Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Abstract

Retroperitoneal fibrosis (RPF) is a rare disorder consisting of idiopathic and various secondary forms and characterized by chronic inflammatory infiltrates and marked fibrosis in the retroperitoneal space. In idiopathic RPF (IRPF), 35–60% of cases have been reported to be IgG4-related RPF, the retroperitoneal lesions of IgG4-related disease (IgG4-RD). IRPF can frequently lead to renal insufficiency mediated by urinary tract obstruction and hydronephrosis irrespective of being IgG4-related or not. Clinical pictures, laboratory and imaging findings, and location of the urinary tract obstruction are generally similar in IgG4-related and non-IgG4-related IRPF although multiple organ involvement and serum IgG4 elevation may be characteristic of the IgG4-related forms. Periaortic/periarterial lesions are the most frequent cause of renal insufficiency. Although the response to glucocorticoids is generally good, relapse does occur in a considerable proportion of patients, and may require an additional immunosuppressive agent and/or urological intervention in cases with multiple relapses or refractory obstructive uropathy. In general, the prognosis of patients with IRPF is good, but careful attention needs to be paid to chronic kidney disease as a major complication and rupture of the affected aorta/artery as a life-threatening one. Further studies are necessary to better understand the pathogenesis of the disease and to establish the optimal diagnostic and therapeutic strategies for it.

A Case of Contained Rupture of the Common Iliac Artery with Idiopathic Retroperitoneal Fibrosis: Efficacy of Surgical Treatment and Immunosuppressive Therapy at 2-Year Follow-Up

“…Of the patients with RF, 10%-20% are categorized as having IgG4-related disease (IgG4-RD), which is characterized as a fibroinflammatory condition with infiltrating IgG4-positive plasma cells. 9) In the present case, laboratory data and immunohistochemical findings did not support IgG4-RD. Consistently, physical examination and an extensive CT scan did not detect any prevalent IgG4-RD lesions, either.…”

“…Hydronephrosis and/or ureter obstruction have been found in 33% to 67% of idiopathic RF patients regardless of the presence of IgG4-RD. 9) Hydronephrosis is caused by massive lesions at the renal pelvis, encasement of the ureter by a periaortic or periureteral lesion, and renal parenchymal lesions in RF. However, parenchymal lesions are rarely observed in non-IgG4-related idiopathic RF.…”

Clues to mortality trends and their related factors in IgG4-related disease: A Japanese single-centre retrospective study

“…If not treated early and appropriately, progressive brosis in the affected organs causes persistent organ dysfunction, such as chronic salivary and lacrimal gland dysfunction due to IgG4-related dacryoadenitis and sialadenitis [5] and diabetes mellitus and pancreatic exocrine insu ciency due to type 1 autoimmune pancreatitis (AIP) [6,7]. Particularly, serious complications in IgG4-RD include biliary obstruction, cirrhosis, and portal hypertension due to IgG4-related sclerosing cholangitis (IgG4-SC) [6,7], renal dysfunction due to IgG4-related kidney disease [8,9] or IgG4-related retroperitoneal brosis causing ureteral obstruction [10], and aortic aneurysm formation and/or aortic dissection due to IgG4-related periaortitis [11]. Furthermore, some recent studies have focused on the potential risk of malignancy in IgG4-RD patients which also can worsen the long-term prognosis of patients.…”

Primary retroperitoneal fibrosis presenting as a renal mass

“…[ 2 ] Since one-tenth of the patients with RPF have renal involvement, it is imperative to rule out malignancy in these cases. [ 3 ] The features differentiating RPF from malignancy have not yet been defined. [ 2 ] Wang et al .…”