Background: Immunoglobulin (Ig)G4-related disease affects nearly every organ, and its clinical course varies depending on the involved organ; however, its occurrence in the mediastinum is rarely reported.
Case Presentation: A 58-year-old woman presented with a posterior mediastinal tumor along the thoracic spine on imaging. Based on her elevated serum IgG4 level of 349.7 mg/dL, IgG4-related disease was suspected. Since the tumor was growing and malignancy could not be excluded, surgical resection was performed for definitive diagnosis. Robot-assisted thoracoscopic surgery was performed via the left semipronation and right thoracic approaches. The irregularly-shaped tumor was located on the level of the seventh to ninth thoracic vertebra, along the sympathetic nerve. A malignancy was not excluded based on the appearance of the tumor. The tumor had poor mobility. The sympathetic nerves, intercostal arteries, and veins were also excised. In this case, the articulated forceps, used during the robotic surgery, were useful in achieving complete tumor resection along the vertebral body. The pathological examination revealed IgG4-positive plasma infiltration, which fulfilled the criteria for IgG4-related diseases. The postoperative course was uneventful, and the patient underwent follow-up on an outpatient basis without additional medications.
Conclusion: The clinical presentation of IgG4-related disease varies, based on the involved organs. This case was rare because the mediastinum was involved, and it emphasized the effectiveness of surgical resection.
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http://dx.doi.org/10.1186/s13019-024-02655-5 | DOI Listing |
Int Immunopharmacol
January 2025
Department of Rheumatology and Clinical Immunology, Department of Health Management, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, China; Department of Health Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China. Electronic address:
Medicina (Kaunas)
January 2025
Department of Internal Medicine (Nephrology), Faculty of Medicine, Ufuk University, 06510 Ankara, Turkey.
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated, fibroinflammatory, multiorgan disease with an obscure pathogenesis. Findings indicating excessive platelet activation have been reported in systemic sclerosis, which is another autoimmune, multisystemic fibrotic disorder. The immune-mediated, inflammatory, and fibrosing intersections of IgG4-RD and systemic sclerosis raised a question about platelets' role in IgG4-RD.
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Nephrology, Ningbo No.2 Hospital, Ningbo, Zhejiang, China.
Background: Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibrous inflammatory disease. Recently, an association between IgG4-RD and tuberculosis (TB) has been reported.
Case Summary: We report a 56-year-old man complaining of a cough and poor appetite for 2 months and oliguria for 1 day.
Reumatismo
January 2025
Rheumatology Unit, Department of Clinical Internal, Anesthesiologic and Cardiovascular Sciences, Sapienza University of Rome; Unicamillus, Saint Camillus International University of Health Sciences, Rome.
Objective: To assess the adherence to the vaccination campaign against SARS-CoV-2 in patients with immunoglobulin-G4-related disease (IgG4-RD) and to evaluate the development of local and systemic adverse events (AEs) following vaccination. Additionally, to investigate the rate and outcome of SARS-CoV-2 infection in IgG4-RD patients.
Methods: Patients with IgG4-RD in follow-up before the onset of the SARS-CoV-2 pandemic were contacted by telephone and asked to answer an ad hoc questionnaire regarding their vaccination status against SARS-CoV-2 and related AEs following vaccination.
Oxf Med Case Reports
January 2025
Department of Rheumatology, Faculty of Medicine, Damascus University, Damascus, Syria.
IgG4-related disease (IgG4-RD) is a rare but increasingly recognised condition that can involve multiple organs, including the kidneys which often presents as tubulointerstitial nephritis. Treatment with glucocorticoids is the first line of therapy, but other options may be needed in refractory cases. This case report explores a 68-year-old female, diagnosed with the patient initially responded to glucocorticoids but had a relapse, leading to progressive renal insufficiency and ultimately death.
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