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Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Pol+J+Radiol 2018 ; 83 (ä): e54-62 Nephropedia Template TP
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Imaging findings in Erdheim-Chester disease: what every radiologist needs to know #MMPMID30038679
Kumar P; Singh A; Gamanagatti S; Kumar S; Chandrashekhara SH
Pol J Radiol 2018[]; 83 (ä): e54-62 PMID30038679show ga
Erdheim-Chester disease (ECD) is a rare sporadic non-Langerhans cell histiocytic (LCH) proliferative disorder with systemic predilection. It usually affects adults in the 5th-7th decades of life and has non-specific clinical manifestations. Its suspicion is often heralded by the presence of characteristic radiological findings and subsequently confirmed by demonstration of CD68-positive xanthogranulomatous infiltrates on histopathology. Despite being a non-malignant entity, it might be fatal due to organ dysfunction. Imaging plays a key role in the diagnosis, management, and follow-up. Imaging findings are essential to establish the diagnosis, assess actual disease burden, and explore the aetiopathogenesis and therapeutic options to halt disease progression and associated morbidity.