

Efficacy, tolerance and acceptability of pulsed dye laser on facial and neckline telangiectasias in systemic scleroderma: a prospective open-label monocentric study in 21 patients.

Facial and neckline telangiectasias have an underestimated yet important impact on quality of life of patients with systemic scleroderma (SSc). This monocentric, prospective, open-label, intra-patient comparative study was conducted in 21 consecutive patients with SSc. Patients underwent 4 sessions of PDL 8 weeks apart.

Evolution of the Therapeutic Management of Giant Cell Arteritis: Analysis of Real-Life Practices over Two Timeframes (2014-2017 and 2018-2020).

To determine how therapeutic strategies for giant cell arteritis (GCA), especially glucocorticoid (GC) management, evolved between 2014 and 2020. Consecutive GCA patients followed for at least 24 months in a single tertiary center were enrolled and separated into two groups: those diagnosed from 2014 to 2017 and those diagnosed from 2018 to 2020. GC doses (mg/kg/day) were analyzed at onset, at Month 3 (M3) and, if continued, at M6, M12, M18 and M24.

Efficacy and tolerance of methotrexate in a real-life monocentric cohort of patients with giant cell arteritis.

Objectives: To assess the indications, efficiency and tolerance profiles of methotrexate (MTX) in patients with giant cell arteritis (GCA) in a real-life setting.

Methods: From a monocentric database of >500 GCA patients, we retrospectively selected 49 patients who received MTX between 2010 and 2020. Cumulative glucocorticoid (GC) doses, the number of relapses and GC-related adverse events were recorded before, during and after MTX.

Characteristics of ANCA-associated vasculitis with aneurysms: Case series and review of the literature.

Introduction: ANCA-associated vasculitis (AAV) is an exceptional cause of small and large vascular aneurysms. Here, we present the phenotypic characteristics of patients with AAV associated with the presence of aneurysms.

Methods: We conducted a retrospective multicenter study and a systematic review of the literature.

The diagnostic challenge of patients with anti-U1-RNP antibodies.

Anti-U1-RNP antibodies are necessary for the diagnosis of mixed connective tissue disease (MCTD), but they are also prevalent in other connective tissue diseases, especially systemic lupus erythematosus (SLE), from which distinction remains challenging. We aimed to describe the presentation and outcome of patients with anti-U1-RNP antibodies and to identify factors to distinguish MCTD from SLE. We retrospectively applied the criteria sets for MCTD, SLE, systemic sclerosis (SSc) and rheumatoid arthritis (RA) to all patients displaying anti-U1-RNP antibodies in the hospital of Caen from 2000 to 2020.

Impact of Glucocorticoid Cumulative Doses in a Real-Life Cohort of Patients Affected by Giant Cell Arteritis.

Objectives: To describe the impact of cumulative glucocorticoid (GC) doses on related adverse events (AEs) in giant cell arteritis (GCA) in a real-life setting.

Methods: The medical charts of the last 139 consecutive GCA patients followed in a tertiary centre were retrospectively analysed. The cumulative GC doses were calculated, and the main GC-related AEs were collected during the follow-up.

Early infectious risk in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis according to remission-induction therapy.

Objective: Few comparative data exist on early infections secondary to remission-induction therapy (RIT) with rituximab (RTX) versus cyclophosphamide (CYC) in newly diagnosed anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients. We compared and analysed the rates and predictors of severe infection in such patients within the first 6 months following RIT.

Method: From the Caen University Hospital databases, we included all consecutive adults newly diagnosed with ANCA-positive granulomatosis with polyangiitis or microscopic polyangiitis between January 2006 and December 2019.

Tolerance of glucocorticoids in giant cell arteritis: a study of patient-reported adverse events.

Objective: To assess patients' self-reported glucocorticoid (GC)-related adverse events (AEs) in a GCA population.

Methods: A questionnaire was sent to the 100 patients most recently diagnosed with GCA in a tertiary centre. This questionnaire included open- and close-ended questions on the disease and GC effects.

Impact of Giant Cell Arteritis and Its Treatment on the Patient's Quality of Life: A Single-Center Self-Assessment Study.

Little is known about the impact of giant cell arteritis (GCA) and its treatment on patient-reported physical, mental, and psychic quality of life (QoL). In this monocentric study, a questionnaire was sent to the 100 last patients diagnosed with GCA and followed-up in a single tertiary center. Their physical, mental and psychic status were self-assessed via close-ended questions, the 12-item short form survey (SF-12) and the 15-item geriatric depression scale (GDS).

Rituximab plus belimumab in non-infectious refractory cryoglobulinemia vasculitis: A pilot study.

Objective: To report the efficacy of rituximab plus belimumab in patients with refractory cryoglobulinemia vasculitis (CV).

Methods: Belimumab was administered intravenously at a dose of 10 mg/kg on days 0, 14, 28 and then every month in association with rituximab in 4 patients with refractory CV. Demographic, clinical and laboratory characteristics, treatment modalities and outcomes were recorded.

