Primary ovarian insufficiency occurs when the ovaries stop functioning as they should before age 40. When this happens, your ovaries don't produce typical amounts of the hormone estrogen or release eggs regularly. This condition is also called premature ovarian failure and often leads to infertility.

Primary ovarian insufficiency is sometimes confused with premature menopause, but these conditions aren't the same. Women with primary ovarian insufficiency can have irregular or occasional periods for years and might even get pregnant. But women with premature menopause stop having periods and can't become pregnant.

Restoring estrogen levels in women with primary ovarian insufficiency helps prevent some complications that occur as a result of low estrogen, such as osteoporosis.

Signs and symptoms of primary ovarian insufficiency are similar to those of menopause or estrogen deficiency. They include:

• Irregular or skipped periods, which might be present for years or develop after a pregnancy or after stopping birth control pills
• Difficulty getting pregnant
• Hot flashes
• Night sweats
• Vaginal dryness
• Dry eyes
• Irritability or difficulty concentrating
• Decreased sexual desire

When to see a health care provider

If you've missed your period for three months or more, see your health care provider to determine the cause. You can miss your period for a number of reasons — including pregnancy, stress, or a change in diet or exercise habits — but it's best to get evaluated whenever your menstrual cycle changes.

Even if you don't mind not having periods, it's advisable to see your provider to find out what's causing the change. Low estrogen levels can lead to bone loss and an increased risk of heart disease.

Primary ovarian insufficiency may be caused by:

• Chromosome changes. Some genetic disorders are associated with primary ovarian insufficiency. These include conditions in which you have one typical X chromosome and one altered X chromosome (mosaic Turner syndrome) and in which X chromosomes are fragile and break (fragile X syndrome).
• Toxins. Chemotherapy and radiation therapy are common causes of toxin-induced ovarian failure. These therapies can damage genetic material in cells. Other toxins such as cigarette smoke, chemicals, pesticides and viruses might hasten ovarian failure.
• An immune system response to ovarian tissue (autoimmune disease). In this rare form, your immune system produces antibodies against your ovarian tissue, harming the egg-containing follicles and damaging the egg. What triggers the immune response is unclear, but exposure to a virus is one possibility.
• Unknown factors. The cause of primary ovarian insufficiency is often unknown (idiopathic). Your health care provider might recommend further testing to find the cause, but in many cases, the cause remains unclear.

Factors that increase your risk of developing primary ovarian insufficiency include:

• Age. The risk goes up between ages 35 and 40. Although rare before age 30, primary ovarian insufficiency is possible in younger women and even in teens.
• Family history. Having a family history of primary ovarian insufficiency increases your risk of developing this disorder.
• Ovarian surgery. Surgeries involving the ovaries increase the risk of primary ovarian insufficiency.

Complications of primary ovarian insufficiency include:

• Infertility. Inability to get pregnant can be a complication of primary ovarian insufficiency. In rare cases, pregnancy is possible until the eggs are depleted.
• Osteoporosis. The hormone estrogen helps maintain strong bones. Women with low levels of estrogen have an increased risk of developing weak and brittle bones (osteoporosis), which are more likely to break than healthy bones.
• Depression or anxiety. The risk of infertility and other complications arising from low estrogen levels causes some women to become depressed or anxious.
• Heart disease. Early loss of estrogen might increase your risk.

Most women have few signs of primary ovarian insufficiency, but your health care provider may suspect the condition if you have irregular periods or are having trouble conceiving. Diagnosis usually involves a physical exam, including a pelvic exam. Your provider might ask questions about your menstrual cycle, exposure to toxins, such as chemotherapy or radiation therapy, and previous ovarian surgery.

Your provider might recommend one or more tests to check for:

• Pregnancy. A pregnancy test checks for an unexpected pregnancy if you're of childbearing age and missed a period.
• Hormone levels. Your provider may check the levels of a number of hormones in your blood, including follicle-stimulating hormone (FSH), a type of estrogen called estradiol, and the hormone that stimulates breast milk production (prolactin).
• Chromosome changes or certain genes. You may have a blood test called a karyotype analysis to look for unusual changes in your chromosomes. Your doctor may also check to see if you have a gene associated with fragile X syndrome called FMR1.

Treatment for primary ovarian insufficiency usually focuses on the problems that arise from estrogen deficiency. Your health care provider might recommend:

• Estrogen therapy. Estrogen therapy can help prevent osteoporosis as well as relieve hot flashes and other symptoms of estrogen deficiency. Your provider may prescribe estrogen with the hormone progesterone, especially if you still have your uterus. Adding progesterone protects the lining of your uterus (endometrium) from precancerous changes that may be caused by taking estrogen alone.

