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View article: Caractéristiques des patients présentant une rechute majeure dans le cadre d’une artérite à cellules géantes : une étude cas-témoins multicentrique
Caractéristiques des patients présentant une rechute majeure dans le cadre d’une artérite à cellules géantes : une étude cas-témoins multicentrique Open
View article: Health-related quality of life measurement in adults with sickle cell disease in steady state: Experience of one French reference center
Health-related quality of life measurement in adults with sickle cell disease in steady state: Experience of one French reference center Open
Sickle cell disease (SCD) is a genetic disease of public health concern. Adult patients face various disease-related complications, which affect their quality of life (QoL). Few studies have examined relationships between these events and …
View article: Chemotaxis overrides the killing response in alloreactive CTLs, providing vascular immune privilege during cellular rejection
Chemotaxis overrides the killing response in alloreactive CTLs, providing vascular immune privilege during cellular rejection Open
Graft endothelial cells (ECs) express donor alloantigens and encounter cytotoxic T lymphocytes (CTLs) but are generally spared during T cell-mediated rejection (TCMR), which predominantly affects epithelial structures. The mechanisms under…
View article: Évaluation des traitements anti-rhumatismaux modificateurs de la maladie de Still : étude rétrospective sur une cohorte de 187 patients
Évaluation des traitements anti-rhumatismaux modificateurs de la maladie de Still : étude rétrospective sur une cohorte de 187 patients Open
View article: 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
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 Open
View article: IA en santé : guide pour l’interniste averti
IA en santé : guide pour l’interniste averti Open
Artificial intelligence (AI) is experiencing considerable growth in medicine, driven by the explosion of available biomedical data and the emergence of new algorithmic architectures. Applications are rapidly multiplying, from diagnostic as…
View article: Flare of bullous lupus erythematosus in patients treated with anifrolumab for discoid lupus erythematosus: 2 cases
Flare of bullous lupus erythematosus in patients treated with anifrolumab for discoid lupus erythematosus: 2 cases Open
View article: Recurrent myocarditis revealing a desmoplakin cardiomyopathy successfully treated with anakinra
Recurrent myocarditis revealing a desmoplakin cardiomyopathy successfully treated with anakinra Open
View article: Vasodilator drugs and heart-related outcomes in systemic sclerosis: an exploratory analysis
Vasodilator drugs and heart-related outcomes in systemic sclerosis: an exploratory analysis Open
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 inv…
View article: Impact of autoantibody status on stratifying the risk of organ involvement and mortality in SSc: experience from a multicentre French cohort of 1605 patients
Impact of autoantibody status on stratifying the risk of organ involvement and mortality in SSc: experience from a multicentre French cohort of 1605 patients Open
Introduction Systemic sclerosis (SSc) is a rare autoimmune disease currently classified into two subgroups based on skin extension. The aim of this study was to determine in a large cohort whether the determination of autoantibody (AAb) pr…
View article: Impaired microvascular function in patients with sickle cell anemia and leg ulcers improved with healing
Impaired microvascular function in patients with sickle cell anemia and leg ulcers improved with healing Open
Summary Leg Ulcer (LU) pathophysiology is still not well understood in sickle cell anaemia (SCA). We hypothesised that SCA patients with LU would be characterised by lower microvascular reactivity. The aim of the present study was to compa…
View article: Contribution of major histocompatibility complex class II immunostaining in distinguishing idiopathic inflammatory myopathy subgroups: A histopathological cohort study
Contribution of major histocompatibility complex class II immunostaining in distinguishing idiopathic inflammatory myopathy subgroups: A histopathological cohort study Open
Idiopathic inflammatory myopathies (IIM) are rare, acquired muscle diseases; their diagnosis of is based on clinical, serological, and histological criteria. MHC-I-positive immunostaining, although non-specific, is used as a marker for IIM…
View article: Prevalence estimation of a rare disease with the French National Rare Disease Registry: example of TNF receptor associated periodic syndrome (TRAPS)
Prevalence estimation of a rare disease with the French National Rare Disease Registry: example of TNF receptor associated periodic syndrome (TRAPS) Open
Background rare diseases (RD) have progressively emerged as public health priority in many countries. Epidemiology still presents obstacles and extracting data from public health system remains insufficient. In France, RD database set up i…
View article: The CARMEN-France registry of adult patients with immune thrombocytopenia and autoimmune hemolytic anemia in France
The CARMEN-France registry of adult patients with immune thrombocytopenia and autoimmune hemolytic anemia in France Open
View article: Controversies in the pathophysiology of leg ulcers in sickle cell disease
Controversies in the pathophysiology of leg ulcers in sickle cell disease Open
Summary Patients with sickle cell disease (SCD) often experience painful vaso‐occlusive crises and chronic haemolytic anaemia, as well as various acute and chronic complications, such as leg ulcers. Leg ulcers are characterized by their un…
View article: Recurrent (or episodic) fever of unknown origin (FUO) as a variant subgroup of classical FUO: A French multicentre retrospective study of 170 patients
Recurrent (or episodic) fever of unknown origin (FUO) as a variant subgroup of classical FUO: A French multicentre retrospective study of 170 patients Open
This study extends the known yield of recurrent FUO and highlights the importance of repeated complete clinical examinations to discover potential diagnostic clues during follow-up. Moreover, their overall prognosis is excellent.
View article: Prognostic value of 18 FDG-PET at diagnosis and follow-up in giant cell arteritis: An observational restrospective study
Prognostic value of 18 FDG-PET at diagnosis and follow-up in giant cell arteritis: An observational restrospective study Open
PET vascular activity scores at diagnosis and at follow-up PET/CT performed outside a period of GCA activity do not display high performance to predict the occurrence of subsequent relapse in LV-GCA patients.
