Itsenko-Cushing's disease: be attentive to striae on the body

The problem of timely diagnosis and effective treatment of Itsenko-Cushing's disease is still far from being resolved, but medicine has already achieved some success in this area. Despite the relatively low prevalence of this disease, the problem of hypercortisolism is considered relevant. This is due to certain difficulties in its diagnosis due to insufficient knowledge of general practitioners about this pathology. The disease has a poor prognosis in the absence of proper treatment.

Itsenko-Cushing's disease is a severe neuroendocrine disease accompanied by hyperfunction of the adrenal glands. Among the reasons why the disease manifests are brain injuries, tumors of internal organs; in women, it may begin to manifest itself after childbirth. In this case, hypersecretion of adrenocorticotropic hormone occurs, due to which the subcortical and stem structures of the brain are affected.

A little more than half a century ago, the 5-year survival rate of patients with Itsenko-Cushing's disease did not exceed 50%, and if the level of cortisol in the blood was normalized, it increased to 86%. The problem is greatly exacerbated by the fact that Itsenko-Cushing's disease may remain undiagnosed for a long time. The median time from onset of symptoms to diagnosis is 6 years. As a rule, the disease is detected after a change in the attending physician or during hospitalization for complications, and only a third of cases are diagnosed at the primary level. The reasons for this are both the low alertness of general practitioners and the insufficient level of knowledge about this pathology, as well as the partly non-specific picture of the disease and the absence of pathognomonic symptoms.

Among the main signs of the disease, which the doctor must pay attention to, are the following:

• Obesity: fat is deposited on the shoulders, abdomen, face, breasts and back. Despite the fat body, the arms and legs of patients are thin. The face becomes moon-shaped, round, the cheeks are red.
• Rose-purple or purplish streaks (stretch marks) on the skin.
• Excessive growth of body hair (women grow mustaches and beards on their faces).
• In women - menstrual disorders and infertility, in men - a decrease in sexual desire and potency.

In the treatment of Itsenko-Cushing's disease, special hopes are placed on new pharmacological agents, and the first drug approved for medical therapy was the somatostatin analogue pasireotide (Signifor).

Clinical case of successful medical treatment of Itsenko-Cushing's disease

Patient V., aged 39, was admitted with the following complaints:

• presence of stretch marks (stretch marks) on the abdomen, arms and legs;
• hyperemia and vascular network on the face;
• vision impairment, lacrimation;
• fluctuations in blood pressure (BP) with palpitations, periodic increase in blood pressure up to 170-180 / 100-110 mm Hg. st.;
• frequent headaches;
• gradual weight loss against the background of excess body weight (from 106 to 95 kg);
• height reduction by 6 cm;
• tendency to bruise with minimal trauma.

Anamnesis. The first symptoms were noted in 2010 after suffering severe psycho-emotional stress, when signs of a depressive state, stretch marks on the abdomen, arms, legs, and increased blood pressure appeared. In January 2011, he underwent an examination and received treatment at the Lviv Regional Phthisiopulmonological Center for tuberculosis. From 2010 to 2012, he was examined and treated at the Lviv Regional Cardiology Center for arterial hypertension.

Diagnosis

Laboratory data for 2012:

• blood cortisol level - 630.47 ng / ml (at a rate of 50-250);
• ACTH - 127 pg/ml (norm 7-69);
• HbA1c - 7.3%.

Laboratory data for 2013 are as follows:

• blood cortisol level - 1009 ng / ml (at a rate of 170-720);
• ACTH - 117 pg/ml (norm 7-69);
• HbA1c - 11.6%.
• Computed tomography of the abdominal cavity and retroperitoneal space: the adrenal glands are evenly enlarged in size, the contours are clear, even; the structure is not changed, does not contain calcifications;
• lipomatosis of the abdominal cavity, retroperitoneal lymph nodes are not enlarged;
• severe osteoporosis, multiple consolidated rib fractures.

Computed tomography of the head: the size of the sella turcica is not changed, no visible volumetric formations in the projection of the pituitary gland were detected, the height of the pituitary gland is up to 8 mm, the width is 11.5 mm, the anterior-posterior dimension is 5.6 mm, the pituitary tissue is homogeneous, destructive changes in the back no Turkish saddle found.

MRI of the pituitary gland: the pituitary gland has a normal shape and signal intensity; the Turkish saddle has not been changed; optic chiasm without features; cavernous sinus and visible part of the internal carotid artery without features.

Treatment performed:

• has been on bromocriptine 0.01 g/day since 2012;
• diabetes therapy – metformin 1.7 to 2 g/day, since 2013 – insulin therapy in combination with metformin 2 g/day;
• treatment of osteoporosis - combination of calcium with vitamin D3;
• Hypertension therapy - ACE inhibitors.

At the time of the appeal, the following indicators were noted:

• daily urine cortisol level (at a rate of 28.5-213.7 mcg / 24 h) - 8765.0 mcg / 24 h;
• blood cortisol level (at a rate of 50-250 ng / ml) - 639.52 ng / ml;
• HbA1c - 9.1%;
• BP - up to 180/110 mm Hg. st.;
• body weight - 92 kg.

The patient was prescribed the drug Signifor at a dose of 600 mg 2 r / day. After 2 months of therapy, the following changes were noted:

• daily urine cortisol level (at a rate of 28.5-213.7 mcg / 24 h) - 1155.6 mcg / 24 h;
• blood cortisol level (at a rate of 50-250 ng / ml) - 259 ng / ml;
• HbA1c - 8.5%;
• blood pressure - not higher than 145/95 mm Hg. st.;
• body weight - 86 kg.

Thus, Signifor provided not only a pronounced decrease in the level of cortisol in the blood and urine, but also a significant decrease in the severity of symptoms of the disease already in the first 2 months of therapy.

According to the portal "Health of Ukraine"

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