Addison's disease (synonym bronze disease) is a clinical syndrome caused by chronic insufficiency of the adrenal cortex. The most common causes of adrenal insufficiency are primary adrenal glands, tumors of the adrenal cortex, treatment with cytostatic agents (myelosan, mustard gas derivatives). The pathogenesis of symptoms in Addison's disease is based on a decrease in the production of cortisone and aldosterone. The lack of the first is associated with dysfunction gastrointestinal tract, cardiovascular disorders, low level fasting blood sugar; with a deficiency of the second - disorders of salt metabolism, dehydration, .
The development of the disease is gradual. At first there is weakness, fast fatiguability, weight loss. Symptoms of dysfunction then appear digestive tract: , complete absence, changing , from time to time severe pain in a stomach. Blood pressure is significantly reduced. Developing. The most striking symptom of the disease is a bronze coloration of the skin, depending on the accumulation of pigment in it (Fig. 1). It appears mostly in places not covered by clothing (face, neck, hands). Places that are naturally pigmented (nipples, anal folds) become more colored. also spreads to the mucous membranes (soft tissue, buccal mucosa).
Characteristic of Addison's disease low content sodium and chlorides in the blood, as well as fasting sugar and elevated levels. The course of the disease is chronic. Complications - sudden or gradual attacks of adrenal insufficiency, sometimes leading to coma (see). significantly improved due to the use of modern and hormonal drugs.
Treatment. Antituberculosis (,) and hormonal therapy (, deoxycorticosterone acetate intramuscularly, cortin subcutaneously or intramuscularly).
Treatment of adrenal coma - see Coma. The diet of a patient with Addison's disease should be varied, food should contain a large number of sodium chloride and a small amount of potassium salts; vitamins are prescribed in large doses, especially (0.4-0.6 g per day). Maximum rest is recommended; work associated with significant neuropsychic and physical stress is contraindicated. Hospitalization is indicated for the initial detection of Addison's disease, with severe forms diseases with a tendency to crises.
Addison's disease(synonyms: hypocorticalism, bronze disease, hypocorticism) - chronic failure adrenal cortex.
Addison's disease occurs as a result of a primary decrease in adrenal cortical function or dysregulation of the adrenal cortex, which ultimately leads to loss of adrenal function. Named after the English dermatologist and pathologist Thomas Addison (1793-1860), who described this disease in 1855 and associated the occurrence of the disease with damage to the adrenal glands. It is observed equally often in men and women, usually between the ages of 30 and 50 years, less often in children and the elderly.
Etiology. The disease most often causes tuberculosis (in 80% of cases) and diencephalo-pituitary damage to the adrenal glands. Much less commonly, the causes of the disease are adrenal toxicosis due to infectious diseases, amyloidosis, adrenal tumors or metastases of tumors to the adrenal glands, syphilis, hemochromatosis, scleroderma.
Adrenal tuberculosis usually occurs as a result of hematogenous metastasis. For clinical signs of Addison's disease to occur, at least 90% of the substance of the adrenal cortex must be destroyed, so the onset of the disease is difficult to recognize.
Pathogenesis. The disease is based on a sharp decrease in the production of hormones from the adrenal cortex. The production of corticosteroids decreases. A lack of mineralocorticoids causes an imbalance of electrolytes (increased sodium and chlorine excretion in the urine, a decrease in their content in the blood: a decrease in potassium excretion in the urine, an increase in it in the blood), dehydration, and hypotension. Impaired production of glycocorticosteroids leads to a drop in blood sugar, increased sensitivity to insulin, a tendency to spontaneous hypoglycemia, an increase in the number of lymphocytes and eosinophils in the blood and maintenance of their number in the blood after the administration of ACTH.
The urinary excretion of 17-ketosteroids and 17-hydroxysteroids also decreases. The production of ACTH is disrupted - the number of basophilic cells in the anterior lobe of the pituitary gland decreases.
