A Positive Corticotropin-Releasing Hormone Test May Obviate the Need For Petrosal Sinus Sampling in Patients With Cushing’s Disease

Posted on May 9, 2026 by MaryO

Abstract

Purpose

Bilateral inferior petrosal sinus sampling (BIPSS) is considered the most accurate procedure to confirm the diagnosis of Cushing’s disease (CD). However, it is a cumbersome and costly procedure. We aim to determine whether the corticotropin-releasing hormone (CRH) test alone might differentiate CD from ectopic adrenocorticotropin (ACTH) syndrome (EAS).

Methods

In this retrospective, observational study we investigated 523 consecutive patients with ACTH-dependent hypercortisolism: 502 with CD and 21 with EAS. All patients underwent a CRH test and surgery for the removal of the ACTH-secreting tumor. Two hundred patients with CD and 17 with EAS underwent BIPSS. The diagnostic CRH test performance included sensitivity (SE), specificity (SP), positive predictive value (PPV), negative predictive value (NPV), accuracy, positive likelihood ratio (PLR), and negative likelihood ratio (NLR).

Results

using a 50% ACTH increase as the threshold, the CRH test correctly classified 438 cases of CD (87%) and 20 cases of EAS (95%). The diagnostic metrics were as follows: SE 87%, SP 95%, PPV 99%, NPV 23%, accuracy 92%, PLR 18.35, and NLR 0.05. BIPSS correctly diagnosed all cases of EAS (100%) and 188 cases of CD (94%).

Conclusions

Our study shows that a positive CRH test reliably differentiates CD from EAS and obviates the need for BIPSS. The performance of CRH test is similar in patients with and without a visible pituitary microadenoma or a microadenoma < 6 mm. Accepting the very low probability of EAS in a patient with positive ACTH response to CRH, we can propose pituitary surgery without performing BIPSS.

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Day 26, Cushing’s Awareness Challenge

Posted on April 26, 2026 by MaryO

So often during the diagnosis phase of Cushing’s I felt lost like this picture – I was walking alone to an unknown place with an unknown future.

My diagnosis was pre-Internet which meant that any information had to be gotten from libraries, bookstores, magazines…or doctors.  In 1983 to 1986 I knew something was terribly wrong but there was no backup from doctors, family or friends.  My first hope was from a magazine (see Day Six)

After I got that first glimmer of hope, it was off to the library to try to understand medical texts.  I would pick out words I did understand – and it was more words each trip.  I made Xerox copies of my findings to read at home and try to digest. (I still have all those old pages!)

All my research led me to Cushing’s.

Unfortunately, the research didn’t lead me to doctors who could help for several years.  That contributed greatly to the loneliness.  If a doctor says you’re not sick, friends and family are going to believe the doctor, not you.  After all, he’s the one trained to know what’s wrong or find out.

I was so grateful when I finally got into a clinical trial at NIH and was so nice not to be alone with this mystery illness.  I was also surprised to learn, awful as I felt, there were Cushies much worse off than I was.

I am so glad that the Internet is here now helping us all know that we’re not alone anymore.

We’re all in this together with help, support, research, just being there.  I love this quote from Catherine at http://wheniwasyou.wordpress.com/2012/03/31/wheniwasyou/

Mary, I am delighted to see you here. Cushings – because of the persistent central obesity caused by (we know now) the lack of growth hormone plus the hypothyroidism I was diagnosed with (but for which treatment was ineffective due to my lack of cortisol) – was one of the things I considered as an explanation for my symptoms. Your site was enormously educational and helpful to me in figuring out what might be happening to me. Those other patient testimonies I referred to? Many of them were the bios you posted. Thank you so much for commenting. I am so grateful for the support and encouragement. I really hope that my experiences will help other undiagnosed hypopituitary patients find their way to a diagnosis. I often used to dream that one day I’d get to say to others what was so often said to me: don’t give up, there will be an answer. I kept believing in myself because people I hadn’t even met believed in me. Now I am finally here and I do hope my story will help others to have faith in their own instincts.

Thanks again. Please do keep in touch.

