Immune checkpoint inhibitor therapy (ICI)-induced myositis (irMyositis) occurs in about 1% of patients treated with anti-PD1 or anti-CTLA-4 antibodies and can be debilitating or even fatal. We compared gene expression profiles from skeletal muscle biopsies between irMyositis patients, patients with spontaneous dermatomyositis (DM, comprising anti-Mi2-positive and anti-TIF1-γ-positive subtypes), and non-diseased controls (NDC). We used the NanoString nCounter PanCancer Immune Profiling Panel to perform differential gene expression (DGE) and pathway enrichment analyses.
View Article and Find Full Text PDFObjective: To determine distinct patterns of patients with autoimmune diseases harbouring anti-Ku antibodies and their respective prognosis.
Methods: Anti-Ku-positive patients were retrieved through four immunology departments. Clusters were derived from unsupervised multiple correspondence analysis, not including the disease's diagnosis, followed by hierarchical clustering.
Introduction: Idiopathic inflammatory myositis (IIM) are systemic diseases, including dermatomyositis (DM), inclusion body myositis (IBM), immune-mediated necrotising myopathy (IMNM), antisynthetase syndrome (ASSD) and overlap myositis (OM). Patients with IIM have an increased risk of premature death, largely due to cardiovascular events (CVE). The aim of this study was to describe specific and non-specific cardiac involvement in patients with IIM, and to assess the occurrence of CVE.
View Article and Find Full Text PDFRecent advances in immunotherapy have significantly improved outcomes for cancer patients. However, therapies such as immune checkpoint inhibitors (ICIs) can lead to immune-related adverse events, including potentially fatal ICI-myocarditis. The diagnosis of ICI-myocarditis is complex, and cardiac MRI plays a crucial role in identifying this condition.
View Article and Find Full Text PDFObjective: The objective of this study was to evaluate the presence of different types of interferon in idiopathic inflammatory myopathies (IIM) and their subgroups using ultrasensitive cytokine detection techniques (SIMOA) and to assess their potential as activity biomarkers.
Methods: Disease activity was measured at the time of serum collection and assessed by manual muscle testing eight (MMT8 score 0-150), muscle enzymes to calculate the Physician Global Assessment (PGA) (0-10). Patients were classified as active if PGA>5.
Background: Immune checkpoint inhibitors (ICIs) may induce overlapping myositis/myasthenia gravis (MG) features, sparking current debate about pathophysiology and management of this emerging disease entity. We aimed to clarify whether ICI-induced (ir-) myositis and ir-MG represent distinct diseases or exist concurrently.
Methods: We performed a retrospective multicenter cohort study.
Introduction: Certain types of muscular dystrophy (MD), notably facioscapulohumeral muscular dystrophy (FSHD), exhibit muscle fiber necrosis with regeneration and a nonspecific inflammatory process. Although rare, the coexistence of MDs and autoimmune myositis has been observed. We hypothesized that, in some circumstances, FSHD may predispose individuals to myositis through muscle damage-induced autoantigen overexpression, contributing to an autoimmune response.
View Article and Find Full Text PDFObjectives: Granulomatous myositis (GM) is a rare entity whose precise clinical features and therapeutic outcomes have not yet been well defined. Given the limited evidence, data from a large cohort of patients is needed to aid in the recognition and management of this condition.
Methods: We retrospectively analyzed our institutional databases to identify patients who had myositis and non-caseating granuloma on muscle biopsy (GM).
Background: Monoclonal gammopathy (MG) has been reported in association with numerous neurological disorders but the spectrum of MG-associated myopathies remains poorly described.
Objective: To report a newly acquired myopathy associated with MG.
Methods: Three adult patients with the same phenotype from two French referral centers were prospectively analyzed.
Arch Cardiovasc Dis
February 2025
Background: Immune checkpoint inhibitor (ICI)-induced myocarditis is a life-threatening adverse drug reaction. Abatacept (a CTLA-4-immunoglobulin fusion protein) has been proposed as a compassionate-use treatment for ICI myocarditis (in combination with corticosteroids and ruxolitinib) but no clinical trial has yet been performed. The abatacept dose can be adjusted using real-time assessment of its target, the CD86 receptor occupancy on circulating monocytes (CD86RO).
View Article and Find Full Text PDFObjectives: To investigate factors associated with DM complete clinical response and overall survival with a focus on the use of immunosuppressive therapies in patients with cancer-associated DM.
Methods: We performed a multicentre, retrospective cohort study. Multivariable survival analyses used a Cox model with time-dependent covariates and adjustments with inverse probability censoring weighting.
Background: Erdheim-Chester disease (ECD) is a rare histiocytosis that may overlap with Langerhans Cell Histiocytosis (LCH). This "mixed" entity is poorly characterized. We here investigated the clinical phenotype, outcome, and prognostic factors of a large cohort of patients with mixed ECD-LCH.
View Article and Find Full Text PDFBackground: Finding sensitive clinical outcome measures has become crucial in natural history studies and therapeutic trials of neuromuscular disorders. Here, we focus on 1-year longitudinal data from quantitative magnetic resonance imaging (MRI) and phosphorus magnetic resonance spectroscopy (P MRS) in a placebo-controlled study of sirolimus for inclusion body myositis (IBM), also examining their links to functional, strength, and clinical parameters in lower limb muscles.
Methods: Quantitative MRI and P MRS data were collected at 3 T from a single site, involving 44 patients (22 on placebo, 22 on sirolimus) at baseline and year-1, and 21 healthy controls.
Objectives: To describe the characteristics of patients with Sjögren's disease (SjD) and inclusion-body myositis (IBM), and how they compare to SjD patients with other inflammatory myopathies (IM).
Methods: Patients were retrospectively recruited from 13 French centres and included if they met the ACR/EULAR criteria for SjD and for IM. They were categorized as SjD-IBM if sub-criteria for IBM were met, or as SjD-other IM if not.