Idiopathic retroperitoneal fibrosis: Clinicopathologic features and differential diagnosis

被引:142
作者
Corradi, D.
Maestri, R.
Palmisano, A.
Bosio, S.
Greco, P.
Manenti, L.
Ferretti, S.
Cobelli, R.
Moroni, G.
Tos, A. P. Dei
Buzio, C.
Vaglio, A.
机构
[1] Univ Parma, Dept Pathol & Lab Med, Pathol Sect, I-43100 Parma, Italy
[2] Univ Parma, Dept Clin Med Nephrol & Hlth Sci, I-43100 Parma, Italy
[3] Hosp Desenzano Garda, Div Nephrol & Hemodialysis, Brescia, Italy
[4] Maggiore Hosp, Div Urol, Parma, Italy
[5] Univ Parma, Dept Radiol, Parma, Italy
[6] Div Nephrol, Policlin Hosp, Milan, Italy
[7] Reg Hosp, Dept Pathol, Treviso, Italy
关键词
acute renal failure; vasculitis; fibrosis; histopathology; inflammation; obstructive uropathy;
D O I
10.1038/sj.ki.5002427
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:742 / 753
页数:12
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