View article: Mepolizumab and benralizumab in patients with severe asthma and a history of eosinophilic granulomatosis with polyangiitis
Mepolizumab and benralizumab in patients with severe asthma and a history of eosinophilic granulomatosis with polyangiitis Open
Introduction Asthma associated with eosinophilic granulomatosis with polyangiitis (EGPA) is often severe and corticosteroid-dependent, leading to significant morbidity. Mepolizumab and benralizumab are humanized monoclonal antibodies targe…
View article: LBP2 Development and validation of a patient-centered autoevaluation questionnaire in systemic lupus erythematosus: LUPIN
LBP2 Development and validation of a patient-centered autoevaluation questionnaire in systemic lupus erythematosus: LUPIN Open
Objectives To develop and evaluate a self-administered questionnaire (LUPIN) to assess SLE disease activity and lupus-relevant PROs. Methods Patient representatives from a nationwide lupus association (AFL+) and lupus experts developed a s…
View article: Diagnostic challenges and updated therapeutic strategies of Kimura’s disease: A case report successfully treated by dupilumab and review
Diagnostic challenges and updated therapeutic strategies of Kimura’s disease: A case report successfully treated by dupilumab and review Open
Rationale: Kimura's disease (KD) is a rare and chronic eosinophilic related-disease, characterized by subcutaneous tissue masses, regional enlarged lymph nodes, hypereosinophilia and elevated serum IgE. KD usually affects young adults in t…
View article: Impact of COVID‐19 on incidence, clinical presentation, and prognosis of acute chest syndrome in patients with sickle cell disease
Impact of COVID‐19 on incidence, clinical presentation, and prognosis of acute chest syndrome in patients with sickle cell disease Open
Acute chest syndrome (ACS) is a frequent complication of sickle cell disease (SCD). Because coronavirus disease 2019 (COVID‐19) increases mortality and morbidity in many diseases, we retrospectively analyzed the impact of SARS‐CoV‐2 infect…
View article: Diagnostic value of elevated serum angiotensin-converting enzyme and lymphopenia in patients with granulomatous hepatitis.
Diagnostic value of elevated serum angiotensin-converting enzyme and lymphopenia in patients with granulomatous hepatitis. Open
Those results suggests that lymphopenia and sACE may be valuable biomarkers for sarcoidosis diagnosis in GH when combined, especially in younger patients.
View article: Inaugural dropped head syndrome and camptocormia in inflammatory myopathies: a retrospective study
Inaugural dropped head syndrome and camptocormia in inflammatory myopathies: a retrospective study Open
Objectives Inaugural axial muscle involvement, defined as dropped head syndrome (DHS) and/or camptocormia (CC), is poorly described in inflammatory myopathies (IM). This study aimed to further characterize IM patients with inaugural DHS/CC…
View article: Sickle cell disease and acute leukemia: one case report and an extensive review
Sickle cell disease and acute leukemia: one case report and an extensive review Open
View article: POS1283 HEART AND SYSTEMIC SCLEROSIS – FINDINGS FROM NATIONAL COHORT STUDY
POS1283 HEART AND SYSTEMIC SCLEROSIS – FINDINGS FROM NATIONAL COHORT STUDY Open
Background Heart involvement is one of the leading causes of death in systemic sclerosis (SSc). Cardiac manifestations are heterogeneous. In particular, prevalence of SSc-related cardiopathy is poorly known due to a lack of consensus in it…
View article: Occurrence of focal myositis during Behçet's disease: The identification of a specific vasculitis‐associated focal myopathy
Occurrence of focal myositis during Behçet's disease: The identification of a specific vasculitis‐associated focal myopathy Open
Aims This study aimed to report the association of focal myositis (FM) and Behçet's disease (BD) and to analyse the main characteristics of such an association. Methods This is a retrospective multicentre study of patients with BD and FM (…
View article: Mortality and Associated Causes in Hemophagocytic Lymphohistiocytosis: A Multiple-Cause-of-Death Analysis in France
Mortality and Associated Causes in Hemophagocytic Lymphohistiocytosis: A Multiple-Cause-of-Death Analysis in France Open
Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome with an overall mortality rate of 40%. A multiple-cause-of-death analysis allows for the characterization of mortality and associated causes over an extended p…
View article: Clinical severity and blood rheology in patients with sickle cell anaemia and co‐existing autoimmune disease
Clinical severity and blood rheology in patients with sickle cell anaemia and co‐existing autoimmune disease Open
Sickle cell anaemia (SCA) is characterised by chronic haemolysis, inflammation and the presence of poorly deformable red blood cells (RBCs). Any condition that may further impair blood rheology, increase haemolysis, oxidative stress and/or…
View article: Infection Risk in Patients with Dermatomyositis Associated with Anti-MDA5 Antibodies: A Historical Cohort Study
Infection Risk in Patients with Dermatomyositis Associated with Anti-MDA5 Antibodies: A Historical Cohort Study Open
Objective: Dermatomyositis associated with anti-MDA5 autoantibodies (DM-MDA5+) is a rare autoimmune disease usually characterized by skin involvement, often-severe lung involvement, and general features. Several reports of infections have …
View article: Input of Major Histocompatibility Complex class II immunostaining in idiopathic inflammatory myopathies
Input of Major Histocompatibility Complex class II immunostaining in idiopathic inflammatory myopathies Open
Background Idiopathic inflammatory myopathies (IIMs) are a group of rare acquired muscular diseases. In healthy muscle, myofibers do not express major histocompatibility complex (MHC) class I and II. It was established that MHC-I positive …