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Retroperitoneal fibrosis

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Retroperitoneal fibrosis is a rare syndrome hallmarked by a fibrosclerotic tissue in the retroperitoneum, often leading to encasement of the ureters. About two-thirds of cases of retroperitoneal fibrosis are idiopathic, while the remaining cases are secondary to a variety of different causes, including drugs, tumors and infections. Idiopathic retroperitoneal fibrosis may be associated with abdominal aorta aneurysms, with vasculitis of the thoracic aorta and of epi-aortic vessels, or both. Most patients present with abdominal and/or low back pain. Serum markers of inflammation are usually, but not invariably, elevated. The diagnosis is secured by computed tomography or magnetic resonance imaging, which typically show an enhancing retroperitoneal mass medially dislodging the ureters. Positron emission tomography can be useful to document the extent and metabolic activity of the inflammatory process. Treatment rests on glucocorticoids with a tapering scheme variably combined with immunosuppressive agents. In cases of ureter obstruction, relief of obstruction by stenting or ureterolysis is required.

Introduction

Retroperitoneal fibrosis is a rare syndrome characterised by the development of fibrosclerotic tissue in the retroperitoneum, which often leads to encasement of the ureters and, less frequently, of blood and lymphatic vessels [1]. About two-thirds of cases of retroperitoneal fibrosis are idiopathic, while the remaining cases are secondary to intake of a number of drugs, infections, malignancies, surgery or exposure to radiation [1], [2]. In this review, we will focus on the idiopathic form of retroperitoneal fibrosis, and discuss the latest developments in the understanding of its pathogenesis, imaging and treatment.

Section snippets

Classification of retroperitoneal fibrosis

There are no formal criteria to classify idiopathic retroperitoneal fibrosis (IRF). Currently, IRF is considered part of the spectrum of chronic periaortitis (CP), a large-vessel vasculitis [3]. Specifically, IRF is characterised by a retroperitonal fibro-inflammatory tissue in the absence of a dilated aorta, while in inflammatory abdominal aorta aneurysms (IAAAs) the fibro-inflammatory tissue develops around a dilated aorta, and in perianeurysmal retroperitoneal fibrosis the fibro-inflammatory

Epidemiology

CP is a rare disease with prevalence of 1.4/100 000 and a yearly incidence of 0.1–1.3/100,000 [9], *[10]. Mean age at onset is approximately 55–60 years *[11], *[12], [13]. Men are affected two to three times more often than females *[11], *[12], [13]. An even stronger male predominance has been observed in patients with IgG4-related CP, including those with aneurysmal forms of CP [7].

Aetiology and pathogenesis

CP is a fibro-inflammatory disorder [14]. Although its pathogenesis is still obscure, its pathological appearance shows the co-existence of fibrous and inflammatory components [15]. Earlier studies proposed that advanced aortic atherosclerosis be regarded as a sine qua non for the development of CP, and that CP arise as an exaggerated localised inflammatory reaction to atherosclerotic plaque components [16]. Findings supporting such a view included circulating antibodies directed against

Clinical manifestations

The most common presenting symptoms of CP are abdominal, lower back or flank pain *[11], *[12], [33]. The pain is usually dull in nature, although colicky pain may occur in the presence of ureter encasement [33]. Renal failure due to obstruction of the ureters resulting in hydronephrosis develops in 42–95% patients, with higher rates being reported from renal compared with rheumatology centres *[11], [13]. Involvement of retroperitoneal blood and lymphatic vessels is less common, occurring in

Laboratory tests

Inflammatory markers, such as the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), are elevated in approximately one-half to two-thirds of patients with CP *[11], *[12], [34]. However, both ESR and CRP lack sensitivity and specificity for the diagnosis of retroperitoneal fibrosis [35]. It is also controversial to what extent ESR and CRP correlate with disease activity [36], [37]. In a longitudinal study, in untreated patients ESR and CRP correlated with pain intensity, while

