title: [Renal involvement in Erdheim-Chester disease].
abstract: 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.
authors.forename: Gioacchino
authors.initials: G
authors.lastname: Li Cavoli
authors.forename: Luisa
authors.initials: L
authors.lastname: Bono
authors.forename: Calogera
authors.initials: C
authors.lastname: Tortorici
authors.forename: Angelo
authors.initials: A
authors.lastname: Ferrantelli
authors.forename: Carmela
authors.initials: C
authors.lastname: Zagarrigo
authors.forename: Franca
authors.initials: F
authors.lastname: Servillo
authors.forename: Carlo
authors.initials: C
authors.lastname: Giammarresi
authors.forename: Angelo
authors.initials: A
authors.lastname: Tralongo
authors.forename: Onofrio
authors.initials: O
authors.lastname: Schillaci
authors.forename: Mario
authors.initials: M
authors.lastname: Coglitore
authors.forename: Ugo
authors.initials: U
authors.lastname: Rotolo
mesh_terms: D031249:Erdheim-Chester Disease / Q000150:complications*; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology*