Publications by authors named "Guillaume-Jugnot P"
Objective: Among specific autoantibodies in DM, the anti-small ubiquitin-like modifier activating enzyme (SAE) antibody is rare. We aim to describe the clinical characteristics, cancer prevalence, and muscle pathology of anti-SAE-positive DM.
Methods: Patients with a diagnosis of DM and sera positive for the anti-SAE antibody were recruited from 19 centres in this retrospective observational study.
Article Synopsis
- The study investigates the connection between immune-checkpoint inhibitor (ICI)-associated myotoxicity, specifically looking at myocarditis and myositis, which can be life-threatening.
- It details a treatment strategy that involved the use of mechanical ventilation for respiratory muscle issues and the administration of the drugs abatacept and ruxolitinib in patients diagnosed with severe ICI myocarditis.
- Results showed a significant drop in myotoxicity-related fatalities from 60% in the initial patient group to just 3.4% in the later group, indicating the effectiveness of early intervention and specific treatment adjustments.
Objective: This study aimed to investigate the relationship between changes in clinical status on daily life physical activity (PA) in patients with idiopathic inflammatory myopathy (IIM).
Methods: Patients with dermatomyositis (DM), immune-mediated necrotizing myopathy (IMNM) or overlap myositis (OM) who presented either a new-onset or relapsing IIM, stable disease on maintenance therapy or were undergoing immunosuppressant tapering were included. Patients were evaluated at inclusion (V0), and at two follow-up visits (V1, 94±12 days from V0; V2, 96±17 days from V1).
Article Synopsis
- The study aimed to assess muscle damage severity in patients with immune-mediated necrotizing myopathy (IMNM) and relate it to clinical features using MRI and the Mercuri score.
- Results showed two distinct patient subgroups in terms of muscle damage: one with mild lesions and the other with severe lesions, with disease duration being the key predictor of muscle damage severity.
- Overall, both IMNM and sporadic inclusion-body myositis (s-IBM) had similar levels of muscle damage, but IMNM specifically impacted pelvic and lumbar muscle groups more severely.
The identification of AQP4-IgG, a specific and pathogenic antibody of NMO/SD has led to a broadening of the clinical spectrum of manifestations of NMO/SD including the presence of encephalic symptoms. Lesions are often distributed on peri‑ependymal area and sometimes affected the diencephalon leading to sleep disorders. We report a case of hypersomnia with polysomnographic documentation during the first attack of NMO/SD.
View Article and Find Full Text PDFIntroduction: The use of autologous haematopoietic stem cell transplantation (AHSCT) in autoimmune disease (AD) patients has increased progressively worldwide. We retrospectively analysed the long-term outcome of AHSCT for AD reported to the French Society for Bone Marrow Transplantation and Cellular Therapy (SFGM-TC).
Method: All French AD patients (≥ 18 years at transplant) with a first AHSCT between 1997 and 2013 were included.
The Francophone Society of Bone Marrow Transplantation and Cellular Therapy (SFGM-TC) organized the 7th allogeneic hematopoietic stem cell transplantation clinical practices harmonization workshop series in September 2017 in Lille, France and updated recommendations for indications and follow-up in autologous hematopoietic stem cell transplantation in autoimmune and autoinflammatory diseases, previously published under the auspices of SFGM-TC.
View Article and Find Full Text PDFSystemic sclerosis is an autoimmune disease characterized by sclerosis (hardening) of the skin and deep viscera associated with microvascular functional and structural alteration, which leads to chronic ischemia. In the hands of patients, ischemic and fibrotic damages lead to both pain and functional impairment. Hand disability creates a large burden in professional and daily activities, with social and psychological consequences.
View Article and Find Full Text PDFObjective: Impaired hand function greatly contributes to disability and reduced quality of life in SSc patients. Autologous adipose-derived stromal vascular fraction (ADSVF) is recognized as an easily accessible source of regenerative cells. We reported positive 6-month safety and efficacy results from an open-label clinical trial assessing s.
View Article and Find Full Text PDFIntroduction: Malignant thymoma or thymic hyperplasia is associated with various autoimmune diseases. Renal disease has rarely been reported in this condition. We report a new case with improvement of renal disease after thymectomy.
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