Catherine

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Day 9, Cushing’s Awareness Challenge

Posted on April 9, 2026 by MaryO

Uh, Oh – I’m nearly a day late (and a dollar short?)…and I’m not yet sure what today’s topic will be.  I seem swamped by everything lately, waking up tired, napping, going to bed tired, waking up in the middle of the night, traveling, work, starting all over again…and my DH was recently diagnosed with cancer which makes everything more hectic and tiring.

It’s been like this since I was being diagnosed with Cushing’s in the mid-1980’s.  You’d think  things would be improved in the last 29 years.  But, no.

My mind wants things to have improved, so I’ve taken on more challenges, and my DH has provided some for me (see one of my other blogs, MaryOMedical).

Thank goodness, I have only part-time jobs (4 0f them!), that I can mostly do from home.  I don’t know how anyone post-Cushing’s could manage a full-time job!

I can see this post morphing into the topic “My Dream Day“…

I’d wake up refreshed and really awake at about 7:00AM and take the dog out for a brisk run.

Get home about 8:00AM and start on my website work.

Later in the morning, I’d get some bills paid – and there would be enough money to do so!

After lunch, out with the dog again, then practice the piano some, read a bit, finish up the website work, teach a few piano students, work on my church job, then dinner.

After dinner, check email, out with the dog, maybe handbell or choir practice, a bit of TV, then bed about 10PM

Nothing fancy but NO NAPS.  Work would be getting done, time for hobbies, the dog, 3 healthy meals.

Just a normal life that so many take for granted. Or, do they?

me-tired

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Day 6: Cushing’s Awareness Challenge

Posted on April 6, 2026 by MaryO

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The above is the official Cushing’s path to a diagnosis but here’s how it seems to be in real life:

Egads!  I remember the naive, simple days when I thought I’d give them a tube or two of blood and they’d tell me I had Cushing’s for sure.

Who knew that diagnosing Cushing’s would be years of testing, weeks of collecting every drop of urine, countless blood tests, many CT and MRI scans…

Then going to NIH, repeating all the above over 6 weeks inpatient plus an IPSS test, an apheresis (this was experimental at NIH) and specialty blood tests…

The path to a Cushing’s diagnosis is a long and arduous one but you have to stick with it if you believe you have this Syndrome.

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Day 1: Cushing’s Awareness Challenge

Posted on April 1, 2026 by MaryO

April is always Cushing’s Awareness Challenge month because Dr. Harvey Cushing was born on April 8th, 1869.

30-posts

Thanks to Robin for this wonderful past logo!  I’ve participated in these 30 days for Cushing’s Awareness several times so I’m not quite sure what is left to say this year but I always want to get the word out when I can.

As I see it, there have been some strides the diagnosis or treatment of Cushing’s since last year.  More drug companies are getting involved, more doctors seem to be willing to test, a bit more awareness, maybe.

April Fool's Day

How fitting that this challenge should begin on April Fool’s Day.  So much of Cushing’s  Syndrome/Disease makes us Cushies seem like we’re the April Fool.  Maybe, just maybe, it’s the doctors who are the April Fools…

Doctors tell us Cushing’s is too rare – you couldn’t possibly have it.  April Fools!

All you have to do is exercise and diet.  You’ll feel better.  April Fools!

Those bruises on your legs?  You’re just clumsy. April Fools!

Sorry you’re growing all that hair on your chin.  That happens as you age, you know.  April Fools!

Did you say you sleep all day?  You’re just lazy.  If you exercised more, you’d have more energy. April Fools!

You don’t have stretch marks.  April Fools!

You have stretch marks but they are the wrong [color/length/direction] April Fools!

The hump on the back of your neck is from your poor posture. April Fools!

Your MRI didn’t show a tumor.  You couldn’t have Cushing’s. April Fools!

This is all in your mind.  Take this prescription for antidepressants and go home.  April Fools!

If you have this one surgery, your life will get back to normal within a few months. April Fools!

What?  You had transsphenoidal surgery for Cushing’s?  You wasted your time and money. April Fools!

I am the doctor.  I know everything.  Do not try to find out any information online. You could not have Cushing’s.  It’s too rare…  April FOOL!

All this reminds me of a wonderful video a message board member posted a while ago:

So now – who is the April Fool?  It wasn’t me.  Don’t let it be you, either!

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