Abstract
Idiopathic retroperitoneal fibrosis (IRPF) is a rare condition of unknown aetiology characterized by chronic non-specific inflammation of the retroperitoneum. The aim of this study is to describe, for the first time, the features of Spanish patients with IRPF. In this retrospective study, a clinical description was performed to examine the histopathological features, radiologic findings, laboratory data and treatment of a cohort of 24 IRPF Spanish patients who were admitted to Vall d’Hebron Hospital in Barcelona (Spain) between 1982 and 2009. Patients with secondary retroperitoneal fibrosis were excluded. Nineteen patients (79.1 %) were male, whereas 5 were female. The mean age at diagnosis was 51.8 ± 16.4 years. Pain (79.1 %) and hydronephrosis (70.8 %) were the most common clinical manifestations. Abdominal computed tomography and ultrasonography were performed on all cases. Acute-phase reactants were elevated in most patients. Thirteen surgical biopsies were performed, and all were consistent with retroperitoneal fibrosis. Steroid therapy was given in 19 cases, and eight patients (42.1 %) required urethral catheterisation. Chronic renal failure (CRF) rate after 2 years of follow-up was 42.8 %, more frequently in patients with higher serum creatinine at diagnosis. IRPF in Spain is a rare condition affecting mainly middle-aged males, with pain and hydronephrosis as the most frequent manifestations. Steroids are the mainstay treatment, and CRF is the main sequel. An earlier diagnosis and uniform treatment protocols could prompt lower CRF rates.
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Vaglio A, Salvarani C, Buzio C (2006) Retroperitoneal fibrosis. Lancet 367:241–251
Koep L, Zuidema GD (1977) The clinical significance of retroperitoneal fibrosis. Surgery 81:250–257
Wu J, Catalano E, Coppola D (2002) Retroperitoneal fibrosis (Ormond's disease): clinical pathologic study of eight cases. Cancer Control 9:432–437
Vaglio A, Corradi D, Manenti L et al (2003) Evidence of autoimmunity in chronic periaortitis: a prospective study. Am J Med 114:454–462
Okada H, Takahira S, Sugahara S et al (1999) Retroperitoneal fibrosis and systemic lupus erythematosus. Nephrol Dial Transplant 14:1300–1302
Ohara H, Nakazawa T, Ando T, Joh T (2007) Systemic extrapancreatic lesions associated with autoimmune pancreatitis. J Gastroenterol 42(Suppl 18):15–21
Kamisawa T, Funata N, Hayashi Y et al (2003) Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut 52:683–687
Hamano H, Kawa S, Ochi Y et al (2002) Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet 359:1403–1404
Baker LR, Mallinson WJ, Gregory MC et al (1987) Idiopathic retroperitoneal fibrosis. A retrospective analysis of 60 cases. Br J Urol 60:497–503
van Bommel EF, van Spengler J, van der Hoven B, Kramer P (1991) Retroperitoneal fibrosis: report of 12 cases and a review of the literature. Neth J Med 39:338–345
Kermani TA, Crowson CS, Achenbach SJ et al (2011) Idiopathic retroperitoneal fibrosis: a retrospective review of clinical presentation, treatment, and outcomes. Mayo Clin Proc 86(4):297–303
Marcolongo R, Tavolini IM, Laveder F et al (2004) Immunosuppressive therapy for idiopathic retroperitoneal fibrosis: a retrospective analysis of 26 cases. Am J Med 116:194–197
Gómez García I, Sánchez Castaño A, Romero Molina M et al (2012) Retroperitoneal fibrosis: single-centre experience from 1992 to 2010, current status of knowledge and review of the international literature. Scand J Urol. doi:10.3109/00365599.2012.747564
Uibu T, Oksa P, Auvinen A et al (2004) Asbestos exposure as a risk factor for retroperitoneal fibrosis. Lancet 363:1422–1426
Parums DV, Brown DL, Mitchinson MJ (2004) Serum antibodies to oxidized low-density lipoprotein and ceroid in chronic periaortitis. Arch Pathol Lab Med 114:383–387
Vaglio A, Palmisano A, Corradi D, Salvarani C, Buzio C (2007) Retroperitoneal fibrosis: evolving concepts. Rheum Dis Clin North Am 33:803–817, vi-vii
Martinez-Valle F, Solans-Laque R, Bosch-Gil J, Vilardell-Tarres M (2010) Aortic involvement in giant cell arteritis. Autoimmun Rev 9:521–524
Vaglio A, Pipitone N, Salvarani C (2011) Chronic periaortitis: a large-vessel vasculitis? Curr Opin Rheumatol 23:1–6
van Bommel EF, Siemes C, Hak LE, van der Veer SJ, Hendriksz TR (2007) Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone. Am J Kidney Dis 49:615–625
Kardar AH, Kattan S, Lindstedt E, Hanash K (2002) Steroid therapy for idiopathic retroperitoneal fibrosis: dose and duration. J Urol 168:550–555
Vaglio A, Palmisano A, Alberici F et al (2011) Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial. Lancet 378:338–346
Acknowledgments
The authors gratefully acknowledge Dr. Eva Balada (Research Unit of Systemic Autoimmune Diseases, Vall d’Hebron University Hospital Research Institute in Barcelona, Spain) and Dr. Yoh Zen (Institute of Liver Studies, King's College Hospital in London, UK) for making valuable contributions to this manuscript.
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Fernández-Codina, A., Martínez-Valle, F., Castro-Marrero, J. et al. Idiopathic retroperitoneal fibrosis: a clinicopathological study in 24 Spanish cases. Clin Rheumatol 32, 889–893 (2013). https://doi.org/10.1007/s10067-013-2225-3
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DOI: https://doi.org/10.1007/s10067-013-2225-3