TY - JOUR
T1 - Retroperitoneal Fibrosis
T2 - Evolving Concepts
AU - Vaglio, Augusto
AU - Palmisano, Alessandra
AU - Corradi, Domenico
AU - Salvarani, Carlo
AU - Buzio, Carlo
PY - 2007/11
Y1 - 2007/11
N2 - Retroperitoneal fibrosis (RPF) is a rare fibro-inflammatory condition that is idiopathic in most cases, but may be secondary to various causes. Although the cause and pathogenesis of the idiopathic form are unknown, immunogenetic factors and immunopathologic/autoimmune mechanisms are probably involved. Idiopathic RPF usually develops around the abdominal aorta and iliac arteries but in some cases may also involve the thoracic aorta and the origin of its major branches, with a pattern similar to that of other forms of large-vessel vasculitis. In addition, the disease is frequently associated with autoimmune conditions affecting other organs. Glucocorticoids alone or in combination with immunosuppressive agents are usually effective treatment options, but the disease frequently has a chronic relapsing course.
AB - Retroperitoneal fibrosis (RPF) is a rare fibro-inflammatory condition that is idiopathic in most cases, but may be secondary to various causes. Although the cause and pathogenesis of the idiopathic form are unknown, immunogenetic factors and immunopathologic/autoimmune mechanisms are probably involved. Idiopathic RPF usually develops around the abdominal aorta and iliac arteries but in some cases may also involve the thoracic aorta and the origin of its major branches, with a pattern similar to that of other forms of large-vessel vasculitis. In addition, the disease is frequently associated with autoimmune conditions affecting other organs. Glucocorticoids alone or in combination with immunosuppressive agents are usually effective treatment options, but the disease frequently has a chronic relapsing course.
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U2 - 10.1016/j.rdc.2007.07.013
DO - 10.1016/j.rdc.2007.07.013
M3 - Article
C2 - 18037118
AN - SCOPUS:36248993397
VL - 33
SP - 803
EP - 817
JO - Rheumatic Disease Clinics of North America
JF - Rheumatic Disease Clinics of North America
SN - 0889-857X
IS - 4
ER -