Pathological anatomy . There is an increase in melanin in the Malpighian layer of the skin, atrophy of fat, muscles, myocardium, tubules, gastric ulceration, bilateral atrophy of the adrenal cortex. The number of basophilic cells of the anterior pituitary gland decreases.
Complaints. Common complaints about extreme fatigue and weakness, bad feeling. Fatigue begins in the morning and intensifies in the evening. Often the patient's attention is attracted by darkening of the skin, which is often regarded as a result of sun exposure. Significant weight loss is combined with lack of appetite, while with thyrotoxicosis and diabetes mellitus When you lose weight, your appetite remains the same or even increases.
Signs. Melasma consists of brown skin in various shades: golden brown, dirty brown, earthy, lemon yellow. Pigmentation is most pronounced in areas of natural pigmentation that are exposed to sunlight or intense friction. The finger folds, palmar grooves darken, backside hands and feet; on the gums, lips, buccal mucosa, in the area anus and the external genitalia, pigmentation acquires a smoky blue color in the form of spots. The hair of patients often darkens.
Melasma is not necessary in Addison's disease.
Patients lose weight up to 10-15 kg, which is not so much due to
tuberculosis (in the case of a specific origin of the disease), as well as with dehydration, destruction of muscle tissue as a result of impaired metabolism of creatine and creatinine, lack of appetite and indigestion.
The size of the heart decreases, which is often visible on fluoroscopy. The reserve power of the heart decreases. Even after minor physical exertion, shortness of breath and tachycardia occur, although severe heart failure rarely occurs due to adynamia. X-ray examination may occasionally reveal calcification of the adrenal glands.
The electrocardiogram reveals a decrease in voltage due to diffuse damage myocardium, PQ and ST distances lengthen, and a decrease in the ST interval is noted.
T waves thicken or become negative in all leads. Atrial or ventricular extrasystole may be observed.
The disease is characterized by hypotension. Systolic pressure from 100 to 80 mmHg. Art., and diastolic 70-40 mm Hg. Art.
If the patient has previously been ill hypertension, blood pressure appears normal or slightly elevated, but subsequently it gradually decreases. With emotional arousal, hypotension can give way to hypertension. Orthostatic hypotension is observed. In this case, dizziness, tachycardia, Blunt pain in the heart area and fainting.
Due to the fact that adrenal tuberculosis is not isolated, it is necessary to look for the primary focus of tuberculosis. Stomach ulcers are often found and duodenum. In addition to anorexia, flatulence, diarrhea, achylia, and pain along the intestines are noted.
Patients are sensitive to cold and painful irritations, their resistance to infections and various adverse effects decreases. Body temperature is often reduced. Sexual function most often decreases.
An early sign of the disease is severe asthenia, which is often preceded. Patients become apathetic, forgetful, unable to concentrate their thoughts and act. The rhythm of sleep is disturbed, and sometimes a state of psychosis with disorientation and hallucinations develops.
Flow. Addison's disease is a chronic disease that begins gradually. Often years pass with relatively the same course of the disease without noticeable deterioration or with slight deterioration.
It is noted that the disease is easier when part of the adrenal cortex is preserved. Addison's disease is more severe in tuberculosis and adrenal tumors.
Dividing the disease into melasma, astheno-adynamic, hypotonic, gastrointestinal, cardiovascular types the predominance of symptoms is very arbitrary, since with Addison’s disease an isolated lesion of any system is not observed.
It is more expedient to subdivide the disease according to the general condition of the patient and the severity of symptoms into mild forms, moderate severity and heavy, not separated from each other by clear boundaries.
At mild form illness to achieve wellness enough dietary changes (adding table salt And ascorbic acid and potassium restrictions).
In case of illness medium shape severity, which occurs most often, it is necessary to use cortisone, hydrocortisone, prednisone, prednisolone, and in severe forms it is necessary to constantly maintain the patient’s satisfactory condition with these hormones and deoxycorticosterone acetate. With this form, the course of the disease becomes more complicated.
