Guideline sources The following summarized guidelines for the evaluation and management of central diabetes insipidus are prepared by our editorial team based on guidelines from the Histiocyte Society (HS 2022), the American College of Radiology (ACR 2019), the Society for Endocrinology (SE 2018), the European Thyroid Association (ETA 2018), and the Endocrine Society (ES 2016). 1 2 3 4 5 Guidelines 1. Diagnostic investigations Initial evaluation: obtain an urgent clinical assessment of volume and hydration status, and measurement of serum sodium, potassium and renal function. E Show 2 more Diagnostic imaging: obtain MRI of the sella with or without IV contrast as initial imaging of adult patients with diabetes insipidus. B Evaluation for hypopituitarism (central adrenal insufficiency): obtain testing for adrenal insufficiency in patients with improved diabetes insipidus without an adrenal insufficiency https://web.pathway.md/diseases/recIxgSZZHDyxSW30 1/4 6/29/23, 3:46 AM Central diabetes insipidus Pathway diagnosis. B Evaluation for hypopituitarism (central hypothyroidism): obtain screening for central hypothyroidism in patients with diabetes insipidus and hypothyroid manifestations. A 2. Medical management Setting of care: Discuss cases of hospitalized patients with central diabetes insipidus with the endocrine team as soon as possible to ensure optimal inpatient care and specialist follow-up thereafter. E Manage patients with hypernatremia as a medical emergency with a level 2-3 care or equivalent high dependency setting. E Treatment goals: Correct serum sodium at a rate of 5 mmol/L in the first hour (or until symptoms improve) and limited to 10 mmol/L per 24 hours in symptomatic patients with hypernatremia developed within 48 hours (acute hypernatremia). E Correct hypernatremia at a rate not exceeding 0.5 mmol/L per hour and limited to 10 mmol/L per 24 hours in patients with no or mild symptoms. E Fluid therapy: provide easy access to water by the bedside and administer desmopressin as prescribed and agreed by the clinical team in orientated patients with normal Glasgow Coma Scale, intact thirst mechanism, and ability to drink. E Show 6 more Desmopressin: As per SE 2018 guidelines, consider offering self-administration of desmopressin in patients able to do so, in line with the recommended prescribed dose agreed with the clinical team. E Show 2 more As per ES 2016 guidelines, consider individualizing therapeutic schedules of desmopressin in patients with diabetes insipidus Offer treatment in all patients with diabetes insipidus, although recognizing that some patients with partial diabetes insipidus may not be bothered by polyuria and may prefer no treatment. Show 3 more Vasopressin: consider administering short-acting SC aqueous ADH as initial therapy for perioperative diabetes insipidus, allowing for safer use in the vast majority of cases where diabetes insipidus resolves spontaneously. C 3. Inpatient care Serial clinical and laboratory assessment: As per SE 2018 guidelines, obtain serum sodium monitoring in able patients at least every 24 hours while on IV fluids to avoid the occult development of hyponatremia in patients requiring maintenance IV fluid therapy and taking regular desmopressin. E https://web.pathway.md/diseases/recIxgSZZHDyxSW30 2/4 6/29/23, 3:46 AM Central diabetes insipidus Pathway Show 3 more As per ES 2016 guidelines, consider undertaking careful titration of desmopressin and fluid intake with frequent weighing and serum sodium level monitoring in patients with adipsic diabetes insipidus. 4. Specific circumstances Pregnant patients: consider continuing desmopressin during pregnancy and adjusting doses if required in pregnant patients with preexisting diabetes insipidus. C Patients with Langerhans cell histiocytosis: obtain laboratory evaluation for diabetes insipidus and anterior pituitary function in patients with Langerhans cell histiocytosis presenting with polyuria/polydipsia or involvement of the pituitary/hypothalamus axis on cranial imaging. A Patients with adrenal insufficiency: consider monitoring for the development of diabetes insipidus after initiating glucocorticoid replacement in patients with adrenal insufficiency as the latter may mask the presence of partial diabetes insipidus. C 5. Quality improvement Medical alert bracelet: consider ensuring that all patients with diabetes insipidus wear an emergency bracelet or necklace to inform clinicians of their condition if incapacitated. E Hospital requirements: alert the endocrinology or alternative appropriate clinical team about all patients in a hospital or care facility with a diagnosis of central diabetes insipidus. E Show 3 more Clinical findings Symptoms Past medical history Adipsia Brain metastasis Headache CNS lymphoma Nocturia Craniopharyngioma Polydipsia Encephalitis Polyuria Granulomatosis with polyangiitis Seizure Hypopituitarism Visual disturbances Internal carotid artery aneurysm Langerhans cell histiocytosis Past surgical history Lymphocytic hypophysitis Meningitis Recent brain surgery Optic glioma Vascular exam Pinealoma Pituitary apoplexy https://web.pathway.md/diseases/recIxgSZZHDyxSW30 3/4 6/29/23, 3:46 AM Central diabetes insipidus Pathway Dry mucous membranes Pituitary tumor Sarcoidosis Lab findings Septo-optic dysplasia Sheehan's syndrome serum osmolality TBI serum sodium Tuberculosis urine aquaporin 2 plasma arginine vasopressin Vital signs plasma copeptin urine osmolality Hypotension Diagnostic procedures Integument exam vasopressin challenge test Decreased skin turgor Dehydration Serological findings Serum anti-arginine vasopressin-secreting cells antibodies References 1. Maria Fleseriu, Ibrahim A Hashim, Niki Karavitaki et al. Hormonal Replacement in Hypopituitarism in Adults: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2016 Nov;101 11 3888 3921. Open 2. S E Baldeweg, S Ball, A Brooke et al. SOCIETY FOR ENDOCRINOLOGY CLINICAL GUIDANCE Inpatient management of cranial diabetes insipidus. Endocr Connect. 2018 Jul;7 7 G8 G11. Open 3. Expert Panel on Neurologic Imaging:, Judah Burns, Bruno Policeni et al. ACR Appropriateness Criteria Neuroendocrine Imaging. J Am Coll Radiol. 2019 May;16 5S S161 S173. Open 4. Persani L, Brabant G, Dattani M et al. 2018 European Thyroid Association ETA Guidelines on the Diagnosis and Management of Central Hypothyroidism. Eur Thyroid J. 2018 Oct;7 5 225 237. Open 5. Gaurav Goyal, Abdellatif Tazi, Ronald S Go et al. Expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults. Blood. 2022 Mar 10;blood.2021014343. 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