This week I decided to address a less common hormonal health issue – non-classical congenital adrenal hyperplasia (NCCAH). Its symptoms can be almost identical to that of PCOS. So what is NCCAH? I will start with a definition of the bigger, more serious condition – congenital adrenal hyperplasia.


Congenital adrenal hyperplasia (CAH) is a group of inherited genetic disorders that affect the adrenal glands.

CAH affects the production of one or more of three steroid hormones: cortisol, which regulates your body’s response to illness or stress; mineralocorticoids, such as aldosterone, which regulate sodium and potassium levels; or androgens, such as testosterone, which are sex hormones. In many cases, CAH results in lack of cortisol and overproduction of androgen. [1]

The classic form can be detected at birth and is life-threatening. Non-classical congenital adrenal hyperplasia is simply a less severe form of CAH, one that starts later in life, sometimes at puberty, adolescence, or even young adults.


Symptoms of NCCAH are very similar to those of PCOS, which is why many women will get a PCOS diagnosis, without further investigations for NCCAH. It also doesn’t help that PCOS is much more common. Just like with PCOS and hypothalamic amenorrhea, getting the proper diagnosis is crucial, because treatment is different. Here are some of the most common symptoms of non-classical congenital adrenal hyperplasia:

  • acne
  • hirsutism
  • anovulation
  • oligomenorrhea
  • amenorrhea
  • low cortisol (poor stress tolerance, a tendency for anxiety)
  • polycystic ovaries
  • male-pattern baldness
  • clitoromegaly
  • early signs of puberty

Not all symptoms will be present. In fact, this is one of the reasons why NCCAH is so hard to diagnose – symptoms can vary a lot from one person to another. For some, androgens may be very high, for others almost normal. Some may have started puberty before 10, other too late, or others at a perfectly normal age. Unlike with PCOS, there is no Rotterdam criteria telling us how many symptoms are required for a diagnosis. NCCAH can only be diagnosed through blood work.

Diagnosis of non-classical congenital adrenal hyperplasia

Blood work is the only thing that can make a difference between NCCAH and PCOS. More specifically, screening should start with testing 17-hydroxyprogesterone (17-OHP). This should be tested in the follicular phase, preferably on day 2 or 3 of the menstrual cycle. Tested randomly, 17-OHP might have a normal value or be inconclusive. Generally, a result of 170-300 ng/dl warrants further investigation [2]. Some will only investigate further for values higher than 200, while others will investigate as soon as there’s an abnormality.

The definitive test is an ACTH stimulation test. For this, blood is first collected, then ACTCH is administered. 30-60 minutes later, another blood sample is collected. 17-OHP and progesterone concentrations are determined through this test. Unlike the simple 17-OHP test, ACTH leaves no room for false positives or false negatives.

Another test can be that of 21-hydroxylase. Patience with non-classical congenital adrenal hyperplasia may have a deficiency of 21-hydroxylase [4]. The ACTH stimulation test remains, however, the one definitive test out there. Despite the condition being genetic, genetic testing is actually not very useful in this case because there are 127 known mutations and few tests are able to actually detect all these mutations [1][3].

Myths surrounding NCCAH vs PCOS

There are a few myths that even some doctors will be inclined to listen to. Most endocrinologists should know the difference, but if you are talking to a GP who is not specifically trained on these conditions, they might be familiar with some of these myths.

For instance, a common misconception is that only lean women can have non-classical congenital adrenal hyperplasia and that the rest have PCOS. False! Lean women can have PCOS, just like overweight women can have NCCAH. Following this idea, it is believed that insulin resistance is not a part of NCCAH. While it is not common and not a diagnostic tool, insulin resistance can actually exist in women with NCCAH, especially in those who go undiagnosed for many years.

Another misconception I have heard refers to the early onset of puberty – if you didn’t have this, it can be NCCAH. FALSE! It can. It is why NCCAH is also called late-onset congenital adrenal hyperplasia. Yes, classical CAH starts at birth. But once we discuss late-onset, that can be prepubescent, but also much later. Many women notice symptoms just when those with PCOS do – when their periods become irregular or when they start getting unwanted body hair or because of severe cystic acne.

Treating non-classical congenital adrenal hyperplasia

Treatment of NCCAH usually depends on the severity of the symptoms. It is a genetic condition, so putting symptoms into remission is, sadly, more difficult than with PCOS.

Medical treatment requires working closely with a doctor. Birth control may be prescribed, but it is, of course, not a real treatment. Furthermore, the pill might increase anxiety, something detrimental for those suffering of NCCAH. Other treatments include hydrocortisone and/or corticosteroids, such as dexamethasone or prednisone. Most doctors will agree that these treatments should also be tried when the symptoms are severe (for instance, very bad hirsutism, or rapid hair loss). As I am not a doctor, I will not go into further discussion about these options, as they are best discussed with an endocrinologist.

Natural remedies can help alleviate symptoms and restore ovulation (if anovulation is a symptom).

  • Acupuncture can help restore ovulation, decrease anxiety, promote relaxation and even reduce inflammation.
  • Black cohosh can lower LH levels if needed and, taken in the follicular phase, it can help thicken the endometrial lining.
  • Evening primrose oil supports healthy cervical mucus, which is crucial for fertility and which is often absent in NCCAH.
  • Saw palmetto can help lower testosterone, thus decreasing male-pattern hair loss and cystic acne.
  • Vitex can support ovulation IF your LH is not already low. Just like with PCOS, LH can be high in women with NCCAH. If this is you, it’d be best to avoid vitex.
  • Spearmint tea reduces testosterone.
  • Inositol encourages ovulation. If you have insulin resistance, inositol is even more important as it helps fight this condition.

Final thoughts

Non-classical congenital adrenal hyperplasia can impact your chances of conceiving. However, this is not a must. The condition can be present in both males and females. As you probably noticed, I only discussed the condition as it presents in women and especially how it differs from PCOS. It is hereditary, so yes, you have a chance of passing it on, but only if your partner has it as well. That’s right! Both parents have to be carriers of a mutation for it to be present in the child.

If you’ve been diagnosed with PCOS (even if you’re insulin resistant) but no matter what you do, you’re not seeing improvements, ask your doctor to test for non-classical congenital adrenal hyperplasia. If your PCOS diagnosis was based on polycystic ovaries, irregular periods and high androgens but you are not insulin resistant, you have another reason to ask for a test. And if on top of your symptoms you add signs of low cortisol, such as constant and unexplained fatigue, anxiety, poor stress tolerance, you should ask to be tested for NCCAH.

  1. Congenital adrenal hyperplasia,
  2. Witchel S. Nonclassic congenital adrenal hyperplasia. Current Opinion in Endocrinology & Diabetes and Obesity. 2012;19(3):151-158. doi:10.1097/med.0b013e3283534db2
  3. Witchel, S. F., & Azziz, R. (2010). Nonclassic congenital adrenal hyperplasia. International journal of pediatric endocrinology2010, 625105. doi:10.1155/2010/625105
  4. Non-classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency

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