Review and comments on two recent papers on Cushing’s disease

From Lewis S. Blevins Jr. M.D. –   This interesting manuscript – BIOCHEMICAL CONTROL DURING LONG-TERM FOLLOW-UP OF 230 ADULT PATIENTS WITH CUSHING DISEASE: A MULTICENTER RETROSPECTIVE STUDY – reports the outcomes data of management of a large multicenter cohort of patients with Cushing’s disease.   It represents a “real world“ experience and reflects the expected outcomes of patients managed in both tertiary and mid-level pituitary centers.  I was surprised at the low initial remission rate. I expected it to be higher as most experienced centers report initial remission rates in the 80 to 90% range for all patients.  Regardless, the study illustrates the need for additional management of more than half of patients who undergo surgery to treat Cushing’s disease. The take-home message is that we need various targeted medical therapies to manage hypercortisolism In Cushing’s patients who have failed to enter remission following surgery, have recurrent disease, and have a delayed or incomplete response to radiotherapy.  Adrenal biosynthesis inhibitors and the glucocorticoid receptor blocker Korlym (mifepristone) are the workhorses in this arena.

The paper’s abstract (see below) summarizes the studies objectives and outcomes or you can click here to read more.

Abstract: Cushing disease (CD) results from excessive exposure to glucocorticoids caused by an adrenocorticotropic hormone-secreting pituitary tumor. Inadequately treated CD is associated with significant morbidity and elevated mortality. Multicenter data on CD patients treated in routine clinical practice are needed to assess treatment outcomes in this rare disorder. The study purpose was to describe the burden of illness and treatment outcomes for CD patients.   


This next paper is a review of guidelines from the American Association of Clinical Endocrinologists (AACE) guidelines for recurrence in Cushing’s Disease 2016.

This review article highlights the complexities in the postoperative evaluation of patients with Cushing’s disease and the determination as to whether a patient has recurrent or persistent disease after surgery. The authors’ recommendations do not, in my opinion, represent anything new or groundbreaking. They highlight that the earliest sign of disease, reflecting an alteration in the diurnal secretion of cortisol, is an elevated late-night salivary cortisol level.  I believe that a combination of tests represents the best approach to aid in clinical decision-making regarding whether a patient has persistent or recurrent hypercortisolism. My approach is to conduct a salary cortisol profile, evaluate the 24-hour urine cortisol, and to perform a 1 mg dexamethasone suppression test.

Abstract Recurrence of hypercortisolemia after initial treatment of Cushing’s disease (CD) is more common than previously thought, with a third of patients suffering a recurrence over their lifetime. Awareness of this high rate and delayed timeline (sometimes decades) of potential recurrence is critical and patients with CD should be monitored at regular intervals throughout their lives  In this manuscript, we review the complex evaluation needed for defining CD remission versus persistent disease after surgery, and focus on challenges in diagnosing early recurrent hypercortisolemia. Late night salivary cortisol appears to be an earlier predictor of recurrence when compared with urinary free cortisol excretion. We also review the criteria suggested to define recurrence of hypercortisolemia in patients treated with medical therapy. Further research is needed to determine the optimal way to evaluate a patient with Cushing’s disease recurrence as well as the risk-benefit ratio of treatment in early, mild recurrent disease. You can read more here

Note from the Editors:  Please contact us if you’d like to review the complete papers.



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