Laboratory data. Hyponatremia, hypochloremia, and hyperkalemia are noted. The excretion of sodium and chlorine in the urine is increased, and potassium is decreased. The body of patients with Addison's disease loses the ability to quickly excrete the injected fluid (Robinson-Power-Kepler test).
Blood sugar is reduced due to decreased gluconeogenesis. The glycemic curve is flat. As the disease worsens, the sugar curve becomes more and more flat, and even if it seems normal, it returns to normal not after 2, but only after 3-4 hours.
Increased sensitivity to insulin is noted, but insulin loading is not recommended due to the risk of severe hypoglycemia. The basal metabolic rate is reduced by no more than 20%.
Creatinuria in the disease reflects destruction muscle fibers. Blood creatine is increased. The amount of blood albumin decreases, and the albumin-globulin ratio decreases accordingly. The number of red blood cells (up to 3 million) and hemoglobin decreases. Due to hyperplasia lymphoid tissue relative lymphocytosis is observed. ROE is slowed down. The absolute number of eosinophils increases, which does not decrease with the administration of ACTH (Thorn test). In a healthy person, the administration of ACTH causes an increase in creatinine, but this is not observed in Addison's disease.
The amount of 11-hydroxysteroids decreases. The method using formaldehydogens for the disease gives 30 11-hydroxysteroids per day (norm 300-800 y), and the use of reducing techniques gives 120 y (norm 240 y).
If the patient secretes corticoids normally, then their amount does not increase under the influence of ACTH.
The release of aldosterone and 17-ketosteroids decreases. If the release of steroids increases after the administration of ACTH, then adrenal insufficiency is associated with the anterior lobe of the pituitary gland, but if the release does not change after the administration of ACTH, then we are talking about primary insufficiency of the adrenal cortex.
Getting worse renal function. Demotion blood pressure up to 50 mm Hg Art. accompanied by anuria, and before this the clearance of creatine, urea and inulin decreases.
Diagnosis and differential diagnosis. The diagnosis of Addison's disease is made on the basis of characteristic clinical signs - adynamia, melasma, hypotension, hypoglycemia, weight loss, digestive tract disorders and laboratory data. In order to cause hidden melasma, mustard plasters are placed on the skin. The disease is distinguished from thyrotoxicosis, in which there may be adynamia, weight loss, and pigmentation. However, with Addison's disease, weight loss is accompanied by a decrease in appetite, and with thyrotoxicosis, appetite is increased.
Unlike hypothyroidism, in which there is also weakness, a decrease in basal metabolism and the release of 17-ketosteroids, a flat sugar curve, with Addison's disease there is no mucous edema, dry skin, the Ketler test is positive, and there is melasma.
Uterine spots of pregnancy, in contrast to pigmentation in Addison's disease, are located on the forehead, around the mouth and eyes, and not on mucous membranes and in places subject to mechanical irritation.
Addison's disease is differentiated from a number of conditions and diseases accompanied by pigmentation of the skin and mucous membranes. With racial and national pigmentation, there are no other signs of Addison's disease, uneven melasma, or bluish pigment spots on the oral mucosa.
Sunburn with fatigue and hypotension is recognized based on history and laboratory data.
In malignant melanoma, the pigmentation, unlike Addison's disease, is heterogeneous, located in the form of “black freckles,” and melanin is found in the urine.
Riel melanosis, which manifests itself in confluent spots on the skin of the face, neck, chest, and back, differs from Addison's disease by the absence of adynamia, hypotension, and hypoglycemia.
For scleroderma, tumors abdominal cavity, malaria, chronic renal failure, melasma is also observed. In these diseases, a functional study of the adrenal cortex allows one to reject the diagnosis of Addison's disease.
With arsenic poisoning, gums appear dark spots, combined with hyperkeratosis of the palms and soles; similar spots on the gums are observed in case of poisoning with lead, bismuth, mercury, and silver nitrate.
With pellagra, darkening of the feet, hands and parts of the limbs adjacent to them is observed. Diarrhea and dementia argue against Addison's disease.
