1,154 results match your criteria: "Internal Medicine and Clinical Immunology[Affiliation]"

Haematological features of telomere biology disorders diagnosed in adulthood: A French nationwide study of 127 patients.

Br J Haematol

November 2024

Hematology and Transplant Unit, French Reference Center for Aplastic Anemia, Saint-Louis Hospital, AP-HP, Université Paris Cité, Paris, France.

Article Synopsis
  • The study examines the blood-related characteristics and overall prognosis of 127 patients with telomere biology disorders (TBD) who were diagnosed after age 15, highlighting a lack of data on this topic.
  • At diagnosis, significant haematological issues were present in nearly 76% of patients, with bone marrow failure (BMF) being the most common, affecting 46.5% of the cases, while some patients also developed additional complications over time.
  • The findings suggest that BMF patients tend to be younger and have a better survival rate compared to those with higher-risk blood cancers, indicating TBD as a complex multi-organ disease needing further research on its evolutionary nature and outcomes.
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Characteristics and outcomes associated with CD2 and CD25 expression on bone marrow mast cells in patients with systemic mastocytosis.

Haematologica

January 2025

Université Paris Cité, INSERM UMR-S1151, CNRS UMR-S8253, Institut Necker Enfants Malades, F-75015 Paris, France; Hôpital Necker Enfants-Malades, Laboratoire d'Onco-Hématologie, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris.

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Background: Biological therapies, such as mepolizumab, have transformed the treatment of severe eosinophilic asthma. Although mepolizumab's short-term effectiveness is established, there is limited evidence on its ability to achieve long-term clinical remission.

Objective: To evaluate the long-term effectiveness and safety of mepolizumab, explore its potential to induce clinical and sustained remission, and identify baseline factors associated with the likelihood of achieving remission over 24 months.

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French protocol for the diagnosis and management of systemic lupus erythematosus.

Rev Med Interne

September 2024

Department of Internal Medicine and Clinical immunology, Reference Centre of Autoimmune Systemic Rare Diseases of North and North-West of France (CeRAINO), Lille University, Inserm, University Hospital of Lille, Lille, France.

Because Systemic Lupus Erythematosus (SLE) is a rare disease, and due to the significant prognostic impact of early management, a diagnosis confirmed by a physician with experience in SLE is recommended, for example from an expert center. Once the diagnosis is confirmed, existing manifestations should be identified in particular, renal involvement by an assessment of proteinuria, disease activity and severity should be determined, potential complications anticipated, associated diseases searched for, and the patient's socioprofessional and family context noted. Therapeutic management of SLE includes patient education on recognizing symptoms, understanding disease progression as well as when they should seek medical advice.

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Treatment modalities of marginal zone lymphoma and overall survival, haematological response, and underlying Sjögren's disease activity: a multicentre, retrospective, observational study.

Lancet Rheumatol

October 2024

Department of Rheumatology, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, Le Kremlin-Bicêtre, Paris, France; Center for Immunology of Viral Infections and Auto-Immune Diseases, INSERM UMR 1184, Université Paris-Saclay, Le Kremlin-Bicêtre, Paris, France. Electronic address:

Article Synopsis
  • Sjögren's disease is an autoimmune condition with a significant risk of developing lymphoma, but effective management strategies for this dual challenge are not well established.
  • A study was conducted involving 106 patients who had both Sjögren's disease and lymphoma, focusing on understanding treatment approaches and outcomes related to lymphoma progression and Sjögren's disease symptoms.
  • The findings highlighted that mucosa-associated lymphoid tissue lymphoma was the most common type in these patients, and specific factors were analyzed to assess their impact on patients’ prognosis and survival rates.
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When and which second-line workup to perform for uveitis: a tertiary care center experience.

