24 results match your criteria: "Centre National de Référence du Lupus[Affiliation]"

BackgroundAssessment of contemporary cardiovascular risk scores using clinically relevant endpoints is lacking in systemic lupus erythematosus (SLE).AimThis study aimed to assess and compare the performances of SCORE2, QRISK3 and PREVENT equations in SLE.MethodsSLE patients with no prior atherosclerotic cardiovascular disease (ASCVD) who underwent a baseline cardiovascular risk assessment including coronary artery calcium (CAC) scoring at the French national SLE reference center between 2014 and 2024 were retrospectively included.

View Article and Find Full Text PDF

Objective: Antiphospholipid syndrome (APS) is an acquired autoimmune prothrombotic condition. Vascular liver disorders (VLD), such as portal vein thrombosis (PVT), Budd-Chiari syndrome (BCS) and porto-sinusoidal vascular disorder (PSVD), are rare and related to an underlying hypercoagulable state in most cases. We aimed to describe the clinical and immunological features of APS patients with VLD.

View Article and Find Full Text PDF

Interferon-α biological activity is associated with disease activity and risk of flare in cutaneous lupus erythematosus: A monocentric study of 184 patients.

J Am Acad Dermatol

May 2025

Sorbonne Université, Faculté de médecine, AP-HP, Service de Dermatologie et Allergologie, Hôpital Tenon, Paris, France; Sorbonne Université, Inserm, Centre d'Immunologie et des Maladies Infectieuses-Paris (Cimi-Paris), INSERM U1135, Paris, France. Electronic address:

Background: Cutaneous lupus erythematosus (CLE) is associated with unpredictable flares and may induce permanent damage. There is currently no biomarker routinely available in CLE.

Objective: To evaluate the performance of interferon-α (IFN-α) biological activity as biomarker of CLE activity and risk of flare.

View Article and Find Full Text PDF

Impacts of immersive 3D videos on students' surgical learning compared to 2D videos: a randomized controlled trial.

Int J Surg

December 2024

Department of Gynecological and Breast Surgery and Oncology, Pitié-Salpêtrière, Assistance Publique des Hôpitaux de Paris (AP-HP), University Hospital, Paris, France.

Background: Unlike other medical specialties, surgery is primarily learned through apprenticeship, by observing surgeons in action during operations. However, the increasing number of medical students and work-hour restrictions limit opportunities for learning in the operating room (OR). These circumstances call for novel technologies, such as immersive video.

View Article and Find Full Text PDF

Objectives: The aim of this study was to compare CMR imaging biomarkers between SLE patients and matched controls.

Materials And Methods: Electronic databases were systematically searched from inception until November 2023. All studies reporting CMR imaging data in SLE patients were included.

View Article and Find Full Text PDF
Article Synopsis
  • Cutaneous lupus erythematosus (CLE) can occur on its own or alongside systemic lupus erythematosus (SLE), where lab tests show specific abnormalities.
  • This study aimed to compare isolated CLE to CLE with lab-confirmed SLE and to validate a scoring system predicting the risk of developing severe SLE (sSLE).
  • Results indicated that patients with CLE and laboratory SLE had a significantly higher risk of progressing to sSLE compared to those with isolated CLE, though the study had limitations due to its small, single-center patient sample.
View Article and Find Full Text PDF

Systemic lupus erythematosus and glucocorticoids: A never-ending story?

Best Pract Res Clin Rheumatol

December 2023

Sorbonne Université, Faculté de Médecine, Groupement Hospitalier Pitié Salpêtrière, Centre National de Référence du Lupus Systémique, du Syndrome des Antiphospholipides et autres maladies auto-immunes, Service de Médecine Interne 2, Institut E3M, CIMI-Paris, Paris, France.

Glucocorticoids (GCs) continue to be essential agents for the management of systemic lupus erythematosus, since there are no other drugs able to active remission of active disease so rapidly. However, their potential for causing irreversible damage greatly limit their use. Fortunately, some strategies may help take advantage of their huge anti-inflammatory power while limiting GC-induced side effects.

View Article and Find Full Text PDF

French practical guidelines for the diagnosis and management of relapsing polychondritis.

Rev Med Interne

June 2023

Internal Medicine Department URM Pavilion C.I.C. 1436 - module plurithématique adulte, hôpital Purpan, CHU de Toulouse, Toulouse, France.

