Immunoglobulin G4-related Disease: A Series of Four Cases
Published: March 1, 2024 | DOI: https://doi.org/10.7860/JCDR/2024/67738.19190
Chandni Thomas, S Divya, Joy Augustine
1. Junior Resident, Department of Pathology, Amala Institute of Medical Sciences, Thrissur, Kerala, India.
2. Associate Professor, Department of Pathology, Amala Institute of Medical Sciences, Thrissur, Kerala, India.
3. Professor and Head, Department of Pathology, Amala Institute of Medical Sciences, Thrissur, Kerala, India.
Correspondence
Dr. Chandni Thomas,
Junior Resident, Department of Pathology, Amala Institute of Medical Sciences, Thrissur-680001, Kerala, India.
E-mail: chandnithomaz@gmail.com
Immunoglobulin G4-related Disease (IgG4-RD) is an immune-mediated fibroinflammatory disease that affects multiple organs, resulting in tumefactive lesions and/or organ dysfunction. This chronic, multiorgan inflammatory process is characterised by the infiltration of IgG4-positive plasma cells and has a variable clinical presentation depending on the organ involved. The present case series discusses four cases (two males and two females) of IgG4-RD involving different sites in patients presented to present Institution. The authors reviewed four cases of IgG4-RD, including clinical details, biochemical, radiological, and histopathological features. The case series includes IgG4-RD masquerading as meningioma, IgG4-related sclerosing cholangitis, IgG4-RD of the thyroid gland, and IgG4-RD of the lacrimal gland. Since IgG4-RD has non specific clinical features, histopathological analysis and immunohistochemistry play a pivotal role in diagnosis. Despite its diagnostic difficulties, earlier recognition is crucial to prevent significant morbidity and extensive fibrosis leading to organ failure.
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