The list of so-called rare or infrequent diseases includes the Cushing’s syndrome, who owes his namebre a Harvey Cushing, who discovered the disorder in 1932. Cushing syndrome occurs when the body has too much cortisol hormone over time. It can be the result of taking too much oral corticosteroids or the body making too much cortisol.
With a higher prevalence in young women and without a genetic cause in 95% of casesWhen the endogenous increase in this hormone produced by the adrenal gland occurs, the reason may be a generally benign tumor, either in the adrenal gland or in the pituitary gland (in 70/80% of cases).
The Dr. Mirtha Guitelman (MN 74370), specialist in endocrinology and advisor to Varifarma, described that “the vast majority are microadenomas and some are even invisible to magnetic resonance imaging.” And he classified them into three types: «ACTH (due to corticotropin or adrenocorticotropic hormone, produced by the pituitary gland that stimulates the production of cortisol) when it is due to an adenoma in the pituitary, ACTH independent, when the tumor is in the adrenal gland or ACTH ectopic ‘.
«When the adenoma is in the pituitary gland, it is called Cushing’s disease, which is the most frequent, and the way in which it manifests is that already known by the increase in cortisol: full moon face, red face, central obesity, thinning of the skin, red-purplish stretch marks, fat dorsal hump, darkening of the armpits and folds due to high insulin, hair loss and spontaneous bruising, among others “, described the specialist.
He also listed a number of symptoms, including diabetes, hypertension, insulin resistance, psychiatric disorders, thrombosis, secondary amenorrhea in 90% of women and sexual dysfunction in men.
After ensuring that patients arrive at the consultation after a one or two year pilgrimage by gynecologists, dermatologists and even psychiatrists, Guitelman admitted that it is a pathology of difficult diagnosis, with a high mortality, and that disease control does not always reverse the possibility of suffering comorbidities.
Studies that detect Cushing’s syndrome
In that sense, the confirmation of the painting is reached mThrough biochemical measurement of cortisol in saliva, blood and urine, an MRI is added to locate the tumor and determine if it is operable. ANDIn the event that this diagnostic imaging technique does not yield accurate results due to the small size of the adenoma, a petrosal sinus catheterization is usually confirmatory.
Unlike what happens with other pituitary tumors, such as acromegaly, where the treatment can be pharmacological or surgical, in this case the indication is “Yes or yes surgery”, as specified by the expert.
“After surgery, it may happen that the person is cured but is left with insufficient this hormone and needs to take corticosteroid, or that he remains with active disease (in 30% of cases”, he highlighted Guitelman, who added: «Another possibility is that there is recurrence within a year or up to 10 years; in that case, if the tumor is found, it is reoperated or if not, a pharmacological treatment is recommended ».
At this point, “the most classic drug is ketoconazole, an off-label drug, that is, it is not approved for this use, since it is to treat mycosis, but through scientific evidence it is indicated.”
“Now there are new drugs, although not all of them are still in the country,” he pointed out, adding: “Among those that are taken orally, methopyrone acts at the level of the adrenal gland, inhibiting the production of cortisol, while osilodrostat and others drugs with high efficacy proven in Europe have not yet been approved in Argentina ». “Among injection drugs, pasireotide, with two subcutaneous injections per day or one monthly, acts at the tumor level with variable effectiveness,” he added.
“Survival depends on the age of diagnosis and the affectation, especially at the cardiological level, that the person presents,” concluded Guitelman.