Can J Ophthalmol

August 2024

Department of Internal Medicine and Clinical Immunology, Nancy University Hospital, Nancy, France; UMR 7365, Ingénierie Moléculaire, Cellulaire et Physiopathologie, Lorraine University, Centre National de la Recherche Scientifique, Nancy, France. Electronic address:

Objective: Uveitis are inflammatory disorders of various etiologies. The first-line etiological workup is currently poorly codified, and some patients undergo sequential investigations. However, what leads the clinician to perform subsequent exams and the relevance of such exams remain to be determined.

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Objectives: The aim of this study was to determine the association between different histological patterns and prognosis in patients with SSc and histologically proven muscle involvement.

Methods: A multicentre retrospective study was conducted of a cohort of scleroderma patients who had undergone muscle biopsy. The biopsies were reviewed in a coordinated manner to classify patients based on histological findings.

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The innate immune system, composed of neutrophils, basophils, eosinophils, myeloid-derived suppressor cells (MDSCs), macrophages, dendritic cells (DCs), mast cells (MCs), and innate lymphoid cells (ILCs), is the first line of defense. Growing evidence demonstrates the crucial role of innate immunity in tumor initiation and progression. Several studies support the idea that innate immunity, through the release of pro- and/or anti-inflammatory cytokines and tumor growth factors, plays a significant role in the pathogenesis, progression, and prognosis of cutaneous malignant melanoma (MM).

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Article Synopsis
  • Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) often overlap, but PMR lacks the vascular inflammation present in GCA, suggesting different underlying mechanisms.
  • *In a study involving 45 isolated PMR patients and 29 PMR/GCA overlap patients, various serum biomarkers related to inflammation and vascular function were measured to differentiate between the two conditions before starting treatment.
  • *Results indicated that certain biomarker ratios, specifically CXCL9/IL-6 and MMP-3/sCD141, were more effective at diagnosing GCA in patients with isolated PMR, demonstrating the potential to guide further examination for those at risk.*
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Article Synopsis
  • Acid sphingomyelinase deficiency (ASMD) is a serious genetic disease that can affect people from childhood to adulthood, caused by problems with a gene called SMPD1.
  • A study reviewed medical records from 27 hospitals in France to learn more about the health and survival of patients with ASMD from 1990 to 2020.
  • The results showed that patients with type A usually did not live past early childhood, while those with type B lived longer, but there were still risks of early death from serious illnesses like neurodegeneration and cancer.
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Article Synopsis
  • - Hypereosinophilic syndrome (HES) is a group of diverse disorders characterized by high levels of eosinophils, with ongoing challenges in diagnosis, understanding, and personalized treatment still needing attention.
  • - There is an urgent need to reduce the time it takes to diagnose and begin treatment for HES, as the condition significantly affects patients' quality of life.
  • - The Italian Society of Allergy, Asthma, and Clinical Immunology (SIAAIC) has launched a national initiative, the InHES network, to unify medical efforts and improve communication about HES, including sharing recent research findings and treatment recommendations.
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Discovery of new myositis genetic associations through leveraging other immune-mediated diseases.

HGG Adv

October 2024

Cambridge Institute of Therapeutic Immunology and Infectious Disease (CITIID), University of Cambridge, Cambridge, UK; Department of Medicine, University of Cambridge, Cambridge, UK; MRC Biostatistics Unit, University of Cambridge, Cambridge, UK.

Article Synopsis
  • Genome-wide association studies (GWASs) are effective in linking genetic variants to various human traits and diseases, but they often require large sample sizes, making it tough to study rarer diseases like myositis, which severely affect patients' quality of life.
  • The researchers used a feature engineering method to leverage data from larger IMD GWASs, discovering 17 immune-mediated diseases genetically related to myositis, including conditions like systemic sclerosis and Sjögren's syndrome.
  • They identified seven potential new genetic links to myositis, hinting that immune system genes may play a role in the disease, and suggest this method could enhance genetic research in other rare conditions as well.
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Article Synopsis
  • Immune checkpoint inhibitors (ICIs) like pembrolizumab have notable clinical advantages but can cause serious side effects known as immune-related adverse events (irAEs), affecting various organ systems.
  • A case study of a female patient revealed that she experienced myxedema coma (MC) and adrenal insufficiency (AI) four months after stopping treatment with pembrolizumab, necessitating treatment with levothyroxine and hydrocortisone.
  • This highlights the need for careful monitoring of patients on ICIs for potential endocrine disorders, as they can lead to severe health complications if not detected early.
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Tinzaparin, an alternative to subcutaneous unfractionated heparin, in patients with severe and end-stage renal impairment: a retrospective observational single-center study.

