Arginine Vasopressin Disorder (Diabetes Insipidus)

Excerpt

Arginine vasopressin disorder is a clinical syndrome characterized by the passage of abnormally large volumes of urine (diabetes) that is dilute (hypotonic) and devoid of dissolved solutes (ie, insipid). They belong to a group of inherited or acquired disorders of polyuria and polydipsia. This is associated with insufficient arginine vasopressin (AVP), antidiuretic hormone (ADH) secretion, or renal response to AVP, resulting in hypotonic polyuria and compensatory/underlying polydipsia. The hallmarks of diabetes insipidus (DI) include polyuria (>50 mL/kg), dilute urine (osmolality <300 mOsm/L), and increased thirst with the intake of up to 20 L/day fluid intake. Untreated DI can cause hypovolemia, dehydration, and electrolyte imbalances.

Terminology

In 2022, the Endocrine Society, European Society of Endocrinology, Pituitary Society, Society for Endocrinology, European Society for Paediatric Endocrinology, Endocrine Society of Australia, Brazilian Endocrine Society, and Japanese Endocrine Society all proposed to change the name of this disorder from central DI to arginine vasopressin deficiency (AVP-D), and nephrogenic DI to arginine vasopressin resistance (AVP-R).

There are multiple reasons to consider changing the name of diabetes insipidus currently. First and foremost, the usage of the term "diabetes" in both diabetes mellitus and diabetes insipidus has confused patients and their caregivers. Although the terms "mellitus" and "insipidus" differentiate between the clinical characteristics of these two distinct causes of polyuria, the common term "diabetes" has led to misunderstandings, mainly when non-endocrine specialists are treating patients with DI. Some healthcare professionals fail to recognize the difference between these two disorders, resulting in severe consequences such as withholding desmopressin treatment in central DI cases, leading to adverse outcomes and even deaths.

In response to these unfortunate and preventable occurrences, national safety alerts have been issued, surveys among endocrinologists have been conducted, and a global task force comprising experienced clinicians involved in the care of DI patients has emerged. These collective efforts have created a strong motivation to change the condition's name. Second, a recent survey published in The Lancet Diabetes & Endocrinology, which included over 1000 patients with central diabetes insipidus, revealed that 85% preferred a name change. The primary reason behind this preference was their encounters with healthcare professionals who exhibited an insufficient understanding of the disease, often confusing it with diabetes mellitus. Notably, 87% of the surveyed patients believed that this lack of knowledge and resulting clinical confusion negatively affected the management of their condition, leading to unnecessary blood sugar measurements and the prescription of medications intended for diabetes mellitus during hospitalization.

Lastly, we believe medical disorder names should reflect the underlying pathophysiology. In the case of diabetes insipidus, the deficiency in the secretion and end-organ effects of the hormone arginine vasopressin (AVP) is now well-established. Therefore, for the reasons above, the working group proposes changing the name of DI to arginine vasopressin deficiency (AVP-D) for central causes and arginine vasopressin resistance (AVP-R) for nephrogenic reasons.