Pigmentation can also be caused by repeated X-ray irradiation of the skin; pigmentary cirrhosis liver, in which the liver enlarges and its function is impaired; pernicious anemia, in which the composition of red blood changes and the color index increases.
Hemochromatosis differs from Addison's disease by the accumulation of iron-containing pigments hemosiderin and hemofuscin in the skin. The diagnosis of hemochromatosis is often based on the presence of ascites and “bronze” diabetes in patients.
The development of adynamia, weight loss, drop in blood pressure without pigmentation can also be observed with sharp decline nutrition as a result chronic infections extra-adrenal localization (for example, with renal tuberculosis).
With neurasthenia, increased irritability, rapid mental and physical fatigue and hypotension are noted, but orthostatic hypotension is not observed, the Ketler test is negative, and 17-ketosteroids remain normal.
With myasthenia gravis, which also occurs with increased fatigue, adrenal function tests are negative.
In salt-losing nephritis, mineralocorticoids lose their ability to influence the tubular epithelium of the kidneys, and as a result, the body loses a lot of sodium and chlorine. The administration of DOX in this case does not bring relief, which occurs only after taking large quantities of table salt.
Treatment. Bed rest. A diet low in potassium is prescribed (no peas, canned food, potatoes, dry fruits, meat, caviar). Give 10 g of table salt per day, or in the form of Addison's elixir with 5 g of sodium citrate in a liter of water with fruit juice.
If swelling occurs, the amount of salt should be reduced. DOXA is injected at 5-10 mg into the muscles once a day or in the form of subcutaneous tablets at 25-50 mg (for 2-3 months), so that 0.3 mg is absorbed per day.
Cortisone is given 12.5-25 mg 2-3 times a day, vitamin C - 300-500 mg per day.
For crises, coma and collapse, cortisone is administered 100 mg every 12 hours, DOXA - up to 15 mg, camphor, caffeine, cordiamine, adrenaline. Needs to be transfused hypertonic solutions, give plenty of fluids, administer streptomycin 0.5-1.0 g per day (course 50 g), give ftivazid, PAS even if there is a suspicion of adrenal tuberculosis.
Transplantation of the adrenal glands or their cortex is ineffective.
Addison's disease has another name - bronze disease. This means a violation of the functioning of the adrenal glands. In turn, this violates hormonal balance, as a result, the synthesis of glucocorticoids decreases or completely disappears.
Addison-Biermer disease has a large number of symptoms, which mainly arise from the involvement of most of the crust. The cause of this disease can be different. In 8 out of 10 cases, Addison-Biermer disease develops due to an autoimmune process in the body.
But sometimes the disease can be accompanied by tuberculosis, which affects the adrenal glands. Pathology can be congenital and inherited. Autoimmune type The disease is most common in the female half of the population.
The most common symptoms of Addison's disease are painful sensations, disturbances in the functioning of the gastrointestinal tract and hypotension. Pathology can lead to metabolic disorders. This disease can also be treated with the help of traditional medicine, which will strengthen the functioning of the adrenal glands and also help in the fight against microbes and inflammation.
General characteristics of the disease
Addison's disease, the photo of which clearly shows the affected area, can occur with both primary and secondary failure. As many people know, pathology affects the glands internal secretion, responsible for the production of some of the most important hormones in the human body. These organs have 2 zones:
- crust;
- brain matter.
Each zone is responsible for synthesizing different types hormones. The medulla produces norepinephrine and adrenaline. They are especially necessary for people in stressful situation, these hormones will help use all the body's reserves.
Other hormones are also synthesized in the cortex.
- Corticosterone. It is necessary for the balance of water and salt metabolism in the body, and is also responsible for the regulation of electrolytes in blood cells.
- Deoxycorticosterone. Its synthesis is also required for water-salt metabolism, and it also affects the efficiency and duration of muscle use.
- Cortisol is responsible for the regulation of carbon metabolism, as well as the production of energy resources.
