A5011553838- Inflammatory Myopathies and Dermatomyositis
- Systemic Sclerosis and Related Diseases
- Systemic Lupus Erythematosus Research
- Diabetes and associated disorders
- Immunodeficiency and Autoimmune Disorders
- Eosinophilic Disorders and Syndromes
- Monoclonal and Polyclonal Antibodies Research
- Sarcoidosis and Beryllium Toxicity Research
- Salivary Gland Disorders and Functions
- Liver Diseases and Immunity
- Vasculitis and related conditions
- Chronic Lymphocytic Leukemia Research
- Platelet Disorders and Treatments
- Lymphoma Diagnosis and Treatment
- Skin Diseases and Diabetes
- Long-Term Effects of COVID-19
- Pathogenesis and Treatment of Hiccups
- Kawasaki Disease and Coronary Complications
- Amoebic Infections and Treatments
- Protein purification and stability
- IgG4-Related and Inflammatory Diseases
- Parasitic Infections and Diagnostics
- Parkinson's Disease and Spinal Disorders
- COVID-19 Clinical Research Studies
- Chronic Myeloid Leukemia Treatments
Université de Strasbourg
2015-2024
Hôpitaux Universitaires de Strasbourg
2015-2024
Hôpital Civil, Strasbourg
2016-2024
Inserm
2023
Objective To refine the spectrum of anti-Ku-associated disease, a condition that is equivocally described by current diagnostic criteria for connective tissue diseases. Methods Among 42 consecutive patients harbouring anti-Ku antibodies, subgroups with similar phenotypes and prognosis were delineated without an priori diagnosis using hierarchical clustering analysis cumulative clinico-biological features recorded during follow-up. Features present at baseline most efficiently predicted...
The Nck-associated protein 1–like (NCKAP1L) gene, alternatively called hematopoietic 1 (HEM-1), encodes a lineage–specific regulator of the actin cytoskeleton. Nckap1l-deficient mice have anomalies in lymphocyte development, phagocytosis, and neutrophil migration. Here we report, for first time, NCKAP1L deficiency cases humans. In two unrelated patients Middle Eastern origin, recessive mutations abolishing expression led to immunodeficiency, lymphoproliferation, hyperinflammation with...
Abstract Objectives Antinuclear antibodies (ANA) are important for the diagnosis of various autoimmune diseases. ANA usually detected by indirect immunofluorescence assay (IFA) using HEp-2 cells (HEp-2 IFA). There many variables influencing IFA results, such as subjective visual reading, serum screening dilution, substrate manufacturing, microscope components and conjugate. Newer developments on testing that offer novel features adopted some clinical laboratories include automated...
<h3>Objective</h3> To refine the predictive significance of muscle granuloma in patients with myositis. <h3>Methods</h3> A group 23 myositis and on biopsy (granuloma-myositis) from 8 French Belgian centers was analyzed compared (1) a without identified (control-myositis) randomly sampled each center (2) 20 sporadic inclusion body (sIBM) (control-sIBM). <h3>Results</h3> All but 2 granuloma-myositis had extramuscular involvement, including signs common sarcoidosis that were systematically...
To refine the prevalence, characteristics and response to treatment of myositis in primary SS (pSS).The multicentre prospective Assessment Systemic Signs Evolution Sjögren's Syndrome (ASSESS) cohort 395 pSS patients with ≥60 months' follow-up was screened by 2017 EULAR/ACR criteria for myositis. Extra-muscular complications, disease activity patient-reported scores were analysed.Before enrolment during 5-year follow-up, suspected 38 confirmed 4 [1.0% (95% CI: 0.40, 2.6)]. Patients but not...
Dear Editor, Anti-eukaryotic initiation factor 2B (eIF2B) autoantibodies are a recently described autoantibody specificity in SSc associated with diffuse cutaneous involvement, interstitial lung disease (ILD) and cytoplasmic pattern on HEp-2 indirect immunofluorescence (HEp-2 IIF) [1–3]. Hitherto only 19 patients have been identified, all by radiolabelled protein immunoprecipitation Here we describe immunoprecipitation-mass spectrometry (IP-MS) as detection method for anti-eIF2B further...
