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IgG4-Related Disease: Emerging Roles of Novel Genetic Variants, Immune Cell Subsets and Therapeutic Targets.
Allergy
August 2025
Centre for Cancer Biology, University of South Australia and SA Pathology, Adelaide, Australia.
IgG4, the least abundant IgG subclass in humans, is increasingly recognised for its involvement in allergic and autoimmune pathologies. Its unique properties, such as the tendency to form half-molecules (one heavy chain and one light chain) and its generally non-inflammatory nature, distinguish it from other IgG subclasses. Its role in immune dysregulation is further underscored by a distinct disease entity called IgG4-related disease (IgG4-RD), a systemic fibroinflammatory disorder characterised by IgG4 plasma cell tissue infiltration, storiform fibrosis and obliterative phlebitis, reflecting a dysregulated immune response affecting multiple organs.
View Article and Find Full Text PDFIgG4-related disease of the tubular gastrointestinal tract: A rare cause of lymphoplasmacytosis, eosinophilia, and mass-forming lesions.
Hum Pathol
September 2025
Department of Pathology, Division of Gastrointestinal and Liver Pathology, Johns Hopkins University, Sheikh Zayed Tower M2101B, 1800 Orleans Street, Baltimore, MD, 21231, USA. Electronic address:
IgG4-related disease (IgG4RD) is a systemic fibroinflammatory condition that can affect multiple organs, but rarely involves the tubular gastrointestinal (GI) tract. We identified patients with definitive or possible IgG4RD of the GI tract diagnosed at our institution. Our cohort included 8 patients with a median age of 37 years (range 15-73) and a slight male predominance (63 %).
View Article and Find Full Text PDFA Spindle Cell Tumor in Disguise: Histoid Nodules Arising From Peripheral Nerves.
Cureus
June 2025
Department of Radiodiagnosis, All India Institute of Medical Sciences, Bhopal, Bhopal, IND.
A 25-year-old male presented with multiple, painless subcutaneous nodules, each measuring 5-6 mm, attached to thickened superficial cutaneous nerves. He also had nodular infiltration of the left earlobe. Histopathology revealed spindle-shaped histiocytes in a storiform pattern, with numerous acid-fast bacilli in Fite-Faraco staining.
View Article and Find Full Text PDFImmunoglobulin G4-related disease of the gallbladder and the biliary tree.
J Gastrointest Surg
September 2025
Department of Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
Background: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a rare, immune-mediated fibroinflammatory disorder that can affect multiple organ systems, including the gallbladder and the biliary tree. This study aimed to characterize the clinical, imaging, and histopathologic features of IgG4-related biliary disease, including gallbladder involvement, and to review current medical and surgical management strategies.
Methods: A comprehensive review of the literature was conducted using PubMed and Embase databases for studies published between 2003 and 2025.
IgG4-related disease involving distinctive areas of skull base: our experience.
Eur Arch Otorhinolaryngol
May 2025
Department of ENT & HNS , All India Institute of Medical Sciences (AIIMS) Raipurs, Gate No. 1, AIIMS Raipur, Great Eastern Road, Tatibandh CG, Raipur, Chhattisgarh, 492099, India.
Background: Immunoglobulin G4-related disease (IgG4-RD) is a rare, systemic fibro-inflammatory condition characterized by lymphoplasmacytic infiltration with IgG4-positive plasma cells, storiform fibrosis, and elevated serum IgG4 levels. It can affect multiple organ systems, often mimicking malignancies or infections. Skull base involvement in IgG4-RD is uncommon and presents diagnostic and therapeutic challenges.
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