The pituitary gland has a great influence on the adrenal cortex; it is a gland that is located in the brain area. The pituitary gland produces a special hormone that stimulates the adrenal glands.
As mentioned above, Addison-Biermer disease has two types. Primary is the disease itself, when the functioning of the adrenal glands is completely disrupted due to negative factors. Secondary involves a decrease in the amount of ACTH synthesized, which, in turn, impairs the functioning of the endocrine glands. In a situation where the pituitary gland produces an insufficient amount of hormones for a long period, degenerative processes may begin to develop in the adrenal cortex.
Causes of the disease
The primary form of Addison-Biermer disease is quite rare. It can be found with equal probability in both men and women. In most cases, the diagnosis is made to people aged between 30 and 50.
There is also chronic form diseases. Such development of pathology is possible due to various negative processes. In almost all cases, namely in 80%, the cause of Addison-Biermer disease is an autoimmune condition of the body. In 1 out of 10 cases, the cause of the pathology is damage to the adrenal cortex by an infectious disease, for example, tuberculosis.
For the remaining 10% of patients, the causes may vary:
- this may be affected long-term use medicines, in particular, glucocorticoids;
- types of fungal infection;
- injury to the endocrine glands;
- amyloidosis;
- tumors of both benign and malignant nature;
- bacterial infections in weakened immune system person;
- pituitary dysfunction;
- genetic predisposition to the disease.
Addison's disease can also cause other syndromes, such as adrenal crisis, which occurs when the concentration of adrenal hormones is too low.
There are the most likely causes of the crisis:
- severe stress condition;
- violations in dosage when drawing up a course of treatment with hormonal drugs;
- infection of the adrenal cortex can aggravate the disease;
- adrenal gland injury;
- circulatory problems, such as blood clots.
Symptoms of the disease
Symptoms of Addison's disease directly depend on the disruption of the synthesis of certain types of hormones. Clinical manifestations of the disease may vary. The determining factors are the form of the pathology and its duration.
The most common clinical manifestations of the pathology are as follows:
- Addison's pathology has its name bronze disease for a reason. Most a clear sign This disease is a pigmentation disorder. The skin changes its color. The shade of the mucous membranes changes. It's all about too much pigmentation. With a lack of adrenal hormones, significantly more ACTH is produced, this is explained by the need to stimulate the functioning of the endocrine glands.
- One of the common clinical manifestations disease is chronic hypotension. This may lead to dizziness and fainting, sensitivity to low temperatures increases.
- If the endocrine glands are not functioning sufficiently, the entire body as a whole weakens. If you have constant fatigue or fatigue, you should consult a medical specialist.
- With this pathology, disturbances in the functioning of the gastrointestinal tract often occur; this can manifest itself in the form of vomiting, constant nausea and diarrhea.
- The disease can affect the emotional component. Depression is one of the clinical manifestations of Addison's disease.
- Patients noted increased sensitivity to irritants. The sense of smell and hearing are enhanced, and a person feels the taste of food better. In most cases, patients prefer to eat salty foods.
- Painful sensations in muscle tissue can also be a symptom of Addison's pathology. This is explained by an increase in potassium concentration in blood vessels.
- As mentioned above, one of the clinical manifestations of the disease is adrenal crisis, which occurs as a result of a sharp decrease in the level of hormones of the endocrine glands. The most popular symptoms of a crisis are pain in the abdomen, low arterial pressure, disturbed salt balance.
Diagnosis of the disease
First of all, patients pay attention to changes in the shade of the skin. This phenomenon signals insufficient activity of adrenal hormones. When contacting a medical specialist in this situation, he determines the ability of the adrenal glands to increase the synthesis of hormones.
Diagnosis of Addison's disease occurs by administering ACTH and measuring the cortisol content in the blood vessels before administration of the drug and 30 minutes after vaccination. If a potential patient does not have problems with adrenal function, cortisol levels will increase. If the concentration of the test substance has not changed, the person has disturbances in the functioning of the endocrine glands. In some cases, for more accurate diagnosis, measure the hormone content in urea.
