Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is recognized as a syndrome caused by multiple pathologies. Since the 2010s, it has been clarified that autoantibodies against membranous proteins localized in the nodes of Ranvier and paranodes are positive in subsets of CIDP patients, leading to proposing a new disease concept called autoimmune nodopathies, which is independent of CIDP, in the revised international CIDP guidelines. This article reviews the significance of these autoantibodies, especially anti-neurofascin 155 and anti-contactin 1 antibodies, which have been the most prevalent and achieved a higher degree of consensus.
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http://dx.doi.org/10.5692/clinicalneurol.cn-001878 | DOI Listing |
Ann Med
December 2025
Department of Hematology, The First Affiliated Hospital of Zhejiang Chinese Medical University (Zhejiang Provincial Hospital of Chinese Medicine), Hangzhou, China.
Objectives: Platelet transfusion refractoriness (PTR) is a frustrating clinical problem, and primary and persistent (P/P) PTR who experienced persistent PTR since the first transfusion was failed to be well recognized. This study aims to investigate the incidence and risk factors for P/P PTR.
Methods: Patients with hematologic disorders who underwent HLA high-resolution genotyping and donor-specific HLA antibody or panel reactive antibody (PRA) testing between January 2019 and March 2023 were reviewed.
Neurol Int
December 2024
Department of Immunology, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iași, Romania.
: Several significant associations between certain Human Leukocyte Antigen (HLA) alleles and myasthenia gravis (MG) subtypes were established in populations from Western Europe and North America and, to a lesser extent, from China and Japan. However, such data are scarcely available for Eastern Europe. This study aimed to analyze the associations of HLA Class I and II alleles with MG and its serological subtypes (with anti-acetylcholine receptor autoantibodies, RAch+MG, and double-seronegative, dSNMG) in myasthenic patients of Romanian descent.
View Article and Find Full Text PDFBrain Nerve
January 2025
Internal Medicine, Morioka Health Cooperative Association, Kawakubo Hospital.
Anti-myelin-associated glycoprotein (Anti-MAG) neuropathy and autoimmune nodopathies with antibodies targeting nodal or paranodal proteins have recently been reclassified as distinct conditions, separate from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). This distinction is based on the clinical homogeneity observed in antibody-positive cases, their unique response to treatment compared to CIDP, and evidence indicating the pathogenic role of these autoantibodies. The significance of identifying conditions outside the CIDP category lies in the elucidation of their distinct pathological mechanisms and providing appropriate immunotherapy accordingly.
View Article and Find Full Text PDFPediatr Nephrol
December 2024
Néphrologie Pédiatrique, Centre de Référence du Syndrome Néphrotique Idiopathique de L'enfant Et L'adulte, Hôpital Necker - Enfants Malades, APHP, Inserm U1163, Institut Imagine, Université Paris Cité, Paris, France.
Childhood idiopathic nephrotic syndrome is an important pediatric kidney disease associated with significant morbidities and even mortality. Several guidelines have been developed to standardize the terminology and patient care among the pediatric nephrology community. Since the publication of these guidelines, there have been major breakthroughs in the disease management and the understanding of underlying pathogenesis through multi-omics investigations, including the identification of anti-nephrin autoantibodies, genetic susceptibility loci, and the pathogenic role of B cell subsets.
View Article and Find Full Text PDFAm J Clin Pathol
December 2024
Department of Pathology and Laboratory Medicine, Emory University Hospital, Atlanta, GA, US.
Objectives: Atrophic gastritis (AG) is characterized by atrophy of gastric glands-in particular, oxyntic glands-in the setting of chronic inflammation; it is often autoimmune. The diagnosis is confirmed by immunohistochemistry (IHC) for gastrin (to confirm biopsy site), and pathologists often use IHC for neuroendocrine markers to evaluate for enterochromaffin-like cell hyperplasia (ECL-H). The utility of neuroendocrine staining is unclear, and we undertook this study to determine whether ECL pattern provided any additional information in cases of Helicobacter-negative AG.
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