Saturday, February 1st, 2014
San Francisco, California
Hosted by Kate Tully, R.N. and Katherine Waidner, R.N.
Cushing’s Patient Advocates – Corcept Therapeutics
Agenda and details to follow
The day will focus on endogenous Cushing’s, a condition caused by high cortisol in your body.
The day will not cover exogenous Cushing’s caused by steroids taken for various health conditions including asthma, arthritis or lupus.
Tuesday, December 31, 2013
Cushing’s Awareness Patient Day
Saturday, February 1st, 2014
San Francisco, California
Hosted by Kate Tully, R.N. and Katherine Waidner, R.N.
Cushing’s Patient Advocates – Corcept Therapeutics
Agenda and details to follow
The day will focus on endogenous Cushing’s, a condition caused by high cortisol in your body.
The day will not cover exogenous Cushing’s caused by steroids taken for various health conditions including asthma, arthritis or lupus.
Wednesday, December 4, 2013
Tonight! Interview with Miriam K (Meeks089), Pituitary Success Story
Miriam writes in her bio:
"I suffered for eight long years with Cushings disease . I had surgery on August 1 , 2012 , I look like a different person , and act like a different person. I would love to share my journy . One that was an emotional roller coaster .It was a long hellish journey .However I would not trade it for anything else in the world.Although I suffered immensly, Cushings has made me who I am today. I have become strong from this disease. Although I suffered many symptoms, the emotional ones were by far the worst.I would love to be interveiwed because I vowed when I was ill to help people when I got better.I want to give people hope ."
MaryONote: Miriam will be interviewed on BlogTalkRadio podcasts December 4, 2013 at 6:00PM Eastern.
If you want to ask Miriam a question, please use the call-in number: (646) 200-0162
Archives will be available at Cushings-Help on BlogTalkRadio a few hours after the conclusion of this interview.
Thursday, November 28, 2013
Wednesday, November 20, 2013
Interview with Miriam K (Meeks089), Pituitary Success Story
Miriam writes in her bio:
"I suffered for eight long years with Cushings disease . I had surgery on August 1 , 2012 , I look like a different person , and act like a different person. I would love to share my journy . One that was an emotional roller coaster .
It was a long hellish journey .However I would not trade it for anything else in the world.
Although I suffered immensly, Cushings has made me who I am today. I have become strong from this disease. Although I suffered many symptoms, the emotional ones were by far the worst.
I would love to be interveiwed because I vowed when I was ill to help people when I got better.
I want to give people hope ."
MaryONote: Miriam will be interviewed on BlogTalkRadio podcasts December 4, 2013.
Friday, November 8, 2013
Rare Disease Week
The House & Senate Congressional Calendars came out this week!
Congress is in session during Rare Disease Week – SaveTheDate!
Feb. 25th – Feb. 28th, 2013
4 Days of Rare Disease Events in Washington DC!
More info coming soon.
Friday, October 25, 2013
Adrenal Insufficiency Video
A video about Adrenal Insufficiency and the need for emergency protocols.
An injection which costs about $10 could save a life.
Please help us make sure it's available to all who need it.
http://youtu.be/yKPnNNM_dIw
Thursday, September 26, 2013
Prince George’s woman works to raise awareness about rare disease
Lanham resident to speak at Patient Education Day event about Cushing’s disease
By Sophie Petit Staff Writer
Greg Dohler/The Gazette
Lanham resident Stacey L. Hardy, a survivor of Cushing’s disease, will speak about her experience with the pituitary disorder at an upcoming event at Johns Hopkins Hospital.
Stacy L. Hardy of Lanham described herself as athletic, which is why she became concerned when in 1996 she mysteriously gained 240 pounds that took five doctors 14 years to determine she had a potentially fatal disease.
Now Hardy said she wants to raise awareness among others who may unknowingly have Cushing’s disease, but are unaware of the symptoms and treatment.
It wasn’t until 2010 that Hardy, now 43, was diagnosed with the disease, a rare disorder that causes the body to release too much cortisol, the body’s stress or “fight or flight” hormone, said Gary Wand, a pituitary gland specialist at The Johns Hopkins Hospital in Baltimore.
Excess cortisol causes weight gain, especially in the stomach, extreme fatigue, muscle aches, anxiety and depression, he said.
“I didn’t even know what Cushing’s was. I was ready to just live with [the symptoms],” Hardy said, adding that by the time she was diagnosed she felt so tired she could barely move.
