Background: 
With only around 500 cases reported in scientific literature, Erdheim-Chester disease (ECD) is an extremely rare form of CD68 positive non-Langerhans cell histiocytosis, characterized by xanthomatous infiltration of tissues in various organs. From the anaesthetic point of view, these patients should be addressed globally, given the multisystemic involvement of their pathology.1 The reported case describes a patient diagnosed with ECD (pulmonary, endocrine and vascular disease) referred for thyroidectomy after being diagnosed with a carcinoma. To our knowledge, the only existing case report describing the anesthetic management of a patient with ECD was published in 2014.1 
Case report: The clinical case concerns a 69-year-old patient referred for a thyroidectomy after a positive biopsy for papillary thyroid carcinoma, with previous diagnosis of ECD. Imaging, analytical, and pathological examinations showed the presence of central diabetes insipidus (DI), as well as the existence of diffuse vascular thickening in several territories including cerebral, thoracic, and abdominal. The anaesthetic management began with evaluation and optimization of the patient in the pre-anaesthetic period, followed by careful monitoring during surgery and throughout the post-operative stay in intensive care. The chosen anaesthetic technique was a balanced general anaesthesia. The multidisciplinary involvement and diligent supervision resulted in a perioperative free of complications. 
Discussion: Given the pulmonary findings, endocrine and cerebrovascular disease and cardiovascular alterations, pre-anaesthetic evaluation and optimization was essential. The main goal of the anaesthetic management was to achieve the best hemodynamic stability possible and to prevent any critical events.

References: 
1.	Hanriharan U. et al. Erdheim – chester disease: clinical pearls for the anesthesiologist. J Anaesthesiol Clin Pharmacol. 2014 Apr-Jun, 30 (2): 297-298
2.	Diamond E. et al. Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. Blood, 24 Jul 2014;vol 124, n4

Learning points:  ECD is a rare multisystemic disorder with an anaesthetic approach scarcely described in medical literature; these patients present a challenge to the anesthetist and should be addressed globally.

Multisystemic Erdheim-Chester disease: case report outlining anaesthetic considerations and management