Given the overlapping clinical features of sino-nasal immunoglobulin 4-related disease (IgG4-RD) to rhinitis or rhinosinusitis, this paper aims to delineate this rare, isolated manifestation significant to physicians for their daily practice and researchers contributing to this field.
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http://dx.doi.org/10.1002/ccr3.4207 | DOI Listing |
Mod Rheumatol Case Rep
December 2024
Department of Rheumatology, Kobe City Medical Center General Hospital, Kobe, Hyogo, Japan.
IgG4-related disease (IgG4-RD) is a systemic, immune-mediated, fibroinflammatory disorder that affects multiple organs. Histopathologically, the supportive findings of IgG4-RD include dense lymphocytic infiltrates, obliterative phlebitis, storiform fibrosis, and elevated numbers of IgG4-positive plasma cells. However, the presence of granulomatous inflammation is generally considered highly atypical, suggesting alternative diagnoses such as sarcoidosis and lymphoma.
View Article and Find Full Text PDFJAMA Neurol
November 2024
Division of Neurology, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil.
Importance: Immunoglobulin G4 (IgG4)-related disease is an increasingly recognized fibroinflammatory condition that can involve multiple organs, including the pachymeninges. The understanding of IgG4-related pachymeningitis (IgG4-RP) remains limited because of its rarity and the predominance of knowledge derived from case reports and case series.
Objective: To systematically review and synthesize the clinical presentation, investigation findings, and prognosis of IgG4-RP to better understand its diagnosis and management.
Rev Esp Enferm Dig
September 2024
Gastroenterology, Unidade de Saúde Local do Algarve - Unidade de Faro, Portugal .
Diagnosing IgG4-related sclerosing cholangitis (IgG4-SC) presents significant challenges, especially when serum IgG4 levels are normal and other organs are not involved. We report a case of a 51-year-old patient with typical symptoms of biliary obstruction, including jaundice and abdominal pain. Despite normal serum IgG4 levels, imaging suggested cholangiocarcinoma, leading to extensive surgical intervention.
View Article and Find Full Text PDFSAGE Open Med Case Rep
August 2024
Department of Rheumatology, Veteran Affairs Central California Health System, Fresno, CA, USA.
IgG4-related disease is a fibroinflammatory condition characterized by dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells affecting multiple organs. Though the most common renal manifestation of IgG4-related disease is tubulointerstitial nephritis, it can rarely present as secondary membranous nephropathy. We present a case of a 75-year-old male with phospholipase A2 receptor-negative membranous nephropathy as an atypical manifestation of IgG4-related disease.
View Article and Find Full Text PDFJ Cutan Pathol
November 2024
Dermatology Department, Virgen del Rocío University Hospital, Seville, Spain.
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