At 5 feet, 4 inches tall, Hardy said she reached 365 pounds during her struggle with the disease.
“We knew something for a while wasn’t right, but I never thought it would be something like that,” said Hardy’s daughter, Paij Hardy, 21, a student at Baltimore City Community College.
Just three out of every one million people are diagnosed with Cushing’s each year, said Wand, who estimates he sees 30 patients per year worldwide.
In 2011, Hardy underwent 16 hours of surgery at Sinai Hospital in Baltimore to remove four tumors from her pituitary gland, located at the base of the brain that controls the release of cortisol.
Today, she is 100 pounds lighter, with the weight still rapidly coming off, and said she is determined to serve as a lifelong support and education source for her fellow “cushies” — others with Cushing’s disease.
Hardy will speak Saturday at the Johns Hopkins Pituitary Gland Center’s fifth annual Patient Education Day, an event to raise awareness about the disease, Wand said.
Since the pituitary gland is the size of a kidney bean, Hardy underwent several brain scans before doctors, who previously suggested she might have leukemia or needed to diet and exercise more, could tell there were tumors on her gland, she said.
Hardy’s experience with delayed diagnosis and misdiagnosis is not unique, Wand said.
Cushing’s is a “subtle” disease, which is difficult to diagnose, and not everyone exhibits the same symptoms, he said.
If left untreated for more than a decade, the disease is fatal, but removing the pituitary gland tumors has proved extremely successful, Wand said.
“I’m evidence that there’s help out there,” Hardy said. “I can move. I can almost run. I can bend over and pick up a box. Oh my goodness, I can dance.”
Thursday, September 12, 2013
Enzyme linked to obesity
Researchers find that increased levels of an enzyme in fat cells lead to dangerous levels of abdominal obesity.
Previous studies have shown that the stress hormone cortisol can lead to an accumulation of fat round the abdomen. For instance, people with Cushing’s disease – where there’s excess cortisol in the blood – have too much abdominal fat. It’s bad for health to have fat in this area – it’s linked to diabetes and heart disease. That’s why it’s healthier to be a ‘pear shape’ rather than an ‘apple shape’. The distribution of fat in your body really does matter.
Researchers in Scotland and the US have now focussed upon an enzyme that produces cortisol to see what effect it has on abdominal fat. Working on mice genetically-modified to produce the enzyme – and therefore cortisol – in fat cells, they find that even a small increase in levels produces dramatic effects. The mice, compared with normal animals, gained fat in the abdominal area even on a low fat diet. They developed diabetes, high blood pressure, and also tended to eat more. It opens up the possibility of further studies on human obesity, and also perhaps could lead to therapies that block this enzyme and so reduce fat accumulation.
Wednesday, September 11, 2013
Hormonal disorder may make weight loss more challenging
(HealthDay News) -- Losing weight is never a piece of cake, but there are some medical conditions that may make it even more difficult.
The American Academy of Family Physicians says these conditions may make weight loss more of a challenge:
The American Academy of Family Physicians says these conditions may make weight loss more of a challenge:
- Hormonal disorders, such as diabetes, hypothyroidism, Cushing's disease and polycystic ovary syndrome.
- Cardiovascular problems, including heart-valve disorders and congestive heart failure.
- Disorders affecting sleep, including obstructive sleep apnea and upper airway resistance syndrome.
- Eating disorders, such as bulimia and carbohydrate craving syndrome.
Thursday, August 29, 2013
The Man Unable to Feel Fear
Jordy is a British man who has been dealing with Cushing's and many surgeries.
http://www.youtube.com/watch?v=CZaGnFTjSsc
He finds rollercoasters boring, barely broke a sweat zip-wiring off the Tyne bridge and even a parachute jump did not raise his heart rate.
Just a few years ago even the thought of daredevil exploits would have terrified him, but now Jordy Cernik is frightened of nothing.
While that might sound an ideal scenario, the 38-year-old’s new-found bravery is actually the unexpected side-effect of surgery for a rare condition.
Cushing’s Syndrome resulted in the dad-of-two having an operation to remove the gland which produces adrenalin, the hormone which makes us feel scared.
He says: “I would never have had the guts to do any of this, but now nothing fazes me. I’m up for anything – I’m even thinking about doing a wing-walk on a plane too.
“I nearly did a bungee jump a few years ago, but I just couldn’t do it.
