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Idiopathic central diabetes insipidus in a large cohort of patients: the hypopituitarism ENEA rare observational (HEROS) study

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Abstract

Central Diabetes Insipidus (CDI) is mainly associated with structural pathologies of the hypothalamic-pituitary area. Etiologies underlying CDI are identified in most patients, however idiopathic CDI is reported in 13–17% of cases after excluding other etiologies. The Hypopituitarism ENEA Rare Observational Study (HEROS study) retrospectively collected data of patients with idiopathic CDI from 14 pituitary centers in 9 countries. The cohort included 92 patients (59 females 64%), mean age at diagnosis was 35.4 ± 20.7 years, and a mean follow up of 19.1 ± 13.5 years following CDI diagnosis. In 6 women, diagnosis was related to pregnancy. Of 83 patients with available data on pituitary imaging, 40(48%) had normal sellar imaging, and 43(52%) had pathology of the posterior pituitary or the stalk, including loss of the bright spot, posterior pituitary atrophy or stalk enlargement. Anterior pituitary hormone deficiencies at presentation included hypogonadism in 6 (6.5%) patients (5 females), and hypocortisolism in one; during follow-up new anterior pituitary deficiencies developed in 6 patients. Replacement treatment with desmopressin was given to all patients except one, usually with an oral preparation. During follow up, no underlying disease causing CDI was identified in any patient. Patients with idiopathic CDI following investigation at baseline are stable with no specific etiology depicted during long-term follow-up.

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Funding

ENEA supported the production of the on-line platform for reporting the patients` data by the participating investigators.

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Authors and Affiliations

  1. Endocrine Institute, Rabin Medical Center – Beilinson Hospital, 39 Jabotinsky St, Petach Tikva, Israel

    H. Masri Iraqi, A. Akirov & I. Shimon

  2. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

    H. Masri Iraqi, A. Akirov & I. Shimon

  3. Endocrinology Research Centre, Moscow, Russia

    E. Pigarova

  4. Medical University Sofia, Sofia, Bulgaria

    S. Zacharieva

  5. Federico II University of Naples, Naples, Italy

    A. Colao

  6. Soroka University Medical Center, Beer Sheva, Israel

    L. Baraf

  7. Laiko University Hospital, Athens, Greece

    M. Tsoli

  8. Neuroendocrine Department, Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, Belgrade, Serbia

    M. Doknic

  9. University of Genova, Genoa, Italy

    S. Ricci Bitti

  10. University of Turin, Turin, Italy

    R. Giordano

  11. University Hospital of Padova, Padua, Italy

    M. Barbot

  12. Medical University of Warsaw, Warsaw, Poland

    P. Witek

  13. Tel Aviv-Sourasky Medical Center and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

    M. Serebro

  14. Medizinische Klinik and Poliklinik IV, Klinikum Der Universität München, LMU München, Munich, Germany

    M. K. Auer

  15. Semmelweis University, Budapest, Hungary

    M. Tóth

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  1. H. Masri Iraqi
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  2. E. Pigarova
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  3. S. Zacharieva
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  4. A. Colao
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  8. S. Ricci Bitti
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  16. I. Shimon
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Contributions

All authors contributed patients’ data and reviewed the manuscript. HMI and IS wrote the manuscript.

Corresponding author

Correspondence to H. Masri Iraqi.

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The authors have no competing interests to declare.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Iraqi, H.M., Pigarova, E., Zacharieva, S. et al. Idiopathic central diabetes insipidus in a large cohort of patients: the hypopituitarism ENEA rare observational (HEROS) study. Pituitary 25, 982–987 (2022). https://doi.org/10.1007/s11102-022-01277-1

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  • DOI: https://doi.org/10.1007/s11102-022-01277-1

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