Treatment of pathology
During the course of treatment, special attention should be paid to diet. It must be varied, it must contain the necessary amount of proteins, fats and carbohydrates to provide the body. It is especially worth paying attention to vitamins B and C. They can be found in bran, wheat, fruits and vegetables. In addition, the patient is recommended to drink more decoctions based on rose hips or black currants.
With Addison's disease, the sodium content in the body decreases, for this reason it is recommended to focus on salty foods. In addition, the pathology is characterized by an increased concentration of potassium in the blood vessels; it is recommended not to include foods that are rich in potassium in the diet. These include potatoes and nuts. Patients are advised to eat as often as possible. Before going to bed, medical experts recommend eating dinner to reduce the chance of hypoglycemia in the morning.
Almost all folk recipes are aimed at stimulating the adrenal cortex. ethnoscience has a mild effect, there are practically no side effects. Application folk recipes will not only improve the functioning of the adrenal glands, but will also have a positive effect on the condition of the entire body as a whole. Using this approach, you can normalize the functioning of the gastrointestinal tract and counteract inflammatory processes of a chronic nature. It is recommended to use several recipes in turn, this will avoid the body becoming addicted.
Prevention and prognosis
If therapy was started in a timely manner and all recommendations of a medical specialist were followed, the outcome of the disease will be favorable. The disease will not affect life expectancy in any way. In some cases, Addison's disease is accompanied by a complication - adrenal crisis. In such a situation, you should immediately seek advice from a medical specialist. A crisis can be fatal. Addison's disease is accompanied by fatigue, weight loss and loss of appetite.
Changes in the shade of the skin do not occur in all cases; deterioration in the functioning of the endocrine glands occurs gradually, so it is difficult for a person to detect this on their own. In such a situation, a critical condition develops abruptly and unexpectedly for the patient. Most often, the reason is some negative factor, such as stress, infection or injury.
Since Addison's disease is often autoimmune in nature, there are practically no preventive measures. You should monitor your immune system, avoid drinking alcoholic beverages and smoking. Medical specialists It is recommended to pay attention in time to the manifestations of infectious diseases, especially tuberculosis.
The disease, named after Addison, who described it in 1855, is based on atrophic and destructive processes in the adrenal glands. Other names for Addison's disease: hypocortisolism, Addison's disease, bronze disease, chronic adrenal insufficiency, Addison's disease .
Addison's disease: symptoms
Addison's disease usually begins to appear between the ages of 20 and 40, but is also observed in children and even the elderly.
In men Addison's disease occurs more often than in women. The disease can develop both in persons who were previously completely healthy (sometimes without any previous diseases, sometimes after suffering from influenza or another infection), and in persons with a history of one or another endocrine disease.
In severe cases, the symptomatology of Addison's disease is very characteristic: muscle weakness (adynamia), mental lethargy, fatigue, decreased ability to work, gastrointestinal disorders, decreased blood pressure (hypotension) and a smoky-bronze coloration of the skin (melasma) and mucous membranes appear. shells. Sometimes the symptoms of the disease resemble, but with careful collection of anamnesis it is possible to identify distinctive features, allowing to completely exclude the diagnosis of hypothyroidism.
Usually, a dark color appears on the face, extensor surfaces of the hands, in closed areas exposed to friction by clothing (belt), genitals, perineum, on the mucous membranes of the mouth, tongue, gums, etc. Sometimes the entire skin takes on a smoky bronze color. The sudden weight loss is also noteworthy. The weakness is so great that patients usually most forced to lie in bed for days.
Signs from the gastrointestinal tract are varied: nausea, vomiting, loss of appetite, a feeling of heaviness in the epigastric region, diarrhea, attacks of pain in the intestines, decreased secretory activity of the stomach.
The predominance of one or another symptom has made it possible to identify special types of Addison's disease:
1) melasma;
2) adynamic;
3) hypotonic;
4) gastrointestinal.
The initial symptoms of Addison's disease in most cases are adynamia and general asthenia. And according to most authors, the most basic symptom of Addison's disease is asthenia.
