Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) involves multiple organs, including the lungs and central nervous system. Lung lesions are frequently reported as mass lesions or non-specific interstitial pneumonia, whereas organising pneumonia (OP) due to IgG4-RD is rare. Furthermore, limited information is currently available on hypertrophic pachymeningitis (HP). We herein report a case of IgG4-RD complicated with OP and HP. The diagnosis was confirmed based on the serum concentration of IgG4 and the results of salivary gland and transbronchial lung biopsies. HP did not respond to steroid monotherapy and was also resistant to rituximab and intravenous cyclophosphamide; however, the combination therapy of methotrexate and dexamethasone was effective.
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http://dx.doi.org/10.1093/mrcr/rxac025 | 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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