High ACTH levels despite steroid replacement

Good afternoon, everyone! I have an interesting situation, and I would appreciate your thoughts and insight.

When I was first diagnosed with primary adrenal insufficiency, my ACTH levels were found to be very high, in the 9000 pg/mL range. The endocrinologist that I originally saw started me on 10 mg of hydrocortisone (HC) in the morning, and 10 mg of HC at night. A few months later, my ACTH levels were found to have decreased to around 700 pg/mL, but the endocrinologist had me increase my dosage to 20 mg HC in AM, and 10 mg HC in PM. At one point about a year ago, my ACTH levels measured as low as 72 pg/mL, which is just above the normal range.

In February of this year, my ACTH levels were found to have gone back up to 1,800 pg/mL. As a result of this test, my former endocrinologist then had me increase my HC dosage to 20 mg AM, and 20 mg PM, and added fludrocortisone 0.1 mg daily. When more blood work a month later found my ACTH levels to still be in the 1,800 pg/mL range, my former endocrinologist had me increase HC dosage yet again, to 40 mg AM, and 20 mg PM. At this point I emailed him asking if this high of a dosage could have negative effects in the future, such as osteoporosis or cataracts. He replied tersely, saying that I shouldn't worry about that, and that my HC dosage needed to be as high as necessary to bring down my ACTH levels. The implication was that a high ACTH could kill a person with adrenal insufficiency.

Due to a change in my wife's and my insurance coverage, I decided to switch providers, and saw a new endocrinologist a few weeks ago. She was somewhat shocked to learn that my former endocrinologist had continually ramped up my HC dosage every time he found my ACTH levels to be higher than a normal range. She told me what was most important was that I was receiving enough steroid replacement, and that my ACTH levels may never come down to a normal range again. She also stated that to her knowledge, a high ACTH level on its own is not dangerous. My ACTH levels most recently measured at 1,080 pg/mL a week and a half ago. The new endocrinologist's recommendation now is to slowly taper down my HC dosage over several weeks, ending up back at a maintenance dosage of 20 mg AM, and 10 mg PM.

I wonder how two specialists in managing Addison's disease or primary adrenal insufficiency can have such opposing recommendations. For those of you with adrenal insufficiency, have you found your ACTH levels to still be significantly above normal, despite steroid replacement? Have the endocrinologists that you have seen been concerned about a continued high ACTH level? Have they ever stated that a high ACTH level alone could be dangerous to your health?

Thank you for your replies. Perhaps this could spark another discussion on what to do when our doctors contradict each other. I hope that you are all doing well.

4 Replies

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  • Hi Aaron,

    Someone on this board had also posted he always has high ACTH levels but is taking adequate dosage and there was no cause for concern. My endo has the same viewpoint as your previous one. I've had Addison's for 38 years and I started with 10 mg/am and 5 mg/ pm of cortisone for many years. Because of my chronic fatigue complaint, my endo about a year ago suggested an increase to 5 mg of Prednisone (I had switched for its longer half life which equals to 20 mg cortisone) . When my ACTH levels did not decrease satisfactory, he had me increase to 6 mg and most recently to 7 mg which I did not do since I already have osteoporosis and I did not want further risk (my brother also has Addison's and osteoporosis). I always thought it was ridiculous logic to continually increase your cortisone to lower ACTH. Your blood cortisone is only a snapshot in time of what your levels are. Perhaps the variance is attributed to what time you took your morning cortisone. I also read on an Addison's online community board that dosage >25 mg increases adverse effects.

    I now know that my fatigue is primarily caused by AMN's spasticity. When I go to the gym, I take 4 g of prednisone/am and 2.5 mg of cortisone before I leave and an additional 2.5 mg after my workout. I'd be interested in what others have to say and their ACTH levels.

    Note: I just reviewed ACTH levels since 2009. High was 905 and low was 50.

    One lab test: ACTH was 580 pg/mL (Standard Range <=46 pg/mL) and morning cortisol was 1.5 ug/dL (Standard Range 8.0 - 25.0 ug/dL).

  • I have had widely fluctuating ACTH levels like yours in the past.

    In my experience with several endocrinologists managing my Addison's, I have discovered, like you, that there are two schools on this matter. The first looks at the ACTH level to determine how they should treat your Addison's generally (i.e. dosages, when to take it). But in my experience, these endos don't necessarily expect your levels to be in the "normal" range. The second focus more on symptoms---how are you feeling? Fatigue? Weight loss or gain? Often this approach will accompany an ACTH test to "check in," if you will. I do believe, and have heard from endos, that excessively high ACTH over the long run can be dangerous and tumor-forming in the pituitary gland. So, in my experience, an elevated ACTH is not unusual for Addison's patients, but ACTH levels still matter. I much prefer the latter approach: how are you feeling?

  • Hello Aaron, my situation has some differences and some similarities. I am 51, diagnosed six years ago. I am officially "borderline Addisonian" which translates into normal cortisol but very high ACTH (average of 700, or 14 times normal).

    Basically my anterior pituitary gland works overtime to allow my adrenal gland to produce a normal level of cortisol. I don't take steroids but I have them with me at all times in case of "adrenal crisis".

    I am supposed to get tested every three months, but in the absence of symptoms (nausea, fatigue, faintness, hyper-pigmentation), I do it every 6 months or so.

    My experience with endos is that 95% of them are thyroid-and-diabetes monkeys, and you have to find the one who knows about adrenal function. My wonderful endo is Anne Porzig at Palo Alto Medical Foundation (CA) but all hell broke loose when she was out on maternity leave and the regular bozos took over.

  • Thanks for your replies. It seems clear that managing adrenal insufficiency is not an exact science, and even the experts differ in their preferred methods.

    What is not always clear is whether the fatigue we often experience is a neurological or adrenal issue (or both). We may never receive definitive answers on this.

    Keep pressing on, everyone!