Imaging findings in Erdheim-Chester disease: what every radiologist needs to know
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Publication date: 2018-02-04
Pol J Radiol, 2018; 83: 54-62
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.
Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D et al: Erdheim- Chester disease. Clinical and radiologic characteristics of 59 cases. Medicine 1996; 75: 157-169.
Mazor RD, Manevich-Mazor M, Shoenfeld Y. Erdheim-Chester disease: A comprehensive review of the literature. Orphanet J Rare Dis 2013; 8: 137.
Lodhi U, Sarmast U, Khan S et al. Multisystem radiologic manifestations of Erdheim-Chester disease. Case Rep Radiol 2016; 2016: 2670495.
Dion E, Graef C, Miquel A et al. Bone involvement in Erdheim-Chester disease: Imaging findings including periostitis and partial epiphyseal involvement. Radiology 2006; 238: 632-639.
Antunes C, Graça B, Donato P. Thoracic, abdominal and musculoskeletal involvement in Erdheim-Chester disease: CT, MR and PET imaging findings. Insights Imaging 2014; 5: 473-482.
Munoz J, Janku F, Cohen PR et al. Erdheim-Chester disease: Characteristics and management. Mayo Clin Proc 2014; 89: 985-996.
Kim MS, Kim CH, Choi SJ et al. Erdheim-chester disease. Ann Dermatol 2010; 22: 439-443.
Drier A, Haroche J, Savatovsky J et al. Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR imaging findings. Radiology 2010; 255: 586-594.
Sedrak P, Ketonen L, Hou P et al. Erdheim-Chester disease of the central nervous system: New manifestations of a rare disease. Am J Neuroradiol 2011; 32: 2126-2131.
De Abreu MR, Chung CB, Biswal S et al. Erdheim-Chester disease: MR imaging, anatomic, and histopathologic correlation of orbital involvement. Am J Neuroradiol 2004; 25: 627-630.
Alper MG, Zimmerman LE, Piana FG. Orbital manifestation of Erdheim-Chester disease. Trans Am Ophthahalmol Soc 1983; 81: 64-85.
Rush WL, Andriko JA, Galateau-Salle F et al. Pulmonary pathology of Erdheim-Chester disease. Mod Pathol 2000; 13: 747-754.
Pan A, Doyle T, Schlup M et al. Unusual manifestation of Erdheim- Chester disease. BMC Gastroenterol 2011; 11: 77.
Wittenberg KH, Swensen SJ, Myers JL. Pulmonary involvement with Erdheim-Chester disease: Radiographic and CT findings. Am J Roentgenol 2000; 174: 1327-1331.
Dion E, Graef C, Haroche J et al. Imaging of thoracoabdominal involvement in Erdheim-Chester disease. Am J Roentgenol 2004; 183: 1253-1260.
Haroche J, Cluzel P, Toledano D et al. Images in cardiovascular medicine. Cardiac involvement in Erdheim-Chester disease: Magnetic resonance and computed tomographic scan imaging in a monocentric series of 37 patients. Circulation 2009; 119: e597-598.
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