2Retroperitoneal fibrosis
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.
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