"Now I just take whatever is thrown at me and if a challenge helps me raise money for charity, the more daring the better.”
Over the past four months he has completed the parachute jump and zip-wired from the top of Newcastle’s Tyne Bridge and now he is getting ready to complete the last of a trio of challenges – next month’s Bupa Great North Run.
“The doctors didn’t tell me this could be one of the side-effects of the operation,” says Jordy. “But then the condition is so rare I don’t think they know everything about Cushing’s yet.
“Doing the skydive was the ultimate test. I thought that if I was ever going to get scared again then that would be the moment.
"But as we took off in the plane I felt nothing, and when I edged towards the door to jump I felt nothing, and even when I leapt out and pulled my parachute, I didn’t feel scared at all.
“It can be quite frustrating as well though.
"The first time I realised I had changed was when I went on the rides at a theme park with my kids and I just didn’t feel a thing. I just sat there, bored.”
However, the last of his hat-trick of challenges, the Run, will require him to push through the ever-present pain which he has endured for years as a result of Cushing’s.
Britain’s biggest mass participation event, for which The Daily Mirror is a media partner, takes place over a 13.1 mile course from Newcastle to South Shields.
But the syndrome has left Jordy, from Jarrow, near Newcastle, with arthritis, back problems and brittle bones. Worse still, the absence of adrenalin means he now lacks one of the body’s natural painkillers.
“I’m always in pain,” he says. “I’ve just had to learn to zone it out day-to-day and I’m going to have to do that even more when I’m on the run.”
Cushing’s affects around one in 50,000 people in Britain.
It causes a malfunction of the adrenal and pituitary glands which means increased amounts of corticosteroids are produced – often leading to massive, irregular weight gain.
In just three years 5ft 8in Jordy ballooned from 11st 5lb to almost 17st.
While his limbs remained slim, the former Territorial Army recruit saw the pounds pile around the major organs in his torso and head.
“I went through years of hell and I can only describe it as living in someone else’s body,” says the part-time radio presenter and events host.
“I developed this big round moon face and really quite large man boobs, which was so embarrassing.
“But there was absolutely nothing I could do about it. I could go to the gym six days a week and still couldn’t lose any of the weight.
"One of the worst things was that people would stare.
"Sometimes they’d take the mickey – often to try and make me feel better, by making light of things – but it would almost always hurt my feelings.
“And my career as a presenter suffered. I tried to play up to the character of being a big, jolly chap but I always felt I was too fat for TV, which is what I would have liked to do a lot more of.”
But it was the effect on his home life with wife Tracy, 43, and daughters Aimee, seven, and four-year-old Eive that for him was far worse.
“I had other really difficult symptoms which included profuse sweating which meant I couldn’t even hold my kids without wrapping them in towels first,” he says.
“Anyone who has children knows how hard that is, not to be able to do normal things. I often used to be in tears.
"Another symptom was extreme grumpiness, so I would find myself suddenly getting really angry and just exploding at them, plus I was always too exhausted to play with them. It was terrible.”
Jordy believes he can trace his symptoms back 15 years although his Cushing’s was only diagnosed in 2005.
He had visited his local surgery with a string of complaints, but by chance saw a different doctor one day and the syndrome was diagnosed.
“I don’t have any ill-feeling about that,” he says, “because the syndrome can be tricky to spot, partly because it is so rare.”
He went on to have both his pituitary and adrenal glands removed but needed a total of seven operations between 2005 and 2010 and not all went smoothly.
During one to remove his pituitary gland, which is inside the skull, the lining of his brain burst due to the stress of repeated surgery.
And while removing a rib to access the adrenal gland in his torso, his lung was punctured.
That wasn’t the end of the complications. He later developed severe meningitis and ended up on a life-support machine.
“But I still consider myself lucky,” he says. “The doctors told me, ‘You died twice really, you shouldn’t even be here’.”
Things have begun to look up in the past few years, however. The Cushing’s is in remission and Jordy has lost four stone.
His life hasn’t returned to normal entirely – he still has to take 30 pills a day, a cocktail of painkillers and hormones, plus drugs to slow the corrosion of his bones.
He has also been diagnosed with another rare condition, sarcoidosis, which creates nodules of irregular cells in the body and can cause serious complications. He’s convinced he has always had it but it has lain dormant until his body was at its most vulnerable.
