Endocrine Manifestations in a Monocentric Cohort of 64 Patients With Erdheim-Chester Disease

Context: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, characterized by infiltration of foamy histiocytes in multiple organs. Endocrine involvement has mostly been described in case reports. Objective: We performed systematic endocrine evaluation in a large cohort of patients with ECD. Design: This was a single-center observational study conducted between October 2007 and May 2013. Setting: The evaluation was conducted in Pitie-Salpetriere Hospital (Paris, France), a tertiary care hospital. Patients: Sixty-four consecutive patients with ECD (sex ratio, 3.6; mean age, 57.6 years [range, 20–80 years]). Thirty-six patients had follow-up assessments. Interventions: There were no interventions. Main Outcome Measures: Clinical, biological, and morphological evaluations of pituitary, gonadal, adrenal, and thyroid functions, as well as metabolic evaluation, were performed. Results: Diabetes insipidus was found in 33.3% of patients, frequently as the first manifestation of ECD. Anterio...