A 72-year-old man consulted in November 2012 for abdominal pain the right upper quadrant. The patient had a history of suspected hepatic amebiasis treated Senegal 1985 and has not traveled to endemic areas since 1990. Abdominal CT scan revealed liver abscess. At first, no parasitological tests were performed was with broad-spectrum antibiotics. Only after failure this therapy, serology PCR abscess puncture established diagnosis amebiasis. metronidazole tiliquinol-tilbroquinol. Amebic is most...
Objective Silica is an environmental substance strongly linked with autoimmunity. The aim of this study was to assess the prevalence antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV), including granulomatosis polyangiitis (GPA), microscopic (MPA), and renal limited vasculitis, in a northeastern region France evaluate whether there geospatial association between localization quarries these AAVs. Methods Potential AAV patients were identified using 3 sources: hospital...
Objective Inflammatory myopathies (IM), characterized by muscle inflammation and weakness, are rare systemic diseases. Our previous study estimated an IM incidence rate of 7.98 cases per million people year (95% confidence interval 7.38–8.66) highlighted important variations that were likely because methodologic issues rather than true epidemiologic differences. In this study, we aimed to refine the IM, using 2017 European League Against Rheumatism (EULAR)/American College Rheumatology (ACR)...
We recently recorded a high prevalence of inclusion body myositis (IBM) in patients with Sjögren's syndrome (SS). Whether SS differ from without terms the characteristics is currently unknown. Anti-cytosolic 5'-nucleotidase 1 A (cN1A) has been proposed as biomarker for IBM but also frequent SS. anti-cN1A independently associated still an open question. aimed to assess significance and patients.Cumulative data on all (EULAR/ACR 2017 criteria) screened (ACR/EULAR 2016 single centre were...
Primary antiphospholipid syndrome (PAPS) is a life-threatening clotting disorder mediated by pathogenic autoantibodies. Here we dissect the origin of self-reactive B cells in human PAPS using peripheral blood and bone marrow patients with triple-positive via combined single-cell RNA sequencing, cell receptors (BCR) repertoire profiling, CITEseq analysis single immortalization. We find that (aPL)-specific are present naive compartment, polyreactive, derived from natural repertoire....
Systemic sclerosis is a rare systemic autoimmune disease characterized by microvascular impairment and fibrosis of the skin other organs with poor outcomes. Toxic causes may be involved. We reported case 59-year-old woman who developed an acute after two doses adjuvant chemotherapy docetaxel cyclophosphamide for localized hormone receptor + human epithelial 2-breast cancer. Docetaxel major drug used in treatment breast, lung, prostate cancers, among others. Scleroderma-like skin-induced...
<br><b>Aim:</b> Antinuclear antibodies (ANA) are important for the diagnosis of various autoimmune diseases. ANA usually detected by indirect immunofluorescence assay (IFA) using HEp-2 cells (HEp-2 IFA). There many variables influencing IFA results, such as subjective visual reading, serum screening dilution, substrate manufacturing, microscope components and conjugate. Newer developments on testing that offer novel features adopted some clinical laboratories include...
Le système du complément est composé d'un ensemble de protéines plasmatiques ou membranaires.Les déficits en peuvent être héréditaires acquis fait la présence d'auto-anticorps par consommation.Nous avons évalué les performances analytiques l'automate Optilite ® pour le dosage des fractions C3 et C4 l'évaluation l'activité total.Les CV intra-et inter-séries concordances avec automates utilisés au laboratoire (BNII BCT , Siemens) sont satisfaisantes tout comme concordance entre échantillons...
The most common cause of acute renal failure in systemic sclerosis patients is scleroderma crisis but other etiologies have to be considered such as another autoimmune disease. We report the case a 60-year-old male admitted our hospital with failure. His medical history included diagnosis 6 months ago. Antinuclear antibodies were positive at titer 1:1280 anti-Scl-70 and anti-myeloperoxidase (34 U/mL) antibodies. Scleroderma was suspected. However, antineutrophil cytoplasmic...