Article Synopsis
  • Relapsing polychondritis is a rare inflammatory disease primarily affecting cartilage in middle-aged individuals, often starting with chondritis in areas like the ears, nose, or respiratory tract.
  • Diagnosis relies on clinical signs and ruling out other conditions since no specific lab test confirms it, and symptoms may not appear until years after initial signs.
  • Treatment varies and typically involves a combination of non-steroidal anti-inflammatory drugs and immunosuppressants, with a better outlook for patients with limited cartilage involvement, while respiratory or cardiovascular issues worsen the prognosis.
View Article and Find Full Text PDF

Rapid efficacy of anifrolumab in refractory cutaneous lupus erythematosus.

J Am Acad Dermatol

July 2023

Sorbonne Université, Faculté de médecine, AP-HP, Groupement Hospitalier Pitié Salpêtrière, Centre National de Référence du Lupus Systémique, du syndrome des antiphospholipides et autres maladies auto-immunes, Service de Médecine Interne 2, Institut E3M, CIMI-Paris, Paris, France.

View Article and Find Full Text PDF
Article Synopsis
  • - The study aimed to identify risk factors for patients with discoid lupus erythematosus (DLE) progressing to severe systemic lupus erythematosus (sSLE) and to create a predictive scoring system for this progression.
  • - In analyzing data from 30 patients who developed sSLE and 134 who did not, three main risk factors were identified: being under 25 at DLE diagnosis, having a higher skin phototype, and high antinuclear antibody titers.
  • - The findings suggested that a higher score correlates with increased risk of progression to sSLE, with a score of 0 showing no progression, while a score of 6 or higher indicated about a 40% risk
View Article and Find Full Text PDF

Objectives: We aimed to evaluate the difficulties encountered by systemic lupus erythematosus (SLE) patients during the early COVID-19 pandemic and to evaluate their impact on patient mental health.

Methods: We conducted a nationwide survey including SLE patients from France, recruited by their treating specialist or through a patient association. The survey was administered online or in paper form between November 2020 and April 2021 and included questions aiming at evaluating the difficulties encountered during the early COVID-19 pandemic (March to August 2020).

View Article and Find Full Text PDF
Article Synopsis
  • Kikuchi-Fujimoto disease (KFD) is a self-limiting condition causing lymphadenitis, and a similar pattern called "Kikuchi disease-like inflammatory pattern" (KLIP) has been noted in skin lesions, potentially indicating lupus.
  • A study compared 13 lupus patients with KLIP to 39 control lupus patients without KLIP, finding a higher prevalence of systemic lupus erythematosus (SLE) and severe disease in those with KLIP.
  • Treatment with thalidomide showed effective results in clearing skin lesions for KLIP patients, highlighting the need for close monitoring in patients at risk of developing severe SLE.
View Article and Find Full Text PDF

Two-Year, Randomized, Controlled Trial of Belimumab in Lupus Nephritis.

N Engl J Med

September 2020

From the Division of Rheumatology, Northwell Health, Donald and Barbara Zucker School of Medicine at Hofstra-Northwell, Great Neck, NY (R.F.); the Division of Nephrology, Ohio State University, Columbus (B.H.R.); Pôle de Pathologies Rhumatismales Inflammatoires et Systémiques, Institut de Recherch

Background: In adults with active lupus nephritis, the efficacy and safety of intravenous belimumab as compared with placebo, when added to standard therapy (mycophenolate mofetil or cyclophosphamide-azathioprine), are unknown.

Methods: In a phase 3, multinational, multicenter, randomized, double-blind, placebo-controlled, 104-week trial conducted at 107 sites in 21 countries, we assigned adults with biopsy-proven, active lupus nephritis in a 1:1 ratio to receive intravenous belimumab (at a dose of 10 mg per kilogram of body weight) or matching placebo, in addition to standard therapy. The primary end point at week 104 was a primary efficacy renal response (a ratio of urinary protein to creatinine of ≤0.

View Article and Find Full Text PDF

From a 1-year survival of less than 50% before the discovery of glucocorticoids to over 90% at 10 years in most dedicated centres, the spectrum of SLE has profoundly evolved. Despite this improvement, several major challenges currently remain. The aim of this review is to analyse what are, according to us, the 10 most important contemporary challenges in the management of SLE.

View Article and Find Full Text PDF

Clinical and immunological features of antiphospholipid syndrome in the elderly: a retrospective national multicentre study.

Rheumatology (Oxford)

June 2019

Département de Médecine Interne, Hôpital Bichat, Université Paris Diderot, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris, Paris, France.

Objective: APS mainly affects women who are of child-bearing age. We aimed to describe the clinical and immunological features of APS patients diagnosed after the age of 60.

Methods: The Elderly-Phospholipid study is a national, multicentre, retrospective study involving all APS (2006 Miyakis criteria) patients followed in five French tertiary university centres including four national referral lupus and APS centres.