J Thromb Haemost

October 2024

Institut National de la Santé et de la Recherche Médicale (INSERM)-1059 SAnté INgéniérie BIOlogie St-Etienne (SAINBIOSE), Jean Monnet University, Mines Saint-Étienne, France; Department of Pharmacology, Saint-Étienne University Hospital, Saint-Etienne, France.

Article Synopsis
  • Tinzaparin may offer a more manageable treatment option than unfractionated heparin for patients with severe renal impairment, but there's limited data on its pharmacokinetics and complications in this group.
  • This study aimed to analyze the pharmacokinetics of tinzaparin and evaluate bleeding and thrombotic complications in patients with an estimated glomerular filtration rate (eGFR) under 30 mL.min⁻.
  • Results showed that tinzaparin's pharmacokinetics were similar to those in patients without renal impairment, with low rates of major bleeding and no thrombotic complications, indicating it could be a viable alternative for these patients.
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Methotrexate versus conventional disease-modifying antirheumatic drugs in the treatment of non-anterior sarcoidosis-associated uveitis.

Br J Ophthalmol

December 2024

Department of Internal Medicine and Clinical Immunology, Centre National de Références Maladies Autoimmunes et Systémiques Rares, Centre National de Références Maladies Autoinflammatoires Rares et Amylose Inflammatoire and INSERM, UMR S 959, Immunology- Immunopathology-Immunotherapy, Sorbonne Universités, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.

Aims: To compare the safety and efficacy of methotrexate (MTX), mycophenolate mofetil (MMF) and azathioprine (AZA) in non-anterior sarcoidosis-associated uveitis.

Methods: Retrospective study including non-anterior sarcoidosis-associated uveitis according to the revised International Workshop on Ocular Sarcoidosis criteria. The primary outcome was defined as the median time to relapse or occurrence of serious adverse events leading to treatment discontinuation.

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Article Synopsis
  • * A study used advanced techniques to analyze muscle biopsy samples from 26 patients, revealing common features such as heightened immune response markers, myofiber damage, and significant protein accumulation related to cell stress.
  • * Findings indicated that anti-Ku myositis displays unique histopathological characteristics, distinguishing it from other conditions like inclusion body myositis and immune-mediated necrotizing myopathy, while also showing connections to specific syndromes like systemic sclerosis.
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Long-term outcomes of childhood-onset systemic lupus erythematosus.

Rheumatology (Oxford)

July 2024

Department of Pediatric Immunology, Hematology and Rheumatology, Necker Hospital, AP-HP, National Reference Centre for Rheumatic and Autoimmune Diseases in Children, RAISE, Paris and Lyon, France, Institut IMAGINE, Université Paris-Cité, Paris, France.

Objective: Data on the long-term outcome of patients with childhood-onset Systemic Lupus Erythematosus (cSLE) are scarce. Aims of this study were to describe the long-term outcomes of cSLE and to identify factors associated with the development of damage and persistent disease activity.

Methods: We conducted a retrospective multicentre study using data from the PEDIALUP registry of the Juvenile Inflammatory Rheumatism (JIR) cohort database.

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Cluster analysis of clinical manifestations assigns systemic lupus erythematosus-phenotype subgroups: A multicentre study on 440 patients.

