Publications by authors named "Kim H Ly"

Article Synopsis
  • The purpose of this project was to update the 2016 recommendations regarding the management of immunosuppressants or biologics in patients with giant cell arteritis (GCA).
  • A task force of 18 physicians developed 26 validated recommendations after thorough discussion and a >85% consensus process.
  • Key recommendations include using subcutaneous tocilizumab (TCZ) as a first-line treatment when glucocorticoid-sparing is needed, initiating TCZ at diagnosis for patients with specific high-risk conditions, and considering TCZ or methotrexate if glucocorticoid discontinuation is not feasible after 12 months.
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Article Synopsis
  • Giant cell arteritis (GCA) primarily affects women over 50, causing symptoms like headaches and jaw pain, and can lead to severe complications like vision loss due to eye involvement.
  • Diagnosis relies on advanced imaging techniques such as FDG-PET and CT scans, while the temporal artery biopsy is considered the gold standard.
  • Urgent treatment with high doses of corticosteroids is critical to prevent vision loss, and long-term management is necessary to monitor for recurrence and potential cardiovascular issues.
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  • MGUS-associated angioedema due to acquired C1 inhibitor deficiency (AAE-C1-INH) hasn't been specifically characterized before, prompting this study to explore its biological and clinical features over 30 years in France.
  • In a study of 41 patients, most had anti-C1INH antibodies, and treatments included acute management and long-term prophylaxis, with a significant number developing malignant blood disorders like lymphoma or myeloma.
  • The study found a link between the remission of angioedema and the underlying hematological malignancy, emphasizing the need for regular hematological evaluations in patients with MGUS-AE-C1-INH.
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Background: Patients with inflammation of unknown origin (IUO) and fever of unknown origin (FUO) are commonly considered a single population. Differences in underlying causes between both groups may steer the diagnostic work-up.

Methods: PubMed, Embase, Web of Science, and ClinicalTrials.

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Article Synopsis
  • Angioedema due to acquired C1-inhibitor deficiency (AAE-C1-INH) can result from excess consumption of C1-INH or the presence of anti-C1-INH antibodies, commonly linked to lymphoid cancers or monoclonal gammopathies.
  • A study in France assessed the effectiveness of rituximab for treating 55 patients with AAE-C1-INH, revealing that 34 patients achieved remission after an average of nearly 4 years of follow-up.
  • The results indicated that patients without anti-C1-INH antibodies had a better chance of remission; those with lymphoma and those treated with rituximab and chemotherapy experienced lower rates of relapse, suggesting that rituximab
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Introduction: Giant cell arteritis (GCA) is complicated in 10 to 20% of cases by permanent visual ischemia (PVI). International guidelines advocate the use of intravenous pulse of methylprednisolone from 250 to 1000mg per day, for three days, followed by oral prednisone at 1mg/kg per day. The aim of this study is to assess whether this strategy significantly reduces the risk of early PVI of the second eye, compared with direct prednisone at 1mg/kg per day.

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Article Synopsis
  • Still's disease (SD) is an autoinflammatory condition with varying forms, and this study aimed to compare adult-onset SD (AOSD) and systemic juvenile idiopathic arthritis (sJIA) while identifying prognosis.
  • Researchers analyzed data from 238 patients, finding that 64% had AOSD, with symptoms like sore throat and muscle pain being more common in this group, as well as higher levels of liver enzymes and inflammatory markers.
  • Key findings indicated that symptoms like fever and skin rash are linked to better recovery chances, while high lactate dehydrogenase levels suggest a poorer prognosis, supporting the idea that SD can evolve into chronic arthritis if not treated promptly.
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Objectives: To study the percentage, suppressive function and plasticity of Treg in giant cell arteritis (GCA), and the effects of glucocorticoids and tocilizumab.

Methods: Blood samples were obtained from 40 controls and 43 GCA patients at baseline and after treatment with glucocorticoids + IV tocilizumab ( = 20) or glucocorticoids ( = 23). Treg percentage and phenotype were assessed by flow cytometry.

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While the diagnosis of adult-onset Still's disease (AOSD) involves the exclusion of differential diagnoses, the characteristics and value of 18F-Fluorodeoxyglucose (18F-FDG) Positron Emission Tomography coupled with CT (PET/CT) in the management of AOSD remain poorly known. Our retrospective study included patients from four centers, fulfilling Yamaguchi or Fautrel criteria, who underwent a PET/CT during an active AOSD. Thirty-five patients were included.

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Giant cell arteritis (GCA) is a large-vessel vasculitis involving the aorta and its main branches, especially supra aortic branches. Although much progress has been made, the pathophysiology remains incompletely understood. An initial trigger, suspected of infectious origin, lead to the maturation and recruitment of dendritic cells (DC).

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Objectives: To estimate the frequency of different clinical patterns in giant-cell arteritis (GCA) at onset.

Methods: All GCA patients consecutively followed-up in two referral centers for GCA with a biopsy-proven diagnosis and/or large-vessel vasculitis (LVV) demonstrated on imaging were analysed.

Results: We analysed the initial clinical presentation of 693 patients with a median age of 75 [48-94] years and including 486 (70%) women.