View Article and Find Full Text PDF
Article Synopsis
  • A new technology, photonic ring immunoassay (PRI), allows for the simultaneous detection of 12 autoantibodies in whole blood within 15 minutes.
  • In a study with 235 patients, PRI demonstrated over 91% overall agreement with standard lab tests, showing high sensitivity and specificity.
  • Additionally, tests on 199 normal blood donors showed specificities exceeding 97.5%, indicating PRI's potential for quick point-of-care testing and timely results for doctors.
View Article and Find Full Text PDF

Shrinking lung syndrome associated with systemic lupus erythematosus: A multicenter collaborative study of 15 new cases and a review of the 155 cases in the literature focusing on treatment response and long-term outcomes.

Autoimmun Rev

October 2016

AP-HP, Service de Médecine Interne 2, Centre National de Référence du Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et Maladies Auto-immunes Systémiques Rares, Groupe Hospitalier Pitié Salpêtrière, Paris 75013, France; Université Paris VI Pierre et Marie Curie, Sorbonnes Univ

Introduction: Shrinking lung syndrome (SLS) is a rare respiratory manifestation of systemic lupus erythematosus (SLE), characterized by dyspnea, chest pain, elevated hemidiaphragm and a restrictive pattern on pulmonary function tests. Here, we report 15 new observations of SLS during SLE and provide a systematic literature review. We studied the clinical, biological, functional and morphologic characteristics, the treatments used and their efficacy.

View Article and Find Full Text PDF

Thrombophilia Associated with Anti-DFS70 Autoantibodies.

PLoS One

June 2016

Département d'Immunologie, Hôpital Pitié-Salpêtrière (AP-HP), Paris, France; INSERM U1135, Centre d'Immunologie et des Maladies Infectieuses (CIMI), Paris, France.

Context: Anti-DFS70 antibodies are the most frequent antinuclear antibodies (ANA) found in healthy individuals. We assessed the clinical significance of the presence of anti-DFS70 antibodies.

Methods: We defined a group of patients (n = 421) with anti-DFS70 antibodies and a group of patients (n = 63) with a history of idiopathic arterial and/or venous thrombotic disease and/or obstetric complication (i.

View Article and Find Full Text PDF

Purpose: Though recommended, participation of patients with specific expertise in therapeutic education programs (TEP) is rare. This work reports the experience of a national reference centre for rare systemic diseases.

Patients And Methods: Involvement of "expert patients" (EP) has been planned from the development of a TEP dedicated to systemic lupus: patients' roles and required expertise have been defined and linked to the pedagogical tools.

View Article and Find Full Text PDF

[Screening and management of cardiovascular risk factors in systemic lupus erythematosus: Recommendations for clinical practice based on the literature and expert opinion].

Rev Med Interne

June 2015

Service de médecine interne 2, Centre national de référence du lupus systémique, groupe hospitalier Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75013 Paris, France; Sorbonne universités, UPMC université Paris 06, 75013 Paris, France.

Purpose: To develop French recommendations about screening and management of cardiovascular risk factors in systemic lupus erythematosus (SLE).

Methods: Thirty-nine experts qualified in internal medicine, rheumatology and nephrology have selected recommendations from a list developed based on evidence from the literature. For each recommendation, the level of evidence and the level of agreement among the experts were specified.

View Article and Find Full Text PDF

[Assessing the cardiovascular risk in patients with systemic lupus erythematosus].

Rev Med Interne

November 2014

Service de médecine interne 2, Centre national de référence du lupus systémique, groupe hospitalier Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75013 Paris, France; Sorbonne universités, UPMC université Paris 06, 75013 Paris, France; Inserm, U1135, CIMI, 75013 Paris, France.

Multiple factors contribute to the increased cardiovascular risk observed in patients with systemic lupus erythematosus (SLE). Among these are the so-called classical cardiovascular risk factors, the disease itself through its activity, treatments, and complications, and the thrombotic risk due to antiphospholipid antibodies (aPL). Observational studies suggest that most classical cardiovascular risk factors are observed more frequently in SLE patients than in the general population, and that these are insufficient to explain the increased cardiovascular risk observed in most studies.

View Article and Find Full Text PDF

Purpose: The aim of this study was to collect information to design a patient education program (PEP) for patients with systemic lupus erythematosus (SLE), based as much as possible on their expectations.

Patients And Methods: Three different approaches were used for addressing patients' needs: 1) A questionnaire on their expectations in terms of a PEP was sent to the members of SLE associations and offered to patients at the French reference center for SLE, 2) A patients' focus group was conducted, and 3) After the teaching sessions, satisfaction questionnaires were also evaluated.

Results: The patients who answered the expectation questionnaire (n=422, women/men sex-ratio: 12.

View Article and Find Full Text PDF