In the blood, a low hemoglobin content and a decrease in the number of red blood cells are usually noted, and in white blood - relative lymphocytosis. The lymphatic apparatus in many cases turned out to be hyperplastic. ESR is usually sharply slowed down,
From the metabolic side, what deserves attention, first of all, is a decrease in blood glucose (not always observed). The glycemic curve is sluggish. The limit of carbohydrate digestibility is very high. When adrenaline is administered, it does not appear. The percentage of sodium and chlorine in the blood is usually reduced, potassium is increased (hyponatremia and hypochloremia with simultaneous hyperkalemia). In most cases, there is a decrease in cholesterol levels. Amount in urine sodium chloride usually elevated (in severe cases 2-3 times compared to the norm).
The urinary excretion of 17-ketosteroids in severe cases of Addison's disease caused by caseous decomposition of the adrenal glands is sharply reduced. In milder forms of the disease, the decrease in their secretion is not so dramatic.
From the central side nervous system Noteworthy are extreme irritability, rapid nervous fatigue and short temper. Patients become very suspicious and are always aware of their serious condition. Unsatisfactory sleep is often noted.
The course of the disease can be twofold: the disease begins either very imperceptibly, gradually, flows for a long time (in this case, the manifestations either weaken or intensify) or acutely. In the latter case, all signs are clearly identified, and the patient, with symptoms of increasing adynamia, melasma, hypotension and gastrointestinal disorders, dies within a few months or even weeks if the correct treatment. In chronic cases, the disease usually lasts 2-3 years and only occasionally up to 10 years. The introduction of cortin, deoxycorticosterone, cortisone, etc. into therapy significantly prolongs the life of these patients.
There are also (though extremely rare) so-called fulminant cases, when patients die within a few days.
Addison's disease: diagnosis
The diagnosis of Addison's disease in severe cases is not difficult, but in the initial stages it is extremely difficult. Usually one has to take into account, first of all, a triad of symptoms: adynamia, hypotension and melasma, which, as a rule, run in parallel from the very beginning.
Melasma, which is one of the important symptoms Addison's disease also occurs in a number of diseases and conditions:
1) chronic malaria,
2) malignant tumors organs of the abdominal cavity,
3) malignant tumors of the female genital organs,
4) hemochromatosis,
5) pellagra,
6) racial pigmentation of the integument,
7) cirrhosis of the liver,
8) arsenic poisoning,
9) some itchy dermatoses,
10) argyria,
11) scleroderma,
12) Graves' disease,
13) the so-called “tramp skin”,
14) akanthosis nugricans.
We should not forget that sometimes pronounced melasma occurs, which is extremely reminiscent of melasma in Addison's disease, but there is no pigmentation of the mucous membranes, no adynamia, hypotension, disorders of the gastrointestinal tract, or hypoglycemia. This is the so-called false form of Addison's disease. Its genesis is not clear enough.
If you suspect Addison's disease, the endocrinologist, in addition to collecting anamnesis, should prescribe the following studies to the patient:
ACTH stimulation. This test measures cortisol levels before and after administration of the synthetic hormone ACTH. If the patient has adrenal insufficiency, the reaction is absent or mild.
Blood analysis. Based on the level of potassium, sodium, ACTH and cortisol in the patient’s blood, the diagnosis of Addison’s disease can be confirmed/excluded, and the determination of antibodies in the blood can indicate the autoimmune nature of the disease.
Test with insulin hypoglycemia. This test is performed for diagnostic purposes secondary form adrenal insufficiency (Addison's disease), which is caused by diseases of the pituitary gland. The test consists of measuring cortisol and glucose levels at different time intervals after insulin administration. In the absence of pathology, the level of cortisol increases, and glucose, on the contrary, decreases.
Visual tests. An endocrinologist may prescribe a CT (computed tomography) scan of the abdominal cavity for diagnostic purposes possible pathologies and assessment of adrenal gland size. If secondary adrenal insufficiency is suspected, an MRI (magnetic resonance imaging) of the pituitary gland is performed.
