Startseite Retroperitoneal Fibrosis
Artikel Open Access

Retroperitoneal Fibrosis

  • Jennifer Conard und Michelino Mancini
Veröffentlicht/Copyright: 1. Mai 2017

A 50-year-old man with type 2 diabetes mellitus, nonalcoholic fatty liver disease, and sarcoidosis presented to the emergency department with lower abdominal pain for the past 3 months. Physical examination revealed suprapubic discomfort, back pain, stress-induced incontinence, abdominal distention, and scrotal swelling. A computed tomographic angiogram of the abdomen and pelvis showed progressive, circumferential, periaortic internal iliac soft tissue swelling (image A, arrow). Laboratory test results confirmed elevated levels of C-reactive protein and serum complement C3 and C4. A venacavogram showed high-grade infrarenal inferior vena cava stenosis (image B, arrow). The patient was admitted to the hospital and given 1 mg of intravenous methylprednisolone for 3 days. A stent was placed, and the patient was discharged 6 days later. Oral prednisone was prescribed, beginning at a dose of 60 mg and decreasing by 10 mg every 3 weeks. One month after discharge methotrexate was prescribed (5 mg every 12 hours, 1 time weekly) and the patient's symptoms improved.

Retroperitoneal fibrosis is rare, and more than two-thirds of cases are idiopathic.1 Computed tomographic and magnetic resonance images can reveal soft-tissue swelling surrounding the abdominal aorta and iliac arteries, with possible encasement of neighboring structures.2 Management consists of immunosuppressants aimed at inactivation of acute-phase reactants and relapse prevention.2,3 Relapses and progression to end-stage renal disease can be minimized by close follow-up and patient adherence to medication.3


From the Departments of Internal Medicine (Dr Conard) and Medical Education (Dr Mancini) at the Lakeland Health Center in St. Joseph, Michigan. Dr Conard is a third-year resident.
Financial Disclosures: None reported.
Support: None reported.

*Address correspondence to Jennifer Conard, MBA, DO, 1234 Napier Ave, St. Joseph, MI 49085-2112. E-mail:


References

1. Vaglio A , SalvaraniC, BuzioC. Retroperitoneal fibrosis. Lancet. 2006;367(9506):241-251.10.1016/S0140-6736(06)68035-5Suche in Google Scholar

2. Vaglio A , PalmisanoA, AlbericiF, et al. Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomized controlled trial. Lancet. 2011;378(9788):338-346. doi:10.1016/S0140-6736(11)60934-3Suche in Google Scholar

3. Kermani TA , CrowsonCS, AchenbachSJ, LuthraHS. Idiopathic retroperitoneal fibrosis: a retrospective review of clinical presentation, treatment, and outcomes. Mayo Clin Proc. 2011;86(4):297-303. doi:10.4065/mcp.2010.0663Suche in Google Scholar PubMed PubMed Central

Received: 2016-04-18
Accepted: 2016-04-26
Published Online: 2017-05-01
Published in Print: 2017-05-01

© 2017 American Osteopathic Association

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Heruntergeladen am 26.9.2025 von https://www.degruyterbrill.com/document/doi/10.7556/jaoa.2017.067/html
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