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Dr Mehta presented three cases of Cushing’s syndrome with var-
ied presentations, etiologies, and management.
Case 1
A 71-year-old male with coronary artery disease presented with
fatigue and weakness, Cushingoid features, insulin resistance,
and hypokalemic metabolic alkalosis. Laboratory evaluation
showed an elevated 24-h urinary free cortisol and ACTH level.
Pituitary MRI revealed no mass. He failed 1 and 8 mg dex-
amethasone suppression tests. Both whole body PET and
octreotide scans, however, showed no evidence of a source
of ectopic ACTH.
Subsequently, the patient underwent inferior petrosal sinus
sampling, results of which were consistent with a pituitary
source of ACTH. Total hypophysectomy was performed for
a presumed pituitary microadenoma, but pathology instead
showed corticotrophic hyperplasia.
At publication, the patient continued to suffer from persis-
tent Cushing’s syndrome, which is managed medically with
mifepristone. Recent intolerances, however, have led to con-
sideration of bilateral adrenalectomy.
Case 2
A 35-year-old male developed diabetes mellitus, coronary
artery disease, pulmonary embolism, and osteoporosis over
a 1-year period. He was noted to harbor Cushingoid features
and hypokalemic metabolic alkalosis. Random cortisol and
ACTH levels were very elevated. Pituitary MRI revealed a
3-mm microadenoma, yet he failed the 8 mg dexamethasone
suppression test.
The patient then underwent inferior petrosal sinus sampling,
results of which were consistent with a pituitary etiology. Pitu-
itary microadenoma was resected, and pathology confirmed
ACTH-secreting adenoma. At publication, he was being mon-
itored closely postoperatively and exhibited signs of adrenal
insufficiency.
Case 3
A 40-year-old female with history of metastatic pancreatic
neuroendocrine tumor presented with altered mental status,
skin hyperpigmentation, and hypokalemic metabolic alkalo-
sis. Laboratory evaluation showed an elevated 24-h urinary
cortisol and ACTH level.
Results of high-dose dexamethasone suppression and cor-
ticotropin-releasing hormone stimulation tests suggested
ectopic ACTH secretion. Pituitary MRI did not reveal any sig-
nificant mass. ACTH staining of previously resected ovarian
metastases of pancreatic neuroendocrine tumor showed pos-
itive immunoreactivity, confirming the source of ACTH. Given
the lack of surgical options due to metastatic disease, she is
being managed with combination octreotide, ketoconazole,
and mifepristone.
Dr Mehta concluded that the three cases demonstrate the diffi-
culty of applying the theoretical diagnostic algorithm to determine
the etiology of Cushing’s syndrome. In addition, they demonstrate
that treatment for Cushing’s syndrome should be individualized.
PracticeUpdate Editorial Team
Guidelines for treating the new
cardiovascular “extreme risk”
category have been validated
More intense treatment and intervention for
dyslipidemia has been recommended by the American
Association of Clinical Endocrinologists and American
College of Endocrinology, and a new “extreme risk”
category has been introduced. The updated guidelines
and new risk category were presented at AACE 2017.
P
aul Jellinger, MD, of Memorial Regional Hospital South,
Hollywood, Florida, and Yehuda Handelsman, MD, of
Providence Tarzana Medical Center, Tarzana, California,
highlighted the patient benefits of the recently introduced clin-
ical guidelines.
Drs Jellinger and Handelsman underscored the application of
more aggressive treatment to reduce low-density lipoprotein
cholesterol (LDL-C) in:
•
Patients with progressive atherosclerotic cardiovascular dis-
ease who have achieved an LDL under 70 mg/dL.
•
Those with established atherosclerotic cardiovascular disease
and diabetes, stage 3 or 4 kidney chronic kidney disease, or
heterozygous familial hypercholesterolemia.
•
Those with a history of premature cardiovascular disease
Patients with the above characteristics are now categorized
as being at cardiovascular “extreme risk.” Treatment goals for
these patients include:
•
LDL cholesterol <55 mg/dL
•
Non-HDL cholesterol <80 mg/dL
•
ApoB <70 mg/dL
Coronary artery calcium score and inflammatory markers are
also valuable in stratifying risk.
The groundbreaking guidelines also assess the following:
•
Adding ezetimibe and PCSK9 inhibitors in patients with car-
diovascular disease who are unable to reach LDL cholesterol
goals with statin therapy.
•
Screening for cardiovascular risk in female patients using the
Reynolds Risk Score or Framingham Risk Assessment Tool.
•
Special guidance for the diagnosis and management of dys-
lipidemia in children and adolescents as early as possible to
decrease the long-term risk of adult cardiovascular events.
Dr Jellinger said, “While suggestive evidence pointed in the
direction of better outcomes with more aggressive LDL treat-
ment, IMProved Reduction of Outcomes: Vytorin Efficacy
International Trial (IMPROVE-IT) was the first prospective trial
to demonstrate that driving LDL down to 53 provided a clear,
significant benefit in reducing cardiovascular outcomes in high-
risk groups.”
“By expanding the group of patients studied in IMPROVE-IT to
other very high-risk situations, our knowledge base expanded con-
siderably. The new information led to the creation of the new risk
category, a broader range of disease stages, and accompanying
groundbreaking treatment and intervention recommendations.”
PracticeUpdate Editorial Team
AACE 2017
11
VOL. 1 • NO. 1 • 2017