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Pan-neurofascin autoimmune nodoparanodopathy: A case report and literature review.
Medicine (Baltimore)
January 2025
Department of Neurology (Nerve-Muscle Unit), Reference Center for Neuromuscular Diseases "AOC," ALS Reference Center, University Hospitals of Bordeaux (Pellegrin Hospital), University of Bordeaux, Bordeaux, France.
Rationale: Locked-in syndrome (and its variant, completely locked-in state) generally has a high mortality rate in the acute setting; however, when induced by conditions such as acute inflammatory polyradiculoneuropathy, it may well be curable such that an attempt at cure should be systematically sought by clinicians.
Patient Concerns: A 52-year-old man presented with acute tetraparesia and areflexia, initially diagnosed as Guillain-Barré syndrome. Despite appropriate treatment, his condition deteriorated, evolving into a completely locked-in state.
Associated factors related to production of autoantibodies and dermo-epidermal separation in bullous pemphigoid.
Arch Dermatol Res
January 2025
Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, 9 Dongdan 3rd Alley, Beijing, 100730, China.
Bullous pemphigoid (BP) is a debilitating autoimmune skin blistering disease, characterized by the deposition of specific autoantibodies at the dermal-epidermal junction. This leads to an inflammatory cascade involving the activation of complement proteins, mast cell degranulation, immune cell recruitment, and the release of proteases by granulocytes. While several cytokines and signaling pathways have been implicated in the pathogenesis of BP, the precise mechanism behind autoantibody production remains unclear.
View Article and Find Full Text PDFPatients with antiphospholipid antibodies preferentially seek health information from physicians: a cross-sectional online patient quantitative survey.
Rheumatol Int
January 2025
Division of Rheumatology, Department of Medicine, University of Calgary, Cumming School of Medicine, Calgary, AB, Canada.
Little is known about how patients with antiphospholipid syndrome (APS) or antiphospholipid antibodies (aPL) access and trust health information. This research aimed to: describe the sources of information most frequently accessed/trusted by patients with APS/aPL; identify if individuals with APS/aPL perceived their health had been negatively impacted by various sources and document obstacles to accessing health information. Patients meeting Revised Sapporo Criteria for APS or with ≥1 positive aPL on ≥2 occasions were recruited to an online survey regarding their health information use at diagnosis and within 6 months preceding survey completion.
View Article and Find Full Text PDFImpact of Thrombopoietin Receptor Agonists on Pathophysiology of Pediatric Immune Thrombocytopenia.
Curr Issues Mol Biol
January 2025
Children & Adolescent Hematology-Oncology Unit, Second Department of Pediatrics, School of Medicine, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece.
Immune thrombocytopenia (ITP) in pediatric patients is a common cause of isolated thrombocytopenia. Various pathophysiological mechanisms are implicated in ITP pathogenesis, including the production of autoantibodies against components of platelets (PLTs) by B-cells, the activation of the complement system, phagocytosis by macrophages mediated by Fcγ receptors, the dysregulation of T cells, and reduced bone marrow megakaryopoiesis. ITP is commonly manifested with skin and mucosal bleeding, and it is a diagnosis of exclusion.
View Article and Find Full Text PDFImmunohistochemistry for Immunoglobulin G4 on Paraffin Sections as a Diagnostic Test for Pemphigus.
Am J Dermatopathol
February 2025
Department of Dermatology and Venereology, Faculty of Medicine, Medical University of Plovdiv, Plovdiv, Bulgaria.
Pemphigus is a group of autoimmune bullous diseases mediated by autoantibodies most often of the immunoglobulin G class, subclasses immunoglobulin G1, and immunoglobulin G4 (IgG4), directed against desmosomal adhesion proteins of keratinocytes. This study aimed to evaluate IgG4 immunoreactivity on paraffin sections using immunohistochemistry in patients with pemphigus as a diagnostic test. Fifty formalin-fixed paraffin-embedded specimens from patients with pemphigus were selected.
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