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Chronic Periaortitis in Marfan Syndrome: A Case Report.
Int J Rheum Dis
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Department of Rheumatology and Immunology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
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Intern Emerg Med
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Rheumatology Division, University Hospital Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander, Spain.
Aortitis and periaortitis refer to the inflammation of the aortic wall and the surrounding tissues. Both conditions are associated with various diseases and express nonspecific manifestations. Early diagnosis and treatment are crucial to improve the prognosis of the disease.
View Article and Find Full Text PDFUpdate on IgG4-related periaortitis/retroperitoneal fibrosis and periarteritis -recent clinical, diagnostic and therapeutic advances.
Semin Arthritis Rheum
February 2025
Department of Hematology and Immunology, Kanazawa Medical University, Kahoku-gun, Japan. Electronic address:
Background: IgG4-related disease (IgG4-RD) is a systemic, chronic immune-mediated inflammatory disorder that, similar to sarcoidosis, can affect various organs and tissues. IgG4-related periaortitis (PAo)/retroperitoneal fibrosis (RPF) is among the five major manifestations of IgG4-RD. Despite introduction of the ACR and EULAR classification criteria for IgG4-RD in 2019, diagnosing IgG4-related PAo/RPF and periarteritis (PA) remains challenging because obtaining biopsies from these lesions is difficult.
View Article and Find Full Text PDFMultimodality imaging review of retroperitoneal fibrosis.
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March 2025
Department of Radiology and Biomedical Imaging, Yale School of Medicine, New Haven, USA.
Retroperitoneal fibrosis (RPF) is a rare fibroinflammatory disease with idiopathic and secondary causes. Idiopathic disease is more common and is believed to be immune mediated; associations with autoimmune diseases and/or inflammatory disorders such as IgG4 related disease are often present. Common complications include hydronephrosis and venous stenosis and/or thrombosis.
View Article and Find Full Text PDFOrmond's Disease - 26 Years of Experience at One Centre.
Prague Med Rep
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Department of Clinical and Experimental Pathology, IKEM, Prague, Czech Republic.
Ormond's disease is a systemic autoimmune disease with serious complications. We present our retrospective analysis of 83 patients diagnosed with and treated for idiopathic retroperitoneal fibrosis (Ormond's disease) in our department from 1997 to 2023. In this retrospective study, we analysed the diagnostic approaches, the clinical history and surgical and immunosuppressive therapies, and their subsequent effects on our patients.
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