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Clinical Images  |   May 2017
Retroperitoneal Fibrosis
Author Notes
  • 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: jconard@lakelandhealth.org
     
Article Information
Endocrinology / Gastroenterology / Imaging / Hypertension/Kidney Disease / Urological Disorders / Diabetes / Clinical Images
Clinical Images   |   May 2017
Retroperitoneal Fibrosis
The Journal of the American Osteopathic Association, May 2017, Vol. 117, 340. doi:10.7556/jaoa.2017.067
The Journal of the American Osteopathic Association, May 2017, Vol. 117, 340. doi:10.7556/jaoa.2017.067
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  
References
Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet. 2006;367(9506):241-251. [CrossRef] [PubMed]
Vaglio A, Palmisano A, Alberici F, 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)60934-3 [CrossRef] [PubMed]
Kermani TA, Crowson CS, Achenbach SJ, Luthra HS. Idiopathic retroperitoneal fibrosis: a retrospective review of clinical presentation, treatment, and outcomes. Mayo Clin Proc. 2011;86(4):297-303. doi: 10.4065/mcp.2010.0663 [CrossRef] [PubMed]