Endocrine and metabolic assessment in adults with Langerhans cell histiocytosis

L. Montefusco, S. Harari, D. Elia, A. Rossi, C. Specchia, O. Torre, G. Adda, M. Arosio

Research output: Contribution to journalArticlepeer-review


Context: Diabetes insipidus (DI) is one of most common complications of Langerhans cell histiocytosis (LCH) but prevalence of anterior pituitary deficiencies and metabolic alterations have not been clearly defined yet. Objectives: Evaluate prevalence of endocrine and metabolic manifestations in a cohort of patients affected by Pulmonary LCH. Methods: Observational cross-sectional study on 18 adults (7 M/11 F, 42. ±. 12. years) studied for complete basal and dynamic endocrine lab tests and glucose metabolism. Results: Hypothalamic-pituitary endocrine alterations were found in 9 patients: 9 had DI, 5 Growth Hormone Deficiency (GHD), 5 central hypogonadism, 3 central hypothyroidism and 1 central hypoadrenalism. Hyperprolactinemia and hypothalamic syndrome were found in 2 patients each. All these central endocrine alterations were always associated to DI. Five of the 10 MRI performed showed abnormalities. Prevalence of obesity and glucose alterations (either DM or IFG/IGT) were respectively 39% and 33%, higher than expected basing on epidemiological data on general Italian population. Multi-system-LCH without risk-organ involvement (LCH MS-RO-) seems to have slightly higher prevalence of insulin resistance, glucose alterations and metabolic syndrome than LCH with isolated lung involvement (LCH SS lung+). A papillary BRAFV600E positive thyroid carcinoma was diagnosed in one patient. Conclusions: The presence of anterior pituitary deficiencies should be systematically sought in all LCH patients with DI both at diagnosis and during the follow-up by basal and dynamic hormonal assessment. Patients with pulmonary LCH, particularly those with MS disease, have a worse metabolic profile than general population. Occurrence of papillary thyroid carcinoma has been reported.

Original languageEnglish
Pages (from-to)61-67
JournalEuropean Journal of Internal Medicine
Publication statusPublished - 2018


  • Anterior pituitary
  • Diabetes insipidus
  • Glucose metabolism
  • Langerhans cell histiocytosis

ASJC Scopus subject areas

  • Internal Medicine


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