At present the nodules can only be found on his skin and he’s being monitored to ensure that it doesn’t spread to his internal organs.
Thanks to the surgery, his life has improved enormously since 2010.
In July he had a breast reduction op which not only improved his appearance but also removed the dangerous accumulation of fat around his heart.
Part of this new chapter involves taking part in the Great North Run and raising money for the Cash for Kids appeal run by his local radio station Metro Radio.
The appeal aims to help children and young people in the North East who are disabled or have special needs, or those who suffer from abuse or neglect.
Jordy’s fundraising goal is a relatively modest £1,000, but for him joining the half marathon’s 56,000 participants on September 15 will be as rewarding as hitting his target.
“I really don’t know if I’ll be able to complete the course.” he says. “But I’m looking forward to it and I’m going to give it my best shot.
“Not feeling fear may feel like the power of a superhero, but what I really need for the Great North Run is superhero strength.”
The Bupa Great North Run is Britain’s biggest mass participation event and is organised by Nova International.
It will include world class athletes Mo Farah, Haile Gebrselassie and Kenenisa Bekele – plus 56,000 other runners.
The event is live on BBC One on Sunday 15th September between 9.30am to 13.30
For more information, visit www.greatrun.org
Friday, August 23, 2013
2nd Annual Patient Advocacy Summit
Come join us for our 2nd Annual “RARE Patient Advocacy Summit”
to be held on Friday, September 20, 2013
at The Balboa Bay Club & Resort in Newport Beach, CA.
to be held on Friday, September 20, 2013
at The Balboa Bay Club & Resort in Newport Beach, CA.
Seating is limited for in-person participation.
Webcast registration available for those unable to attend in person.
From Symptom to Cure: The Journey of a Rare Disease Advocate ~ Equipping Patients to Make a Difference
Join Global Genes | RARE Project for a unique and interactive educational experience at our 2nd Annual Patient Advocacy Summit on Friday, September 20, 2013. There is no charge to participate in this event.
A rare diagnosis changes everything. It crashes plans and dreams, knocks you off your feet, and requires a continual investment of time and money as you try to determine what should be your next step. The purpose of the RARE Patient Advocacy Summit is to help patient ADVOCATES become successful ACTIVISTS and to provide the discussion, insights and tools to move down this advocacy path, equipped and prepared.
The summit will offer practical advice, case studies and networking opportunities as we learn from one another. The goal is to have patient advocates walk away with a better understanding of the challenges they will face and where they can be most effective in helping advocate for their disease/disorder.
Attendees will:
- Learn how to get started: obtain 501c3 status, write grants, leverage PR effectively and utilize social media to spread your message.
- Collaboration: Understand how to successfully work with other rare disease stakeholders, patient advocates, the FDA and other government entities.
- Learn the importance of patient registries, the different types of registries and how advocates can support them.
- Explore the role of foundations and advocates related to scientific discovery and drug development.
- Gain a broad understanding of the scientific process, including diagnostic and research methodologies and collaborations with academia and industry.
At the end of this day-long event, each participant will gain perspective on the complexities and questions that need to be considered in order to become effective advocates for the rare disease patients and help advance therapies in the rare diseases we represent.
Who Should Attend:
- Rare disease patients, caregivers, family members and friends
- Patient advocates
Whether you are new to this rare disease journey or an experienced traveler, an individual advocate or part of an existing rare disease organization, you will gain value from this event.
Sponsor Information
To become a sponsor or for more information, please contact Nicole Boice. We look forward to seeing you at this year’s summit.
Thursday, August 22, 2013
Seminar: Putting Patients First
September 30, 2013 | 9:30 am - 2:30 pm
W Hotel, 515 15th Street, NW, Washington, DC
W Hotel, 515 15th Street, NW, Washington, DC
How do patients, providers, and payers know whether health information is credible, accurate, useful or appropriate?
Comparative effectiveness research (CER) has the potential to improve health outcomes by helping people make better-informed decisions. But how do we know that CER will generate information that is useful?
You can help us find the answers by joining us on September 30 for a conversation and symposium featuring a broad range of health care stakeholders—patients, providers, policymakers, payers, researchers, and those who fund research.
We’ll focus on an effort led by the National Health Council to create a framework to guide the development of CER, evaluate its results, and assist in communicating the findings to the right audiences.
Be a part of the conversation—register today and add your voice to our efforts to make CER useful. Tweet about it using #useCER.
