Retroperitoneale Fibrose, Morbus Ormond, chronische Periaortitis, ... [Retroperitoneal fibrosis, M. Ormond, periaoartitis, ...?]

Details

Serval ID
serval:BIB_2B05004790CB
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Title
Retroperitoneale Fibrose, Morbus Ormond, chronische Periaortitis, ... [Retroperitoneal fibrosis, M. Ormond, periaoartitis, ...?]
Journal
Therapeutische Umschau
Author(s)
Vogt B., Meier P., Burnier M.
ISSN
0040-5930
Publication state
Published
Issued date
2008
Volume
65
Number
5
Pages
265-268
Language
german
Notes
Publication types: English Abstract ; Journal Article ; Review
Abstract
Retroperitoneal fibrosis or Morbus Ormond is a rare disease characterized by inflammatory fibrosis of the retroperitoneal space and the abdominal aorta often including the common iliac arteries. The abdominal aorta can be enlarged leading to a classification of retroperitoneal fibrosis with or without aneurysm of the abdominal aorta. The classic form of retroperitoneal fibrosis usually presents without aneurysm of the abdominal aorta. Clinical presentation is not specific and includes abdominal, flank or back pain. Mechanisms, initiating factors and pathophysiology of the disease have remained unclear to date, however there is evidence that autoimmune mechanisms are involved. The most relevant complication of retroperitoneal fibrosis is the involvement of the ureter leading to obstructive nephropathy and requiring relief from obstruction. Because of the low frequency of the disease associated with a great variety of presentation no randomized controlled trials are available. Drug therapy is limited to steroids alone or in combination with immunosuppressive drugs such as azathioprine or even cyclophosphamide. The response to medical therapy is variable from patient to patient. Clinical endpoints are general symptoms, sedimentation rate, and imaging studies with abdomen CT or MRI.
Keywords
Humans, Immunosuppressive Agents/therapeutic use, Retroperitoneal Fibrosis/classification, Retroperitoneal Fibrosis/diagnosis, Terminology as Topic
Pubmed
Create date
17/07/2008 10:52
Last modification date
03/03/2018 15:24
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