[Cardiovascular involvement in Erdheim-Chester syndrome: clinical and therapeutic implications].

[erdheim-chester disease]

Erdheim-Chester disease (ECD ) is a rare form of non-Langerhans ' cell histiocytosis of unknown etiology and its incidence is constantly increasing . ECD is characterized by a xantomatous or xanthogranulomatous infiltration of various tissues by foamy histiocytes surrounded by fibrosis . ECD is characterized by multi-organ involvement and is generally associated with a poor prognosis with a median survival of 32 months after diagnosis . Cardiovascular involvement concerns mainly the thoraco-abdominal aorta and pericardium . Less frequently , infiltration affects the myocardial tissue , especially the right atrium , and the valvular endocardium . Recently , the involvement of the vena cava has also been described . The diagnosis of ECD is made by the identification of foamy histiocytes CD 68 positive and CD 1 a /S 100 negative embedded in a polymorphic inflammatory tissue on biopsy . Despite the adoption of several therapeutic strategies until now prognosis has remained poor . Interferon-Î± can be considered the first line therapy , but its effects on central nervous system and cardiovascular localization have been shown to be often poor . In this context a combined treatment with the anti-TNFÎ± monoclonal antibody infliximab and methotrexate seems to be effective and well tolerated .