Retroperitoneal fibrosis – a report of five cases

Retroperitoneal fibrosis (RPF) is a rare disease, characterized by inflammation and deposition of fibrotic tissue in the vicinity of the abdominal aorta and iliac arteries. We present a report of five patients admitted to our department between January 2014 and February 2017, diagnosed with RPF. Abdominal pain was the most common presenting symptom; however, in one patient, RPF was identified accidentally in routinely performed ultrasonography. In 4 cases, corticosteroids (CS) in combination with azathioprine were applied as first-line therapy, whereas one patient was treated with intravenous methylprednisolone pulses followed by oral CS. In this paper, clinical features as well as laboratory and radiographic findings together with management and treatment outcomes in patients with RPF are discussed. Given the rarity of the condition, it seems important to report every single case of RPF to help establish its management algorithm.

[1]  Priyanka Minocha,et al.  Presentation of idiopathic retroperitoneal fibrosis at a young age: A rare case report. , 2016, Intractable & rare diseases research.

[2]  M. Prucha,et al.  Combination of Steroids and Azathioprine in the Treatment of Ormond's Disease--A Single Centre Retrospective Analysis. , 2016, Prague medical report.

[3]  N. Eliakim‐Raz,et al.  Retroperitoneal fibrosis: case series of five patients and review of the literature. , 2016, Revista brasileira de reumatologia.

[4]  R. Sehgal,et al.  Idiopathic retroperitoneal fibrosis: clinicopathologic features and outcome analysis , 2016, Clinical Rheumatology.

[5]  A. Seremak-Mrozikiewicz,et al.  [Retroperitoneal fibrosis--case report and literature review]. , 2015, Ginekologia polska.

[6]  Chang-Keun Lee,et al.  Clinicopathologic characteristics of IgG4-related retroperitoneal fibrosis among patients initially diagnosed as having idiopathic retroperitoneal fibrosis , 2015, Modern rheumatology.

[7]  A. Vaglio,et al.  Idiopathic and secondary forms of retroperitoneal fibrosis: a diagnostic approach. , 2015, La Revue de medecine interne.

[8]  S. Ferretti,et al.  Methotrexate plus prednisone in patients with relapsing idiopathic retroperitoneal fibrosis , 2013, Annals of the rheumatic diseases.

[9]  N. Sainani,et al.  Rethinking Ormond’s Disease , 2013, Medicine.

[10]  C. Alberti Revisiting the retroperitoneal fibroses: are there any significant news about their aetiopathogenesis and diagnostic approaches? , 2012, European review for medical and pharmacological sciences.

[11]  J. Sass,et al.  Cyclophosphamide is a highly effective and safe induction therapy in chronic periaortitis: a long-term follow-up of 35 patients with chronic periaortitis , 2011, Annals of the rheumatic diseases.

[12]  S. Achenbach,et al.  Idiopathic retroperitoneal fibrosis: a retrospective review of clinical presentation, treatment, and outcomes. , 2011, Mayo Clinic proceedings.

[13]  O. Matsui,et al.  Retroperitoneal Fibrosis: A Clinicopathologic Study With Respect to Immunoglobulin G4 , 2009, The American journal of surgical pathology.

[14]  K. Farrington,et al.  Successful Use of Steroids and Ureteric Stents in 24 Patients with Idiopathic Retroperitoneal Fibrosis: A Retrospective Study , 2008, Nephron Clinical Practice.

[15]  S. Ferretti,et al.  Idiopathic retroperitoneal fibrosis: clinicopathologic features and differential diagnosis. , 2007, Kidney international.

[16]  T. Hendriksz,et al.  Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone. , 2007, American journal of kidney diseases : the official journal of the National Kidney Foundation.

[17]  C. Ponticelli,et al.  Long-term outcome of idiopathic retroperitoneal fibrosis treated with surgical and/or medical approaches. , 2006, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[18]  P. Bassi,et al.  Immunosuppressive therapy for idiopathic retroperitoneal fibrosis: a retrospective analysis of 26 cases. , 2004, The American journal of medicine.

[19]  K. Hanash,et al.  Steroid therapy for idiopathic retroperitoneal fibrosis: dose and duration. , 2002, The Journal of urology.

[20]  M. Piegsa,et al.  [Retroperitoneal fibrosis]. , 1993, Deutsche medizinische Wochenschrift.