Sunday, August 18, 2013
Bio Categories
- Acromegaly
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Monday, August 12, 2013
ShineOut, an Information and Support Program for Cushing's Disease
ShineOut is an information and support program for people with Cushing’s disease, and their friends and family.
Sign up here: ShineOut
Sunday, August 11, 2013
Cyclic Cushing’s syndrome: a clinical challenge
+Author Affiliations
- 1Department of Internal Medicine, Canisius Wilhelmina Ziekenhuis, PO Box 9015, 6500 GS Nijmegen, The Netherlands and 2Department of Endocrinology, University Medical Centre Groningen, University of Groningen, PO Box 30001, 9700 RB Groningen, The Netherlands
- (Correspondence should be addressed to: R P F Dullaart; Email:r.p.f.dullaart@int.umcg.nl)
Abstract
Cyclic Cushing’s syndrome (CS) is a rare disorder, characterized by repeated episodes of cortisol excess interspersed by periods of normal cortisol secretion. The so-called cycles of hypercortisolism can occur regularly or irregularly with intercyclic phases ranging from days to years.
The majority of patients with cyclic CS have clinical signs of CS, which can be either fluctuating or permanent. In a minority of patients, clinical signs of CS are absent. The fluctuating clinical picture and discrepant biochemical findings make cyclic CS extremely hard to diagnose. Clinicians should therefore be aware of this clinical entity and actively search for it in all patients with suspected CS but normal biochemistry or vice versa.
Frequent measurements of urinary cortisol or salivary cortisol levels are a reliable and convenient screening tool for suspected cyclic CS. Cortisol stimulation or suppression tests may give spurious results owing to spontaneous falls or rises in serum cortisol at the time of testing. When cyclic CS is biochemically confirmed, further imaging and laboratory studies are guided by the presence or absence of ACTH dependency. In cases of suspected ectopic ACTH production, specific biochemical testing for carcinoids or neuroendocrine tumours is required, including measurements of serotonin in platelets and/or urine, chromogranin A and calcitonin.
Read the entire article here: http://www.scribd.com/doc/159503297/Cyclic-Cushing%E2%80%99s-syndrome-a-clinical-challenge
Saturday, August 3, 2013
FDA Puts Strict Limits on Oral Ketoconazole Use
By John Gever, Deputy Managing Editor, MedPage Today
SILVER SPRING, Md. -- Oral ketoconazole (Nizoral) should never be used as first-line therapy for any type of fungal infection because of the risk of liver toxicity and interactions with other drugs, the FDA said Friday.
The agency ordered a series of label changes and a new medication guide for patients that emphasize the risks, which also include adrenal insufficiency. It noted that the restrictions apply only to the oral formulation, not topical versions.
Late Thursday, the chief advisory body for the FDA's European counterpart went further. The EU's Committee on Medicinal Products for Human Use (CHMP) recommended that member nations pull oral ketoconazole from their markets entirely.
Both the FDA and the CHMP cited studies indicating high risks of severe, acute liver injury in patients taking the drug. Studies using the FDA's adverse event reporting system and a similar database in the U.K. indicated that liver toxicity was more common with oral ketoconazole than with other anti-fungals in the azole class.
The FDA also said that oral ketoconazole "is one of the most potent inhibitors" of the CYP3A4 enzyme. This effect can lead to sometimes life-threatening interactions with other drugs metabolized by CYP3A4, and also to adrenal insufficiency, since the enzyme also catalyzes release of adrenal steroid hormones.
"This accounts for clinically important endocrinologic abnormalities observed in some patients (particularly when the drug is administered at high dosages), including gynecomastia in men and menstrual irregularities in women," the FDA said.
The only indication for oral ketoconazole still supported by the FDA is for use in life-threatening mycoses in patients who cannot tolerate other anti-fungal medications or when such medications are unavailable.
In such instances, the FDA said, physicians should assess liver function before starting the drug. It is contraindicated in patients with pre-existing liver disease, and patients should be instructed not to drink alcohol or use other potentially hepatotoxic drugs.
Adrenal function should also be monitored in patients using the drug.
The CHMP also indicated the topical formulations of ketoconazole should stay on the market, but it found no basis for keeping the oral form available for any purpose.
"Taking into account the increased rate of liver injury and the availability of alternative anti-fungal treatments, the CHMP concluded that the benefits did not outweigh the risks," the panel indicated in a statement.
