Autologous haematopoietic stem cell transplantation for rheumatic diseases: best practice recommendations from the EBMT Practice Harmonization and Guidelines Committee.

Immune-mediated rheumatologic and musculoskeletal diseases (RMDs) comprise a heterogeneous group of systemic conditions that affect the connective tissues of the musculoskeletal system and internal organs. Immune-mediated RMDs are driven by chronic autoimmune responses and typically require continuous or repeated administration of immunosuppressive or biologic disease-modifying drugs. Although generally effective, these therapies can cause both short- and long-term side effects and may fail to control the disease with risk of irreversible tissue damage.

Urinary soluble CD163 is useful to predict lupus nephritis activity and to monitor standard-of-care therapy response.

Objectives: This study aims to assess urinary soluble CD163 normalized to creatinuria (usCD163/Cre) alongside conventional biomarkers as indicators of renal activity and therapeutic response in lupus nephritis (LN).

Methods: A monocentric and retrospective cohort analysis involving 214 patients with systemic lupus erythematosus (SLE) was conducted, among whom 129 were referred as LN and assessed longitudinally. Of these, 39 underwent kidney biopsy at sample collection.

Rituximab Versus Conventional Therapy for Remission Induction in Eosinophilic Granulomatosis With Polyangiitis : A Randomized Controlled Trial.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is an eosinophilic antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Rituximab has emerged as the standard of care in other types of ANCA-associated vasculitis, but controlled studies on its use in EGPA are yet lacking.

Objective: To compare rituximab with conventional strategy for the induction of remission in patients with EGPA.

[Assessment of internship sites in Internal Medicine and Clinical Immunology, 7 years after the Residency Training Program Reform: Findings from a 2024 year-end survey in France].

Objective: To compile a list of approved training sites for the Residency Training Program [Diplôme d'Études Spécialisées] in Internal Medicine and Clinical Immunology (DES-MIIC) in France.

Method: All local coordinators of the DES-MIIC were contacted to establish the list of approved internship sites for the MIIC DES within their geographical subdivision.

Results: We listed 244 approved training sites, of which 87 (35.

Hand surgical and injectable treatments in systemic sclerosis: A systematic review of published cases.

Introduction: Systemic sclerosis is a systemic autoimmune disease that can affect the hands leading to a deterioration in function. Unfortunately, current medical treatments are considered insufficient to improve patients' quality of life. However, innovative surgical and injectable treatments are available.

Interferon-γ release assay as an emergent powerful biomarker in systemic lupus erythematosus.

Objectives: To investigate the ex vivo IFN-γ release assay (IGRA) as a biomarker of SLE activity and disease outcome.

Methods: This retrospective study, conducted between 2008 and 2024 at a single tertiary care center, included 145 SLE patients at various disease stages. Data were collected on spontaneous IFN-γ levels (IGRA-nil) and on phytohemagglutinin-induced IFN-γ levels (IGRA-PHA, after subtracting the IGRA-nil result).

Efficacy and safety of Anakinra in idiopathic recurrent pericarditis: Results from the UPACITER study.

Background: Idiopathic recurrent pericarditis (IRP) is a chronic autoinflammatory condition characterized by relapsing episodes of pericardial inflammation. Standard treatment involves colchicine and non-steroidal anti-inflammatory drugs (NSAIDs), with corticosteroids as a second-line therapy. However, many patients develop corticosteroid dependency or resistance, necessitating alternative treatments.

Evaluation of the effects of pre-exposure treatment with hydroxychloroquine on the risk of COVID-19 infection and on the efficacy of anti-COVID-19 vaccination during lupus or Gougerot-Sjögren's disease: Prepcov multicentre trial.

Objectives: Some patients with SLE or Gougerot-Sjögren's disease (GSD) receive long-term treatment with hydroxychloroquine (HCQ), sometimes combined with immunosuppressive therapy (IS). This study sought to assess whether long-term HCQ therapy that had been initiated long before the COVID-19 pandemic had a protective or adverse effect on COVID-19 risk, severity of infection or immunity protection.

Methods: This prospective multicentre study included 547 patients with SLE, GSD, autoimmune hepatitis, primary biliary cholangitis or cured viral hepatitis C divided into four groups according to HCQ (+/-) and IS (+/-) intake prior to the pandemic: HCQ+IS+ (n=112), HCQ+IS- (n=121), HCQ-IS+ (n=115) and HCQ-IS- (n=199).

Total lung capacity is predictive of disease severity and survival in systemic sclerosis: A longitudinal analysis in 2347 patients from the French National Cohort Study.

Background: Total lung capacity (TLC) is seldom assessed in the prediction of systemic sclerosis (SSc) disease severity.

Objective: To describe and analyse TLC in SSc.

Methods: We performed a retrospective multicentre study of SSc patients enrolled in the French national SSc cohort with at least one TLC assessment, described patients based on baseline TLC measurements, modelized TLC trajectories in SSc, and associated TLC measures with disease prognosis.

Real-life use of the PEXIVAS reduced-dose glucocorticoid regimen in granulomatosis with polyangiitis and microscopic polyangiitis.

Background: The PEXIVAS (Plasma exchange and glucocorticoids in severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis) trial showed that a reduced-dose glucocorticoid regimen (redGC) was non-inferior to a standard-dose regimen (standGC) with respect to death or end-stage kidney disease (ESKD) in patients with ANCA-associated vasculitis (AAV). However, the primary endpoint did not include disease progression or relapse, cyclophosphamide was the main induction therapy and rituximab (RTX)-treated patients tended to have a higher risk of death or ESKD with redGC. We aimed to evaluate the real-world use of redGC.

