دورية أكاديمية

Idiopathic retroperitoneal fibrosis: Clinicopathologic features and differential diagnosis.

التفاصيل البيبلوغرافية
العنوان: Idiopathic retroperitoneal fibrosis: Clinicopathologic features and differential diagnosis.
المؤلفون: Corradi, D.1 domenico.corradi@unipr.it, Maestri, R.1, Palmisano, A.2, Bosio, S.1, Greco, P.2, Manenti, L.3, Ferretti, S.4, Cobelli, R.5, Moroni, G.6, Dei Tos, A. P.7, Buzio, C.2, Vaglio, A.2
المصدر: Kidney International. Sep2007, Vol. 72 Issue 6, p742-753. 12p. 1 Black and White Photograph, 5 Diagrams, 5 Charts.
مصطلحات موضوعية: *RETROPERITONEAL fibrosis, *PATHOLOGY, *CLINICAL trials, *DIAGNOSIS, *PATIENTS, *HISTOPATHOLOGY
مستخلص: Idiopathic retroperitoneal fibrosis (IRF) is a rare disease often causing obstructive uropathy. We evaluated the clinicopathologic features of 24 patients with IRF to characterize the histopathology of the disease and to provide a framework for the differential diagnosis with other retroperitoneal fibrosing conditions. Retroperitoneal specimens were analyzed by light and electron microscopy and by immunohistochemistry. Most patients presented with abdominal/lumbar pain, constitutional symptoms, and high acute-phase reactants. Overall, 20 had ureteral involvement and 13 developed acute renal failure. The retroperitoneal tissue consisted of a fibrous component and a chronic inflammatory infiltrate with the former characterized by myofibroblasts within a type-I collagen matrix. The infiltrate displayed perivascular and diffuse patterns containing lymphocytes, macrophages, plasma cells, and eosinophils. The perivascular aggregates had a central core of CD20+ cells and a mantle of CD3+ cells in equal proportions. In the areas of diffuse infiltrate, CD3+ cells outnumbered the CD20+ cells. Most plasma cells were positive for the IgG4 isotype. Small vessel vasculitis was found in the specimens of 11 patients. Our study indicates that a sclerotic background with myofibroblasts associated with a diffuse and perivascular infiltrate mainly consisting of T and B lymphocytes may be a pathological hallmark of IRF.Kidney International (2007) 72, 742–753; doi:10.1038/sj.ki.5002427; published online 11 July 2007 [ABSTRACT FROM AUTHOR]
قاعدة البيانات: Academic Search Index
الوصف
تدمد:00852538
DOI:10.1038/sj.ki.5002427