Erdheim-Chester Disease with Right Atrial Tumor and “Temporal Arteritis”

DOI: 10.4236/ojra.2012.23008   PDF   HTML   XML   4,450 Downloads   7,435 Views  


Erdheim-Chester disease is an unusual syndrome characterized by non-Langerhans cell histiocytosis which can mimic rheumatologic diseases. We report a case of Erdheim-Chester masquerading as giant cell arteritis, which was success-fully managed with anakinra as anti IL-1 therapy.

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J. Skalski, W. Edwards and E. Matteson, "Erdheim-Chester Disease with Right Atrial Tumor and “Temporal Arteritis”," Open Journal of Rheumatology and Autoimmune Diseases, Vol. 2 No. 3, 2012, pp. 37-38. doi: 10.4236/ojra.2012.23008.

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The authors declare no conflicts of interest.


[1] J. Haroche, L. Arnaud and Z. Amoura, “Erdheim-Chester Disease,” Current Opinion in Rheumatology, Vol. 14, No. 1, 2012, pp. 53-59. doi:10.1097/BOR.0b013e32834d861d
[2] A. Aouba, S. Georgin-Lavialle, C. Pagnoux, N. M. Silva, A. Renand, et al., “Rationale and Efficacy of Interleukin-1 Targeting in Erdheim-Chester Disease,” Blood, Vol. 116, No. 20, 2012, pp. 4070-4076. doi:10.1182/blood-2010-04-279240
[3] W. Chester, “über Lipoidgranulomatose,” Virchows Archive, Vol. 279, No. 2, 1930, pp. 561-602. doi:10.1007/BF01942684
[4] J. Haroche, P. Cluzel, D. Toledano, G. Montalescot, D. Touitou, et al., “Cardiac Involvement in Erdheim-Chester Disease: Magnetic Resonance and Computed Tomographic Scan Imaging in a Monocentric Series of 37 Patients,” Circulation, Vol. 119, No. 25, 2009, pp. e597-e598. doi:10.1161/CIRCULATIONAHA.108.825075
[5] E. C. Derrett-Smith and B. Dasgupta, “Erdheim-Chester Disease: Yet another Mimic of Polymyalgia Rheumatica and Giant Cell Arteritis?” Rheumatology, Vol. 44, No. 3, 2005, pp. iii14-iii15.

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