Joint Bone Spine

December 2024

National Referral Centre for Rare Autoimmune and Systemic Diseases, Department of Internal Medicine, université de Paris, hôpital Cochin, AP-HP Centre, Paris, France; Unité Inserm 1153, centre de recherche en épidémiologie et statistiques (CRESS), université de Paris, Paris, France.

Article Synopsis
  • - The study aims to categorize systemic lupus erythematosus (SLE) patients into distinct phenotypes based on various clinical and laboratory characteristics, excluding autoantibody profiles.
  • - Researchers analyzed data from 440 SLE patients, mainly women, and identified three subgroups: one with severe manifestations linked to antiphospholipid syndrome, another with mild articular symptoms, and a third with diverse clinical features and high autoantibody positivity.
  • - The findings emphasize the heterogeneity of SLE, suggesting that different patient subgroups may require tailored treatment approaches based on their specific disease manifestations.
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Impact of conventional and biological disease-modifying anti-rheumatic drugs on arterial lesions in Takayasu arteritis.

Rheumatology (Oxford)

July 2024

Sorbonne Universités, Department of Internal Medicine and Clinical Immunology, Centre de Référence des Maladies Auto-Immunes Systémiques Rares, Centre de Référence des Maladies Auto-Inflammatoires et de l'Amylose inflammatoire (CEREMAIA), F-75013, Paris, France; INSERM, UMR_S 959, F-75013, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Paris, DMU 3ID, France.

Background: The definition of Takayasu arteritis (TAK) remission and disease activity is still unclear. Vascular imaging is an essential tool for following-up patients. Herein, we aimed to compare the evolution of vascular lesions (ie vessel wall thickening and stenosis) under conventional disease-modifying anti-rheumatic drugs (cDMARDs) relatively to biological DMARDs (bDMARDs) in TAK patients followed with the same CT angiography modalities.

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Occurrence of in vivo carbapenem-resistant Campylobacter coli mediated by porA point mutation and overexpression of bla under meropenem treatment.

Clin Microbiol Infect

November 2024

INSERM U1311 DynaMicURe, Normandie University, UNICAEN, UNIROUEN, Caen, France; Department of Infectious Agents, Normandie Univ, UNICAEN, CHU de Caen Normandie, Bacteriology, Caen, France.

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Neuro-Behçet's disease: A review.

Rev Med Interne

October 2024

Department of Internal Medicine and Clinical Immunology, Centre de Référence des Maladies Auto-Immunes Systémiques Rares, Centre de Référence des Maladies Auto-Inflammatoires et de l'Amylose inflammatoire (CEREMAIA), Sorbonne Universités, 75013 Paris, France; Inserm, UMR_S 959; DMU 3ID, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, 75013 Paris, France.

Behcet disease (BD) is a systemic vasculitis which can involve many different organ systems. Neurological involvement (NBD) occurs in 5.3% to 59% of BD patients.

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Unexpected discovery of CRP analytical interference: A case report.

Clin Biochem

August 2024

Biochemistry Department, Federative Institute of Biology, Toulouse University Hospital, Toulouse, France. Electronic address:

Objectives: Immunoturbidimetric assays are sensitive techniques in clinical biology that may be subjected to matrix effects, hook effects or aspecific reactions. Among these, large quantities of immunoglobulins can distort the intensity of the detected signal. This study illustrates the deleterious effect of analytical interference on clinical patient management, and assesses the practical relevance of a recently proposed algorithm for interference investigation.

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Myocarditis is an inflammatory disease of the cardiac muscle that manifests as chest pain, dyspnea, and other signs of heart failure. ST segment changes with elevated cardiac biomarkers mimic acute coronary syndromes. It is most commonly caused by viruses like the Epstein-Barr virus (EBV) and Coxsackie B virus, but it can also be due to cardiotoxic drugs like cyclophosphamide and cocaine or caused by a systemic infiltrative process like sarcoidosis or collagen vascular diseases.

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