Publications by authors named "A Guffroy"
Purpose: Significant improvements in the prognosis for young patients with Primary Immunodeficiency Diseases (PID) and Autoinflammatory Disorders (AID), which together make up the majority of Inborn Errors of Immunity (IEI), have resulted in the need for optimisation of transition and transfer of care to adult services. Effective transition is crucial to improve health outcomes and treatment compliance among patients. Evaluations of existing transition programmes in European health centres identified the absence of disease-specific transition guidelines for PID and AID, as a challenge to the transition process.
View Article and Find Full Text PDFPrimary 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 of patients with triple-positive PAPS via combined single-cell RNA sequencing, B cell receptors (BCR) repertoire profiling, CITEseq analysis and single cell immortalization. We find that antiphospholipid (aPL)-specific B cells are present in the naive compartment, polyreactive, and derived from the natural repertoire.
View Article and Find Full Text PDFArticle Synopsis
- Hughes-Stovin syndrome (HSS) is a rare condition characterized by systemic vasculitis leading to both venous and arterial thrombosis, making it hard to differentiate from pulmonary embolism (PE) early on.
- This study compared 40 HSS patients to 50 PE patients, analyzing demographics, clinical findings, and results from computed tomography pulmonary angiography (CTPA).
- Results revealed that HSS patients were significantly younger and did not have co-morbidities like many PE patients; HSS showed unique vascular changes, including more frequent parenchymal hemorrhage and distinct pulmonary artery aneurysms.*
Introduction: Alveolar echinococcosis is an endemic parasitic disease prevalent in certain cold regions of the Northern Hemisphere, including Eastern France, Switzerland, Germany, Canada, and the United States. Widely underdiagnosed, it is associated with infection by Echinococcus multilocularis, a small tapeworm belonging to the cestode class, capable of causing multi-systemic involvement, particularly in elderly or immunocompromised patients.
Case Report: We present the case of an 82-year-old patient, immunocompromised due to prolonged corticosteroid therapy and treatment with dupilumab.