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- 저자 : ( Alfonso Lope (검색결과 1 건)
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( Marta Perez De Lis Novo ),( Roberto Perez Alvarez ),( Soledad Retamozo ),( Pilar Brito Zeron ),( Xavier Bosch ),( Antoni Siso Almirall ),( Jaume Villalta ),( Ricardo Alvarez Vijande ),( Alfonso Lope 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1
Background: To analyse the possible existence of features associated with IgG4-related disease (IgG4-RD) in patients diagnosed with retroperitoneal fi brosis or mesenteritis. Methods: Study cohort including patients diagnosed with retroperitoneal fi brosis and/ or mesenteritis by imaging studies (CT) in a university hospital during the last 20 years. Results: A total of 22 patients diagnosed with retroperitoneal fi brosis (15 patients) or mesenteritis (7 patients) are described (6 women and 16 men, with a mean age at diagnosis of 64.1 years, range: 46-82. The main clinical manifestations present at diagnosis were abdominal/back pain (n=14), general malaise/fever (n=7) and the presence of edema/thrombosis (n = 6); in one patient, diagnosis was made incidentally by imaging techniques. CT showed infl ammatory masses affecting retroperitoneal (n=17), mesenteric (n=7) and vascular structures, including iliac arteries (n=3), aorta (n=2), renal arteries (n=2) and cava (n=2). In 7 (32%) patients, involvement of other organs suggestive of IgG4-RD (liver/bile tract in 2 cases, periaortitis in 2 cases, pleura, lymph nodes and skin in 1 each, respectively) was found. Diagnosis was confi rmed by biopsy in 8 patients (36%); in any patient, studies to rule out IgG4-RD were performed. The main treatments included corticosteroids (n=17), immunosuppressive agents (azathioprine in 5, methotrexate in 1 and mycophenolate in 1), surgery (n = 5) and the placement of urinary catheters (n = 4). After a mean follow-up of 73 months, 7 patients died (32%) patients (4 of them due to septic shock). Conclusions: Ormond`s disease is a rare clinical entity but associated with a signifi cant morbidity and mortality. His recent inclusion in the clinical spectrum of IgG4-RD diffi cult their retrospective diagnosis, although this study and others recently published suggest that about half of the cases could correspond to IgG4-RD.
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