It recommended that physicians stop prescribing oral ketoconazole and that they should review alternatives in patients currently receiving the drug. The committee also said that patients now taking oral ketoconazole "make a non-urgent appointment" with their physicians to discuss their treatment.
From MedPage Today
Saturday, July 13, 2013
Cushing's Disease - Rare Disease Quick Facts
Cushing's disease is a rare condition due to excess cortisol levels that result from a pituitary tumor secreting adrenocorticotropic hormone (ACTH), which stimulates cortisol secretion. Cushing’s disease should not be confused with Cushing’s syndrome which is increased cortisol levels but that increase can be due to any number of factors. However, Cushing’s disease is the most common form of Cushing’s syndrome.
Symptoms
The symptoms related to Cushing's disease and Cushing's syndrome are the same, since both are related to an excess of cortisol. Also, symptoms vary extensively among patients and that, with the inherent fluctuation in hormone levels make it difficult to diagnosis both conditions.
Changes in physical characteristics of the body
- Fullness and rounding of the face
- Added fat on back of neck (so-called "buffalo hump")
- Easy bruising
- Purplish stretch marks on the abdomen (abdominal striae)
- Excessive weight gain, especially in abdominal region
- Red cheeks
- Excess hair growth on the face, neck, chest, abdomen and thighs
Changes in physiology/psychology
- Generalized weakness and fatigue
- Menstrual disorder
- Decreased fertility and/or sex drive
- High blood pressure that is often difficult to treat
- Diabetes mellitus
- Mood and behavior disorders
Diagnosis
The early stages of Cushing's disease may be difficult to recognize. However, if it is suspected, diagnosis is generally a 2 stage process. First to determine if cortisol levels are high, and if so, why they are high.
Tests to confirm high cortisol levels:
- 24-hour urine cortisol
- Dexamethasone suppression test (low dose)
Tests to determine cause:
- Blood ACTH level
- Brain MRI
- Corticotropin-releasing hormone test
- Dexamethasone suppression test (high dose)
- Petrosal sinus sampling
Treatment
Surgery
- Most patients with Cushing’s disease undergo surgery to remove the pituitary adenoma offers.
- If the tumor is isolated to the pituitary, cure rates of 80-85% are common.
- If the tumor has spread to nearby organs, cure rates of 50-55% are common.
Medicine (approved orphan drugs)
- Approved for patients with Cushing’s disease for whom pituitary is not an option or surgery has been ineffective.
- Signifor is a somatostatin receptor agonist that leads to inhibition of ACTY secretion (and subsequently decreased cortisol levels).
- Approved for patients with Cushing’s syndrome who have type 2 diabetes or glucose intolerance and have failed surgery (or not candidates for surgery).
- Korlym is a glucocorticoid receptor antagonist which in turn blocks the effects of the high levels of cortisol in the body. Korlym is used to treat high glucose levels due to elevated cortisol.
Medicines used but not indicated for Cushing’s disease include
Radiation
- Radiation therapy may be used in some patients and can be very effective in controlling the growth of these tumors.
Prognosis
In most cases, treatment can cure Cushing’s disease. If not treated properly, the chronic hypercortisolism can lead to excess morbidity and mortality due to increased cardiovascular and other risk factors.
For more information
Images courtesy of the open access journal Orhanet Journal for Rare Diseases. Castinetti et al. Orphanet J Rare Dis. 2012 7:41 doi:10.1186/1750-1172-7-41
Friday, July 12, 2013
From The Endocrine Society’s Annual Meeting: Pituitary tumor size not definitive for Cushing's
SAN FRANCISCO – The size of a pituitary tumor on magnetic resonance imaging in a patient with ACTH-dependent Cushing’s syndrome can’t differentiate between etiologies, but combining that information with biochemical test results could help avoid costly and difficult inferior petrosal sinus sampling in some patients, a study of 131 cases suggests.
If MRI shows a pituitary tumor larger than 6 mm in size, the finding is 40% sensitive and 96% specific for a diagnosis of Cushing’s disease as the cause of adrenocorticotropic hormone (ACTH)-dependent Cushing’s syndrome, and additional information from biochemical testing may help further differentiate this from ectopic ACTH secretion, Dr. Divya Yogi-Morren and her associates reported at the Endocrine Society’s Annual Meeting.
