Retroperitoneal Fibrosis and Arterial Lesions

  • Kenji Notohara


The condition of retroperitoneal fibrosis (RPF) has long been known, but since its inclusion in the spectrum of IgG4-RD, the disease concept requires reconsideration. Many cases of RPF correspond to periaortitis showing lesions around the abdominal aorta. Furthermore, similar lesions also occur in the thoracic aorta and aortic branches, and such lesions are now regarded as manifestations of periarteritis or frank arteritis. Unilateral RPF limited to one periureteral or perirenal pelvic area has been also sporadically reported. Marked lymphoplasmacytic infiltration and fibrosis are the characteristic pathological findings, with storiform fibrosis, obliterative phlebitis, and abundant IgG4-positive plasmacytic infiltration present. However, at this time it seems clear that IgG4-RD does not account for all cases of RPF. Some cases of RPF appear to have no association with IgG4-RD despite clinical and radiologic findings that are very similar to those of patients with IgG4-related RPF.


Thoracic Aorta Giant Cell Arteritis Takayasu Arteritis Ergot Alkaloid Retroperitoneal Fibrosis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer Japan 2014

Authors and Affiliations

  1. 1.Department of Anatomic PathologyKurashiki Central HospitalKurashikiJapan

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