Arginine Vasopressin Disorders (Diabetes Insipidus)

Arginine vasopressin disorder, formerly known as diabetes insipidus, represents two conditions that happen when your body lets go of too much urine (pee). It can’t keep (retain) water properly. These conditions are rare but treatable.

An issue with arginine vasopressin (AVP) — a hormone — mainly causes these conditions:

• AVP deficiency (AVP-D) happens when your body doesn’t make enough AVP.
• AVP resistance (AVP-R) happens because your kidneys don’t use AVP properly.

Healthcare providers may also call AVP antidiuretic hormone (ADH). AVP plays a large role in helping your kidneys balance the amount of fluid in your body.

Symptoms of AVP deficiency and AVP resistance (diabetes insipidus)

The main symptoms of arginine vasopressin deficiency and resistance include:

• Needing to pee often, including through the night (nocturia)
• Passing large amounts of light-colored or clear pee
• Extreme thirst (polydipsia)

Most people make 1 to 3 quarts of pee a day. People with AVP-D or AVP-R can make up to 20 quarts of pee a day.

If you stop drinking fluids or don’t get treatment, you can quickly develop dehydration.

These symptoms are a significant sign that something in your body isn’t working as it should. It’s essential to get medical help as soon as possible.

Symptoms in children

Symptoms of arginine vasopressin deficiency or arginine vasopressin resistance in children may include:

• Severe dehydration
• Constipation
• Vomiting
• Fevers
• Irritability
• Growth faltering (failure to thrive)

Causes of arginine vasopressin disorders (diabetes insipidus)

The causes vary based on the type:

• Arginine vasopressin deficiency (AVP-D). Formerly known as central diabetes insipidus, AVP-D happens when your body doesn’t have enough arginine vasopressin (a deficiency). It’s the most common type.
• Arginine vasopressin resistance (AVP-R). This type was formerly known as nephrogenic diabetes insipidus. AVP-R happens when your pituitary gland releases enough AVP, but your kidneys don’t respond to it properly (resistance).

One other type is a rare pregnancy-related form known as gestational arginine vasopressin disorder (gAVP-d). During pregnancy, the placenta may make too much of an enzyme that breaks down AVP. You’re more likely to get gAVP-D if you:

• Are pregnant with multiples
• Have a condition that affects your liver function, like preeclampsia or HELLP syndrome

gAVP-d usually goes away two to three weeks after the pregnancy is over.

Causes of arginine vasopressin deficiency (AVP-D)

AVP-D happens when there’s an issue with your hypothalamus or pituitary gland. Specific causes include:

• An inherited gene variation
• Damage from surgery or a head injury
• Inflammation (granulomas) from conditions like sarcoidosis or tuberculosis
• Tumors

Up to half of the cases of AVP-D have an unknown cause (idiopathic).

Causes of arginine vasopressin resistance (AVP-R)

Specific causes of AVP-R include:

• A blocked urinary tract
• An inherited gene variation
• Certain medications
• Hypercalcemia
• Hypokalemia
• Lithium toxicity

Complications of AVP-D and AVP-R

The main complication of arginine vasopressin disorders is dehydration. This happens when your body loses too much fluid and electrolytes to function as it’s meant to.

You can usually make up for all the fluids you pass in your pee by drinking more liquids. But if you don’t, you could quickly become dehydrated.

Dehydration can be life-threatening. If you have symptoms of dehydration, like dizziness, nausea and fatigue, get to the nearest hospital as soon as possible.

How doctors diagnose AVP-D or AVP-R

Other, more common conditions cause frequent thirst and peeing. So, part of the process of diagnosing AVP-D or AVP-R involves ruling out other conditions. Your healthcare provider may order several tests if you have these symptoms.

A diagnosis of AVP-D or AVP-R involves finding the type and cause.

Tests that diagnose AVP-D or AVP-R

A water deprivation test is the most reliable way to diagnose either AVP-D or AVP-R. If you get this test, you’ll be under constant supervision during the process. This is because it can cause dehydration.

A water deprivation test involves not drinking any liquid for several hours to see how your body responds. If you have AVP-D or AVP-R, you’ll continue to pee large amounts of watery, light-colored urine. Without the condition, you’ll only pee a small amount of concentrated, dark yellow urine.

Your provider may also recommend the following tests:

• Copeptin blood test (copeptin is a byproduct of AVP)
• Blood glucose test to rule out diabetes mellitus
• Urinalysis to check osmolality (the concentration of your urine) and/or to check for ketones
• Imaging tests, like an MRI, to check for damage to your pituitary gland or hypothalamus

How are AVP-D and AVP-R (diabetes insipidus) treated?

Treatment depends on whether you have AVP-D or AVP-R. Even with medical treatment, it’s important to drink water regularly to make sure you don’t get dehydrated.

AVP-D treatment

Desmopressin is the first-line treatment for arginine vasopressin deficiency (AVP-D). It’s a medication that works like AVP. It comes in different forms.

Healthcare providers may also use desmopressin to treat the rare gestational form known as gAVP-d. Drinking more water alone may be enough for the gestational form.

AVP-R treatment

Treatment for arginine vasopressin resistance (AVP-R) is more complicated. It sometimes involves a combination of approaches, like taking the following medications:

• Thiazide diuretics
• Nonsteroidal anti-inflammatory drugs (NSAIDs)

If a medication you take causes AVP-R, your provider can sometimes treat AVP-R by switching the medication to a different one.

When should I see my healthcare provider?

If you have AVP-D or AVP-R, it’s important to see your healthcare provider regularly. They’ll want to run tests to monitor the condition and make sure your treatment is working.

It may be helpful to ask your provider the following questions:

• Do I have AVP-D or AVP-R?
• What caused it?
• Is it chronic or temporary?
• What are my treatment options?
• How much water should I be drinking in a day?
• Is there anything else I can do to manage this condition?
• Are my family members at risk for developing arginine vasopressin disorder?

What can I expect if I have AVP-D or AVP-R?

The outlook (prognosis) for AVP-D and AVP-R is generally good. It usually doesn’t cause serious problems as long as you get treatment and drink enough water.

The risk of complications is higher for:

• Infants
• People over 65
• People with certain mental health conditions or developmental disabilities

These groups of people may have difficulty recognizing their thirst or they may not be able to act on it.

Why is it no longer called diabetes insipidus?

In 2022, several medical organizations decided to change the name of diabetes insipidus to arginine vasopressin deficiency and arginine vasopressin resistance. The main reason for this change is that the shared term of “diabetes” was causing confusion and leading to harmful health effects.

What most people just call “diabetes” is diabetes mellitus. It happens when you have high blood sugar, which causes frequent thirst and urination. It’s much more common than AVP-D and AVP-R (diabetes insipidus). The treatment for diabetes mellitus is very different from the treatment for AVP-D and AVP-R.

The word “diabetes” comes from the Greek word “diabainein,” which means “go through.” This is much like the liquids that quickly go through your body in diabetes mellitus and the arginine vasopressin disorders