Worldwide Prevalence of Diabetes Insipidus
The worldwide prevalence of Diabetes Insipidus (DI) is approximately 1 in 25,000 population, which translates to about 0.004% of the global population. 1
Epidemiological Data
The prevalence of DI is estimated at 1 per 25,000 population worldwide, making it a rare endocrine disorder. 1
This prevalence figure encompasses both central DI (arginine vasopressin deficiency) and nephrogenic DI (arginine vasopressin resistance), though the exact breakdown between these subtypes in population studies is not well-established. 2
The majority of DI cases (approximately 99%) are acquired rather than inherited, with familial/inherited central DI accounting for only about 1% of cases. 1
Important Clinical Context
Central DI is the most common form of DI, resulting from deficiency of arginine vasopressin (AVP) from the pituitary, and typically develops when more than 80% of AVP-secreting neurons are damaged. 1
Nephrogenic DI is less common than central DI and develops secondary to AVP resistance in the kidneys, with more than 280 disease-causing mutations identified affecting the AVP2 protein, V2 receptor, or aquaporin 2 (AQP2). 1
The exact incidence and prevalence of congenital NDI specifically are unknown, but this form is considered particularly rare within the already rare DI population. 3
Diagnostic Challenges Affecting Prevalence Estimates
Many practitioners are unfamiliar with DI diagnosis due to its rarity, which can lead to delayed or missed diagnoses and potentially underestimated prevalence figures. 3
The condition requires specialized testing (water deprivation test or copeptin measurement) to distinguish it from primary polydipsia, which may contribute to underdiagnosis in some populations. 4, 2
DI cannot be cured and requires lifelong management, making accurate prevalence tracking important for healthcare resource allocation. 4