  The combination of hormones may make your period come back, but it won't restore ovarian function. Depending on your health and preference, you might take hormone therapy until around age 50 or 51 — the average age of natural menopause.

  In older women, long-term estrogen plus progestin therapy has been linked to an increased risk of heart and blood vessel (cardiovascular) disease and breast cancer. In young women with primary ovarian insufficiency, however, the benefits of hormone therapy outweigh the potential risks.

• Calcium and vitamin D supplements. Both nutrients are important for preventing osteoporosis, and you might not get enough in your diet or from exposure to sunlight. Your provider might suggest bone density testing before starting supplements to get a baseline measurement.

  For women ages 19 through 50, experts generally recommend 1,000 milligrams (mg) of calcium a day through food or supplements, increasing to 1,200 mg a day for women age 51 and older.

  The optimal daily dose of vitamin D isn't yet clear. A good starting point for adults is 600 to 800 international units (IU) a day, through food or supplements. If your blood levels of vitamin D are low, your provider might suggest higher doses.

Addressing infertility

There's no treatment proved to restore fertility. Some women and their partners pursue pregnancy through in vitro fertilization using donor eggs. The procedure involves removing eggs from a donor and fertilizing them with sperm. A fertilized egg (embryo) is then placed in your uterus.

Learning that you have primary ovarian insufficiency may be emotionally difficult. But with proper treatment and self-care, you can expect to lead a healthy life.

• Learn about alternatives for having children. If you'd like to add to your family, talk to your health care provider about options such as in vitro fertilization using donor eggs or adoption.
• Talk with your provider about the best contraception options. A small percentage of women with primary ovarian insufficiency do spontaneously conceive. If you don't want to become pregnant, consider using birth control.
• Keep your bones strong. Eat a calcium-rich diet, do weight-bearing exercises such as walking and strength training exercises for your upper body, and don't smoke. Ask your provider if you need calcium and vitamin D supplements.
• Keep track of your menstrual cycle. If you miss a period while taking hormone therapy that causes you to have a monthly cycle, get a pregnancy test.

If you'd hoped for future pregnancies, a diagnosis of primary ovarian insufficiency can bring on overwhelming feelings of loss — even if you've already given birth. Seek counseling if you feel it would help you cope.

• Be open with your partner. Talk with and listen to your partner as you both share your feelings over this unexpected change in your plans for growing your family.
• Explore your options. If you don't have children and want them, or if you want more children, look into alternatives to expand your family, such as in vitro fertilization using donor eggs or adoption.
• Seek support. Talking with others who are going through something similar can provide valuable insight and understanding during a time of confusion and uncertainty. Counseling might help you adjust to your circumstances and the implications for your future. Ask your provider about national or local support groups or seek an online community as an outlet for your feelings and a source of information.
• Give yourself time. Coming to terms with your diagnosis is a gradual process. In the meantime, take good care of yourself by eating well, exercising and getting enough rest.

Your first appointment will likely be with your primary care provider or a gynecologist. If you're seeking treatment for infertility, you might be referred to a doctor who specializes in reproductive hormones and optimizing fertility (reproductive endocrinologist).

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test. Make a list of:

• Your symptoms, including missed periods and how long you've been missing them
• Key personal information, such as major stresses, recent life changes and your family medical history
• Your health history, especially your reproductive history, any past surgeries on your ovaries and possible exposure to chemicals or radiation
• All medications, vitamins or other supplements you take, including doses
• Questions to ask your provider

Take a family member or friend along, if possible, to help you remember all the information you're given.

For primary ovarian insufficiency, some questions to ask your provider include:

• What's the most likely cause of my irregular periods?
• What other possible causes are there?
• What tests do I need?
• What treatments are available? What side effects can I expect?
• How will these treatments affect my sexuality?
• What do you feel is the best course of action for me?
• I have other health conditions. How can I best manage them together?
• Should I see a specialist?
• Do you have printed material I can have? What websites do you recommend?

Don't hesitate to ask other questions as they occur to you during your appointment.

What to expect from your provider

Your provider is likely to ask questions, such as:

• When did you start missing periods?
• Do you have hot flashes, vaginal dryness or other menopausal symptoms? For how long?
• Have you had ovarian surgery?
• Have you been treated for cancer?
• Do you or any family members have systemic or autoimmune diseases, such as hypothyroidism or lupus?
• Have members of your family been diagnosed with primary ovarian insufficiency?
• How distressed do your symptoms make you feel?
• Do you feel depressed?
• Have you had difficulties with previous pregnancies?