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Large-vessel involvement (LVI) in giant cell arteritis (GCA) includes different clinical and imaging patterns that are rarely described separately at diagnosis and whose specific cardiovascular outcomes are unknown. We conducted a nationwide retrospective study and included GCA patients with LVI demonstrated on imaging at diagnosis between 2007 and 2017. We analyzed the prognosis of three different imaging patterns of LVI present at diagnosis, with some of them overlapping but with the first one present in all patients: 1) inflammation of the aorta and/or its branches; 2) dilation of the aorta; and 3) stenosis of the aortic branches.

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Objective: The aims of this study were to describe and assess the vascular outcomes of patients with giant cell arteritis (GCA) presenting with only symptomatic isolated limb involvement (LI-GCA).

Methods: We recruited patients from 5 tertiary centers who were diagnosed with GCA based on histology or vasculitis demonstration on imaging and who presented with isolated symptomatic limb involvement at diagnosis. For each included patient, we randomly selected 3 control patients who satisfied the 5 criteria from the American College of Rheumatology at diagnosis.

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The objective of this study was to determine the proportion and characteristics of patients with giant cell arteritis (GCA) who present with isolated inflammatory response and/or fever of unknown origin (IFUO). Using a cohort of 693 consecutive patients in two centers with evidence of GCA on biopsy and/or imaging, we compared the characteristics and outcomes of patients with IFUO at diagnosis to a control group made up of the remaining patients with GCA. Sixty-one (9%) patients initially presented with IFUO.

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Background: The aim of this study was to evaluate tocilizumab (TCZ) as an add-on therapy to glucocorticoids (GC) during the first 3 months of treatment of giant cell arteritis (GCA).

Methods: GCA patients, as defined by ≥3/5 ACR criteria and positive temporal artery biopsy (TAB) or angio-CT-scan or PET-scan-proven aortitis, were included in this prospective open-label study. Prednisone was started at 0.

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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.

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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.

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Objectives: To analyse the 10-year outcomes of 64 patients with non-HBV polyarteritis nodosa (PAN) or microscopic polyangiitis (MPA) and Five-Factor Score-defined poor-prognosis factors enrolled (1994-2000) in the prospective, randomised, open-label CHUSPAN trial.

Methods: The 64 patients were randomised to receive 12 (33: 23 MPA, 10 PAN) or 6 (31: 17 MPA, 14 PAN) cyclophosphamide (CYC) pulses combined with glucocorticoids. Ten-year follow-up of these patients included times to relapse(s), failure(s) and/or deaths calculated from treatment onset.

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Objective: The pathophysiology of giant cell arteritis (GCA) and the mechanisms underlying vascular remodeling, are poorly understood. We aimed to compare vascular smooth muscle cells (VSMCs) from patients with GCA and controls by a proteomic and gene expression profile approach and to identify the signaling pathways involved in proliferation.

Methods: VSMCs were cultured from temporal artery biopsies (TABs) from patients with biopsy-proven GCA (TAB-GCA), biopsy-negative GCA (TAB-GCA), and diagnosis other than GCA (GCA-control).

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Although a glucocorticoid (GC)-sparing strategy is needed for patients with giant cell arteritis (GCA) suffering from refractory disease or serious treatment-related complications, evidence of efficacy in this setting of immunosuppressive drugs and biotherapies is lacking. Herein, we evaluated the GC-sparing effects and tolerability of addition of dapsone (DDS) to prednisone therapy in patients with GCA. We retrospectively assessed data on 18 GCA patients who received DDS as a first-line treatment (DDS-1 group) and 52 patients who received it as a second- or third-line treatment for refractory GCA, with or without excessive GC-related toxicity (DDS-2 group).

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Vascular smooth muscle cells (VSMCs) are highly specialized cells that regulate vascular tone and participate in vessel remodeling in physiological and pathological conditions. It is unclear why certain vascular pathologies involve one type of vessel and spare others. Our objective was to compare the proteomes of normal human VSMC from aorta (human aortic smooth muscle cells, HAoSMC), umbilical artery (human umbilical artery smooth muscle cells, HUASMC), pulmonary artery (HPASMC), or pulmonary artery VSMC from patients with pulmonary arterial hypertension (PAH-SMC).

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Objectives: To describe the features and outcomes of patients with giant cell arteritis who developed venous thrombosis.

Methods: Inception cohort study including 428 newly diagnosed patients of giant cell arteritis from 1976 to 2014. Clinical and biological data and outcomes were analysed by comparing patients with and without venous thrombosis.

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CD8(+) T cells participate in the pathogenesis of some vasculitides. However, little is known about their role in Giant Cell Arteritis (GCA). This study was conducted to investigate CD8(+) T cell involvement in the pathogenesis of GCA.

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Objective: To report the efficacy and safety of tocilizumab (TCZ) for giant cell arteritis (GCA).

Methods: A retrospective multicenter study that included 34 patients receiving TCZ for GCA.

Results: TCZ was effective in all but 6 patients, who still had mild symptoms.

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Objective: Findings from the WEGENT trial and other short-term studies have suggested that azathioprine (AZA) or methotrexate (MTX) could effectively maintain remission of granulomatosis with polyangiitis (Wegener's) (GPA) or microscopic polyangiitis (MPA). This study was undertaken to examine whether differences in rates of relapse or adverse events would appear after discontinuation of these 2 maintenance regimens, when assessed over a longer followup period.

Methods: Long-term outcomes in patients enrolled in the WEGENT trial were analyzed according to their randomized treatment group (AZA or MTX).

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