Pituitary tumors were seen on MRI in 6 of 26 patients with ectopic ACTH secretion (23%) and 73 of 105 patients with Cushing’s disease (69%), with mean measurements of 4.5 mm in the ectopic ACTH secretion group and 8 mm in the Cushing’s disease group. All but one tumor in the ectopic ACTH secretion group were 6 mm or smaller in diameter, but one was 14 mm.
Because pituitary "incidentalomas" as large as 14 mm can be seen in patients with ectopic ACTH secretion, the presence of a pituitary tumor can’t definitively discriminate between ectopic ACTH secretion and Cushing’s disease, said Dr. Yogi-Morren, a fellow at the Cleveland Clinic.
That finding contradicts part of a 2003 consensus statement that said the presence of a focal pituitary lesion larger than 6 mm on MRI could provide a definitive diagnosis of Cushing’s disease, with no further evaluation needed in patients who have a classic clinical presentation and dynamic biochemical testing results that are compatible with a pituitary etiology (J. Clin. Endocrinol. Metab. 2003;88:5593-602). The 6-mm cutoff, said Dr. Yogi-Morren, came from an earlier study reporting that 10% of 100 normal, healthy adults had focal pituitary abnormalities on MRI ranging from 3 to 6 mm in diameter that were consistent with a diagnosis of asymptomatic pituitary adenomas (Ann. Intern. Med. 1994;120:817-20).
A traditional workup of a patient with ACTH-dependent Cushing’s syndrome might include a clinical history, biochemical testing, neuroimaging, and an inferior petrosal sinus sampling (IPSS). Biochemical testing typically includes tests for hypokalemia, measurement of cortisol and ACTH levels, a high-dose dexamethasone suppression test, and a corticotropin-releasing hormone (CRH) stimulation test. Although IPSS is the gold standard for differentiating between the two etiologies, it is expensive and technically difficult, especially in institutions that don’t regularly do the procedure, so it would be desirable to avoid IPSS if it’s not needed in a subset of patients, Dr. Yogi-Morren said.
The investigators reviewed charts from two centers (the Cleveland Clinic and the M.D. Anderson Cancer Center, Houston) for patients with ACTH-dependent Cushing’s syndrome seen during 2000-2012.
ACTH levels were significantly different between groups, averaging 162 pg/mL (range, 58-671 pg/mL) in patients with ectopic ACTH secretion, compared with a mean 71 pg/mL in patients with Cushing’s disease (range, 16-209 pg/mL), she reported. Although there was some overlap between groups in the range of ACTH levels, all patients with an ACTH level higher than 210 pg/mL had ectopic ACTH secretion.
Median serum potassium levels at baseline were 2.9 mmol/L in the ectopic ACTH secretion group and 3.8 mmol/L in the Cushing’s disease group, a significant difference. Again, there was some overlap between groups in the range of potassium levels, but all patients with a baseline potassium level lower than 2.7 mmol/L had ectopic ACTH secretion, she said.
Among patients who underwent a high-dose dexamethasone suppression test, cortisol levels decreased by less than 50% in 88% of patients with ectopic ACTH secretion and in 26% of patients with Cushing’s disease.
Most patients did not undergo a standardized, formal CRH stimulation test, so investigators extracted the ACTH response to CRH in peripheral plasma during the IPSS test. As expected, they found a significantly higher percent increase in ACTH in response to CRH during IPSS in the Cushing’s disease group, ranging up to more than a 1,000% increase. In the ectopic ACTH secretion group, 40% of patients did have an ACTH increase greater than 50%, ranging as high as a 200%-300% increase in ACTH in a couple of patients.
"Although there was some overlap in the biochemical testing, it is possible that it provides some additional proof to differentiate between ectopic ACTH secretion and Cushing’s disease," Dr. Yogi-Morren said.
In the ectopic ACTH secretion group, the source of the secretion remained occult in seven patients. The most common identifiable cause was a bronchial carcinoid tumor, in six patients. Three patients each had small cell lung cancer, a thymic carcinoid tumor, or a pancreatic neuroendocrine tumor. One patient each had a bladder neuroendocrine tumor, ovarian endometrioid cancer, medullary thyroid cancer, or a metastatic neuroendocrine tumor from an unknown primary cancer.
The ectopic ACTH secretion group had a median age of 41 years and was 63% female. The Cushing’s disease group had a median age of 46 years and was 76% female.
Dr. Yogi-Morren reported having no financial disclosures.
From Famiiy Practice News
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