[Renal involvement in Erdheim-Chester disease].

[erdheim-chester disease]

The Erdheim-Chester disease is a rare form of Langherans cells . Since 1987 it is distinguished from other istiocytosis previously identified . The diagnosis of the disease relies on defined radiological (bone imaging ) and pathological (histiocytic infiltration ) criteria . Bone disease is crucial but systemic manifestations are reported more frequently at onset . Renal involvement is always asymptomatic at onset of disease or in the follow-up . In this review we analyze the reports of the literature ; we highlight 3 pathological mechanisms of renal involvement : renal and retroperitoneal infiltration , urinary tract obstruction , renal arteries stenosis . No treatment to date has demonstrated an improvement in survival of patients with EC . Renal involvement is therefore symptomatic (ureteral stenting , percutaneous nephrostomy ) or is adopted a wait-and-see attitude .