[IgA vasculitis secondary to Klebsiella pneumoniae infection].

Introduction: IgA vasculitis is a leucocytoclastic vasculitis of small vessels with immune deposits of IgA. It tends to occur in a post-infectious context, though the pathogenic agent is rarely found.

Observation: We report, for the first time, the case of an 81-year old patient who presented with an acute IgA vasculitis with cutaneous and joint involvement during a Klebsiella pneumoniae respiratory infection.

Characteristics and outcomes of patients with ophthalmologic involvement in giant-cell arteritis: A case-control study.

Purpose: To describe the characteristics and outcome of patients with giant-cell arteritis (GCA)-related ophthalmologic involvement at diagnosis.

Methods: In a retrospective single-center cohort of 409 consecutive patients with GCA, we retrieved 104 patients with visual symptoms at GCA diagnosis and we compared them to 104 age- and sex-matched controls without ophthalmologic involvement. Each visual symptom was associated to an ophthalmologic diagnosis that was centrally re-assessed by an ophthalmologist.

Sustained Response and Rationale of Programmed Cell Death-1-Targeting for Progressive Multifocal Leukoencephalopathy.

In this study, we report a complete (clinical, radiological, and virological) sustained (1 year) response after nivolumab salvage therapy in a progressive multifocal leukoencephalopathy patient. Analyses of the cells infiltrate in a pretreatment brain biopsy suggest that parenchymal programmed cell death-L1 macrophages could be the T-cells partnership in immune exhaustion and virus escape.

Factors Associated with Relapse and Dependence on Glucocorticoids in Giant Cell Arteritis.

Objective: To identify characteristics and factors associated with relapse and glucocorticoid (GC) dependence in patients with giant cell arteritis (GCA).

Methods: We retrospectively analyzed 326 consecutive patients with GCA followed for at least 12 months. Factors associated with relapse and GC dependence were identified in multivariable analyses.

Clinical Effect of Alpha-1 Antitrypsin Deficiency in Antineutrophil Cytoplasmic Antibody-associated Vasculitis: Results from a French Retrospective Monocentric Cohort.

Objective: Deficiency in alpha-1 antitrypsin (AAT) is a possible pathogenic cofactor in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, the clinical effect of AAT deficiency remains poorly established in this setting. This study aimed to describe the clinical phenotypes and outcomes of AAV according to AAT phenotypes.

Efficacy and safety of biologics in relapsing polychondritis: a French national multicentre study.

Objectives: To assess the efficacy and the safety of biologics in a cohort of patients with relapsing polychondritis (RP).

Methods: We conducted a French multicentre retrospective cohort study including patients treated with biologics for RP. Efficacy outcomes were clinical response (partial or complete) and complete response during the first 6 months of exposure, plus daily corticosteroid dose at 6 months.

Giant-cell arteritis: concordance study between aortic CT angiography and FDG-PET/CT in detection of large-vessel involvement.

Purpose: The purpose of our study was to assess the concordance of aortic CT angiography (CTA) and FDG-PET/CT in the detection of large-vessel involvement at diagnosis in patients with giant-cell arteritis (GCA).

Methods: We created a multicenter cohort of patients with GCA diagnosed between 2010 and 2015, and who underwent both FDG-PET/CT and aortic CTA before or in the first ten days following treatment introduction. Eight vascular segments were studied on each procedure.

Aortic involvement in relapsing polychondritis.

Objective: To assess prevalence of aortic involvement in relapsing polychondritis (RP) patients; to evaluate clinical features and long-term outcome of RP patients exhibiting aortitis, aortic ectasia and/or aneurysm.

Methods: One hundred and seventy-two RP patients underwent aortic computed tomography (CT)-scan; they were seen in 3 medical centers.

Results: Eleven patients (6.

Giant-Cell Arteritis: Do We Treat Patients with Large-Vessel Involvement Differently?

Purpose: We aimed to describe the initial treatment that was used in a common hospital-based practice in patients with giant-cell arteritis with and without large-vessel involvement at diagnosis as well as the outcomes in both groups.

Methods: This retrospective multi-center cohort included patients with giant-cell arteritis diagnosed between 2005 and 2015, all of whom had fluorodeoxyglucose (FDG) positron emission tomography combined with computed tomography (FDG-PET/CT) performed at giant-cell arteritis diagnosis and were followed up for ≥12 months. We compared the features, treatment, and outcomes of patients with large-vessel involvement demonstrated on FDG-PET/CT with those of patients with a negative PET/CT.

Giant Cell Arteritis-related Stroke: A Retrospective Multicenter Case-control Study.

Objective: Our aim was to describe patients with giant cell arteritis (GCA)-related stroke and to compare them with a control group of GCA patients without stroke.

Methods: We created a retrospective multicenter cohort of patients with (1) GCA diagnosed according to the American College of Rheumatology criteria between 1995 and 2015, and (2) stroke occurring at the time of GCA diagnosis or occurring within 4 weeks of starting GCA therapy. The control group consisted of GCA patients without stroke.