The added value of coupling anti-dsDNA and anti-chromatin antibodies in follow-up monitoring of systemic lupus erythematosus patients.

Objective: The autoimmune response to chromatin (Chr) or one of the nucleosome components (double stranded (ds)DNA and histones) is typically associated with the development of systemic lupus erythematosus (SLE). Related autoantibodies (Ab) are heterogeneous and, among them, anti-dsDNA Ab are part of the classification criteria and recommended for monitoring SLE with regards to lupus flares and therapy responses. However, anti-dsDNA Ab biomarker performances are weak; therefore, coupling anti-dsDNA with anti-Chr Ab can be proposed, which is the aim of this study.

Allogeneic umbilical cord-derived mesenchymal stromal cells as treatment for systemic lupus erythematosus: a single-centre, open-label, dose-escalation, phase 1 study.

Background: Patients with systemic lupus erythematosus (SLE) with inadequate responses to standard therapies have unmet therapeutic needs. The immunomodulatory, proangiogenic, and antifibrotic properties of mesenchymal stromal cells support their use in treating patients with SLE. We aimed to assess the safety of a single intravenous infusion of allogeneic umbilical cord-derived mesenchymal stromal cells in patients with severe SLE.

Vasodilator drugs and heart-related outcomes in systemic sclerosis: an exploratory analysis.

Background And Aims: Systemic sclerosis (SSc) is an autoimmune connective disease characterised by excessive extracellular matrix deposition and widespread skin and internal organ fibrosis including various cardiac manifestations. Heart involvement is one of the leading causes of death among patients with SSc. In this study, we aimed to assess the effect of various vasodilator treatments.

Urinary soluble CD163 is useful as "liquid biopsy" marker in lupus nephritis at both diagnosis and follow-up to predict impending flares.

Lupus nephritis (LN) diagnosis and follow-up requires noninvasive biomarkers. Therefore, the added value of coupling the urinary soluble (s)CD163/creatinuria ratio with serological markers was evaluated in a real-world clinical practice. To this end, a monocentric and retrospective study was conducted in 139 SLE patients with biopsy-proven nephritis having an active LN (LN-A, n = 63 with a positive SLEDAI-renal score) or inactive (n = 76), as well as 98 non-renal SLE patients.

Risk loci involved in giant cell arteritis susceptibility: a genome-wide association study.

Background: Giant cell arteritis is an age-related vasculitis that mainly affects the aorta and its branches in individuals aged 50 years and older. Current options for diagnosis and treatment are scarce, highlighting the need to better understand its underlying pathogenesis. Genome-wide association studies (GWAS) have emerged as a powerful tool for unravelling the pathogenic mechanisms involved in complex diseases.

History of pre-eclampsia does not appear to be a risk factor for vascular phenotype in women with systemic sclerosis.

Background: Vascular phenotype is associated with a poor prognosis in systemic sclerosis (SSc). The identification of its risk factors could facilitate its early detection.

Objectives: To explore risk factors for a vascular phenotype of SSc, among them a history of pre-eclampsia.

IgG4-related uveitis. A French cohort and literature review.

Introduction: IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder that can affect almost any organ. IgG4-related ophthalmic disease is a protean condition involving the orbit and ocular adnexa. Although a few cases of uveitis have been reported, the exact pattern of IgG4-related intraocular manifestations remains unclear.

HRCT imaging of pulmonary involvement in granulomatosis with polyangiitis and microscopic polyangiitis at disease onset and during follow-up.

Background: The pulmonary involvement in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) is well known at disease onset but data during follow-up (after the induction regimen and when the first relapse occurs) are limited. Our goal was to analyze chest high-resolution computed tomography (HRCT) findings of (ANCA)-associated vasculitis patients.

Method: All consecutive unselected AAV patients over eighteen with positive ANCA status and with HRCT chest performed at the diagnosis were retrospectively enrolled between 2004 and 2019 at the Toulouse University Hospital (France).

Heart and systemic sclerosis-findings from a national cohort study.

Objectives: Heart involvement is one of the leading causes of death in SSc. The prevalence of SSc-related cardiac involvement is poorly known. Our objective was to investigate the prevalence and prognosis burden of different heart diseases in a nationwide cohort of patients with SSc.

[Mobilization and conditioning protocols actualization for autologous stem cell transplantation for autoimmune diseases: Guidelines from MATHEC-SFGM-TC].

The Francophone Society of Bone Marrow Transplantation and Cellular Therapy (SFGM-TC) organized the 13th workshop on hematopoietic stem cell transplantation clinical practices harmonization procedures in September 2022 in Lille, France. The aim of this workshop is to update the mobilization and conditioning protocols for autologous hematopoietic stem cell transplantation for autoimmune diseases, and to specify contraindications for transplant, conditioning regimen selection, immunosuppressive treatment discontinuation before mobilization and disease-specific surveillance.

Role of bronchoscopy for respiratory involvement in granulomatosis with polyangiitis and microscopic polyangiitis.

Objectives: This study describes data from bronchoscopy performed at the diagnosis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

Methods: We conducted a retrospective study between 2004 and 2019 in patients aged >18 years with a diagnosis of microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) who underwent bronchoscopy at onset of the disease. We collected bronchoalveolar lavage (BAL) and histological findings obtained during bronchoscopy.