We searched PubMed without any date limits and EMBASE between 1980 and 2005, mainly using the search terms “retroperitoneal fibrosis” and “periaortitis”; we largely selected articles published in English during the past 10 years without excluding older papers that we considered to be highly relevant to the topics discussed in this Seminar. We also included some review papers and a book chapter, providing insightful overviews on retroperitoneal fibrosis and related diseases.
SeminarRetroperitoneal fibrosis
Section snippets
Epidemiology and diagnosis
The epidemiological characteristics of retroperitoneal fibrosis are not well established, and only data on the idiopathic form are available: a report of a study done in Finland13 noted that idiopathic retroperitoneal fibrosis has an incidence of 0·1 per 100 000 person-years and a prevalence of 1·38 per 100 000 inhabitants in the study area. No clear ethnic predisposition has emerged.
Men are affected twice to three times as often as women; the mean age at presentation is 50–60 years, but
Idiopathic retroperitoneal fibrosis
The pathogenesis of idiopathic retroperitoneal fibrosis is unclear. The leading theory was proposed by Mitchinson and Parums,8, 9, 10 who first defined chronic periaortitis as a range of diseases—including idiopathic retroperitoneal fibrosis—characterised by advanced aortic atherosclerosis, medial thinning, and pronounced adventitial and periaortic inflammation and fibrosis. These investigators suggested that chronic periaortitis could be a consequence of a local inflammatory reaction to
Pathology
The typical macroscopic appearance of idiopathic retroperitoneal fibrosis is that of a white, hard retroperitoneal plaque of varying thickness, which surrounds the abdominal aorta, the iliac vessels, and, in most instances, the inferior vena cava and the ureters. The plaque usually develops between the origin of the renal arteries and the pelvic brim, although presacral extension is not uncommon; only in rare cases does it extend anteriorly to the mesenteric root or posteriorly to the spinal
Clinical manifestations
The clinical manifestations of idiopathic and secondary retroperitoneal fibrosis often overlap, thus they are not useful in the differential diagnosis between the two disease groups. The clinical signs and symptoms that herald the onset of retroperitoneal fibrosis are non-specific; however, patients usually report two types of manifestations: localised (likely due to the presence of the retroperitoneal mass and its mechanic or compressive effects) and systemic (possible expression of the
Laboratory findings
The results of routine laboratory tests are consistent with inflammatory disease: concentrations of acute-phase reactants, such as ESR and CRP, are high in 80–100% of patients.11, 56, 58 These laboratory tests are often used to monitor the clinical course of the disease,56 though they do not always reliably mirror disease activity.59 Azotemia usually depends on the extent of ureteral obstruction. Mild-to-moderate anaemia is usually noted, to which both chronic inflammation and—when
Imaging
Imaging studies are essential in the diagnosis and management of retroperitoneal fibrosis, and can sometimes help to differentiate between idiopathic and secondary disease.
Sonography should be done as a first-line study, especially when an azotemic patient is being assessed; on ultrasound, idiopathic retroperitoneal fibrosis appears as a hypoechoic or isoechoic mass, which can involve the ureters and, thus, cause unilateral or bilateral hydronephrosis.
Intravenous urography usually reveals the
Associated autoimmune diseases
Patients with idiopathic retroperitoneal fibrosis often have mild manifestations of an autoimmune disease—eg, presence of autoantibodies and raised concentrations of acute-phase reactants. In others, their disease actually develops in the setting of well defined systemic autoimmune disorders—eg, systemic lupus erythematosus, vasculitic syndromes60, 65—or associates with the so-called organ-specific autoimmune diseases—eg, Hashimoto's thyroiditis, sclerosing cholangitis.11 The most important
Differential diagnoses
In addition to the above discussed idiopathic and secondary forms of retroperitoneal fibrosis, the retroperitoneum can also be affected by another group of fibrosing disorders, which, unlike retroperitoneal fibrosis, are primarily characterised by a prominent fibroblast proliferation that may or may not be associated with an inflammatory component.80
Retroperitoneal fibromatosis is histologically characterised by a uniform proliferation of fibroblasts, arranged in interlacing bundles. It
Treatment and course
The aims of treatment of idiopathic retroperitoneal fibrosis are multiple: to stop the progression of the fibro-inflammatory reaction, to inhibit or relieve the obstruction of the ureters or other retroperitoneal structures, to switch off the acute-phase reaction and its systemic manifestations, and to prevent disease recurrence or relapse.
Corticosteroids are the most used drugs. They suppress the synthesis of most of the cytokines involved in the acute-phase reaction, reduce the inflammatory
Future perspectives
Retroperitoneal fibrosis is a largely obscure and multifaceted disease. Diagnostic criteria are needed, as are new methods for the differential diagnosis between the idiopathic and secondary forms of the disease. Studies on the complex pathogenesis of the idiopathic disease could elucidate the role of immune-mediated mechanisms and provide new clues for treatment. Finally, the optimum dose and duration of steroid therapy and the role of other potentially useful agents, such as
Search strategy and selection criteria
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