Treatment

Treatment of retroperitoneal fibrosis aims at relieving symptoms, decreasing the size of the retroperitoneal mass, reversing ureteral obstruction and preventing relapses once remission has been achieved. Patients with ureter obstruction should be promptly treated with percutaneous nephrostomy tubes or double-J ureter stents at cystoscopy [1], [49]. In the presence of severe obstruction, ureterolysis followed by omental wrapping of the ureters may be required [1]. Surgery is also mandated in

Conclusions

Many of the pathogenic mechanisms operating in CP still need to be better understood. However, despite our incomplete knowledge about the pathogenesis of CP, we currently have valuable diagnostic tools at hand to arrive at an early diagnosis. Treatment with glucocorticoids, variably associated with immunosuppressive agents and relief of ureter obstruction whenever needed, results in a good prognosis in the majority of patients.

Conflict of interest

The authors have no conflict of interest to declare.

References (61)

  • I. Jansen et al.

    (18)F-fluorodeoxyglucose position emission tomography (FDG-PET) for monitoring disease activity and treatment response in idiopathic retroperitoneal fibrosis

    European Journal of Internal Medicine

    (2010)
  • A.H. Kardar et al.

    Steroid therapy for idiopathic retroperitoneal fibrosis: dose and duration

    Journal of Urology

    (2002)
  • E.F. van Bommel et al.

    Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone

    American Journal of Kidney Diseases

    (2007)
  • R. Marcolongo et al.

    Immunosuppressive therapy for idiopathic retroperitoneal fibrosis: a retrospective analysis of 26 cases

    American Journal of Medicine

    (2004)
  • A. Vaglio et al.

    Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial

    Lancet

    (2011)
  • P.M. Young et al.

    Hypermetabolic activity in patients with active retroperitoneal fibrosis on F-18 FDG PET: report of three cases

    Annals of Nuclear Medicine

    (2008)
  • C. Salvarani et al.

    Positron emission tomography (PET): evaluation of chronic periaortitis

    Arthritis & Rheumatism

    (2005)
  • C. Salvarani et al.

    Vasculitis of the gastrointestinal tract in chronic periaortitis

    Medicine (Baltimore)

    (2011)
  • A. Ghinoi et al.

    Primary Sjogren’s syndrome associated with chronic periaortitis

    Rheumatology (Oxford)

    (2007)
  • H. Umehara et al.

    A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details

    Modern Rheumatology

    (2012)
  • Y. Zen et al.

    Retroperitoneal fibrosis: a clinicopathologic study with respect to immunoglobulin G4

    The American Journal of Surgical Pathology

    (2009)
  • S. Kasashima et al.

    IgG4-related inflammatory abdominal aortic aneurysm

    Current Opinion in Rheumatology

    (2011)
  • E.F. van Bommel et al.

    Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation

    Medicine (Baltimore)

    (2009)
  • K.P. Li et al.

    Idiopathic retroperitoneal fibrosis (RPF): clinical features of 61 cases and literature review

    Clinical Rheumatology

    (2011)
  • D.V. Parums

    The spectrum of chronic periaortitis

    Histopathology

    (1990)
  • D.V. Parums et al.

    Serum antibodies to oxidized low-density lipoprotein and ceroid in chronic periaortitis

    Archives of Pathology & Laboratory Medicine

    (1990)
  • A. Vaglio et al.

    ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis?

    Journal of Internal Medicine

    (2002)
  • A. Vaglio et al.

    Peripheral inflammatory arthritis in patients with chronic periaortitis: report of five cases and review of the literature

    Rheumatology (Oxford)

    (2008)
  • G. Famularo et al.

    Retroperitoneal fibrosis associated with psoriasis: a case series

    Scandinavian Journal of Rheumatology

    (2009)
  • A. Vaglio et al.

    Chronic periaortitis: a large-vessel vasculitis?

    Current Opinion in Rheumatology

    (2011)
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