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Everolimus in Erdheim–Chester disease

Abstract

A 43-year-old man presented with bony pains, repeated pathological fractures with overlying skin ulcerations, and forearm and chest wall swellings. Investigations led to the diagnosis of Erdheim–Chester disease. Treatment with low-dose prednisolone, oral everolimus, and zoledronic acid was started, with a marked improvement in his condition.

References

  1. Dagna L, Girlanda S, Langheim S, Rizzo N, Bozzolo EP, Sabbadini MG, Ferrarini M. Erdheim–Chester disease: report on a case and new insights on its immunopathogenesis. Rheumatology (Oxford) 2010; 49: 1203–1206.

    Article  Google Scholar 

  2. Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, Wechsler J, Brun B, Remy M, et al. Erdheim–Chester disease: clinical and radiologic characteristics of 59 cases. Medicine (Baltimore) 1996; 75: 157–169.

    Article  CAS  Google Scholar 

  3. Serratrice J, Granel B, De Roux C, Pellissier JF, Swiader L, Bartoli JM, et al. ‘Coated aorta’: a new sign of Erdheim–Chester disease. J Rheumatol 2000; 27: 1550–1553.

    CAS  PubMed  Google Scholar 

  4. Pertuiset E, Laredo JD, Lioté F, Wassef M, Jagueux M, Kuntz D, et al. Erdheim–Chester disease: report of a case, review of the literature and discussion of the relation to Langerhans-cell histiocytosis. Rev Rhum Ed Fr 1993; 60: 601–609.

    CAS  PubMed  Google Scholar 

  5. Cline MJ. Histiocytes and histiocytoses. Blood 1994; 84: 2840–2853.

    Article  CAS  Google Scholar 

  6. Gaspar N, Boudou P, Haroche J, Wechsler B, Van Den Neste E, Hoang-Xuan K, et al. High-dose chemotherapy followed by autologous hematopoietic stem cell transplantation for adult histiocytic disorders with central nervous system involvement. Haematologica 2006; 91: 1121–1125.

    CAS  PubMed  Google Scholar 

  7. Bisceglia M, Cammisa M, Suster S, Colby TV. Erdheim–Chester disease: clinical and pathologic spectrum of four cases from the Arkadi M. Rywlin slide seminars. Adv Anat Pathol 2003; 10: 160–171.

    Article  Google Scholar 

  8. Jendro MC, Zeidler H, Rosenthal H, Haller H, Schwarz A. Improvement of Erdheim–Chester disease in two patients by sequential treatment with vinblastine and mycophenolate mofetil. Clin Rheumatol 2004; 23: 52–56.

    Article  Google Scholar 

  9. Egan AJ, Boardman LA, Tazelaar HD, Swensen SJ, Jett JR, Yousem SA, Myers JL. ECD: clinical, radiological and histopathologic findings in 5 patients with ILD. Am J Surg Pathol 1999; 23: 17–26.

    Article  CAS  Google Scholar 

  10. Mossetti G, Rendina D, Numis FG, Somma P, Postiglione L, Nunziata V. Biochemical markers of bone turnover, serum levels of IL6/IL6 soluble receptor and bisphosphonate treatment in ECD. Clin Exp Rheumatol 2003; 21: 232–236.

    CAS  PubMed  Google Scholar 

  11. Myra C, Sloper L, Tighe PJ, McIntosh RS, Stevens SE, Gregson RHS, et al. Treatment of Erdheim–Chester disease with cladribine: a rational approach. Br J Ophthalmol 2004; 88: 844–847.

    Article  CAS  Google Scholar 

  12. Braiteh F, Boxrud C, Esmaeli B, Kurzrock R. Successful treatment of Erdheim–Chester disease, a non-Langerhans-cell histiocytosis, with interferon. Blood 2005; 106: 2992–2994.

    Article  CAS  Google Scholar 

  13. Matsui K, Nagata Y, Hiraoka M. Radiotherapy for Erdheim–Chester disease. Int J Clin Oncol 2007; 12: 238–241.

    Article  Google Scholar 

  14. Aouba A, Georgin-Lavialle S, Pagnoux C, Silva NM, Renand A, Galateau-Salle F, et al. Rationale and efficacy of interleukin-1 targeting in Erdheim–Chester disease. Blood 2010; 116: 4070–4076.

    Article  CAS  Google Scholar 

  15. Vaglio A. A pilot study in the treatment of ECD. ERA-EDTA 2004;75–76.

  16. Goudar R, Shi Q, Hjelmeland M, Keir ST, McLendon RE, Wikstrand CJ, et al. Combination therapy of inhibitors of epidermal growth factor receptor/vascular endothelial growth factor receptor 2 (AEE788) and the mammalian target of rapamycin (RAD001) offers improved glioblastoma tumor growth inhibition. Mol Cancer Ther 2005; 4: 101–112.

    CAS  PubMed  Google Scholar 

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Hussein, M.A., El-Hindawi, A. & Ragab, G. Everolimus in Erdheim–Chester disease. Egypt J Intern Med 25, 159–163 (2013). https://doi.org/10.7123/01.EJIM.0000433902.50829.68

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  • DOI: https://doi.org/10.7123/01.EJIM.0000433902.50829.68

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