In doubtful cases of Addison's disease (in the absence of clearly severe symptoms To clarify the diagnosis) use a water sample (Robinson-Power-Kepler test). This test is based on a delay in the release of water in patients with Addison's disease after a water load and an increase in the release of chlorides with a relative retention of urea.
It is also recommended for the same purpose to determine the so-called uric acid-creatinine index, which is the ratio of the amount excreted with water uric acid to the excreted creatinine. This indicator is healthy people does not exceed 0.26 (average 0.17). A larger index indicates pathological condition body (tumors of the adrenal cortex).
However, it must be remembered that both of these tests (for decreased and increased adrenal function) cannot be decisive for the diagnosis of Addison's disease.
Addison's disease occurs when the adrenal cortex malfunctions. This disease is diagnosed in both men and women, mainly of middle age (from 30 to 40 years). This is enough rare disease, which is registered in one person per 100 thousand population. Addison Birmer's disease can develop as a result of infectious diseases (syphilis, adrenal tuberculosis and typhus), as well as amyloidosis and malignant tumors. In 70% of patients, the disease occurs due to disruptions in the immune system (antibodies begin to mistake body cells for foreign bodies, attack and destroy them). When adrenal cortex cells are destroyed, the synthesis of gluco- and mineralocorticoids (cortisol, 11-deoxycorticosterone, aldosterone) decreases. Scientists have proven that glucocorticoids regulate various biochemical processes in the body (regulates blood pressure, insulin concentration, participates in the regulation of the synthesis of proteins, lipids and carbohydrates).
Hypocortisolism is often observed in patients taking adrenal hormones. In most cases, the etiology of the disease remains unclear. classified into primary and secondary. Primary failure develops when the adrenal tissue is damaged, and the secondary one is caused by insufficient stimulation of them with adrenocorticotropic hormone.
Addison's disease: symptoms
Clinical signs diseases are caused by a lack of mineral and glucocorticoids in the body. Most characteristic features are adynamia, hypotension, dysfunction of the gastrointestinal tract (nausea, loss of appetite, vomiting, constipation, which is often replaced by diarrhea). Some patients experience hypochlorhydria.
Addison's disease is accompanied by increased irritability or depressive state, frequent headaches and insomnia. X-ray examination chest organs shows a decrease in the size of the heart. The electrocardiogram registers (increased concentration of potassium in the blood). Skin pigmentation does not reflect the severity of the disease, but its increase or decrease during treatment indicates effectiveness therapeutic actions.
Addison's disease of secondary origin is characterized by less severe symptoms. Very evident without skin pigmentation (so-called “Addison’s white”). The main cause of this disease is adrenal tuberculosis. Without appropriate treatment, Addison's disease becomes more complicated. Clinical signs intensify: nausea, vomiting, diarrhea, blood pressure drops sharply, and the concentration of potassium in the blood increases. In the peripheral blood, the amount of residual nitrogen, the number of red blood cells, and the hemoglobin content increase. Therefore, if timely assistance is not provided to the patient during the Addisonian crisis, he will die with signs of renal and
The most significant laboratory indicators to make a diagnosis is the content of 17-OX in the blood plasma and 17-KS in the urine, as well as a decrease in blood glucose levels. Morphological examination of the blood indicates lymphocytosis, eosinophilia and slow ESR.
Addison's disease is differentiated from the following diseases: bronze diabetes, pellagra, poisoning with arsene, bismuth and argentum. This disease usually has chronic course with periodic exacerbation. The severity of Addison Birmer's disease is determined by the severity of the main symptoms of the disease, as well as by the number of medications that are necessary to compensate for adrenal insufficiency.
Addison's disease (primary or chronic adrenocortical insufficiency) is a gradually developing, usually progressive insufficiency of the adrenal cortex. It is characterized by various symptoms which include hypotension, hyperpigmentation, may lead to adrenal crisis with cardiovascular collapse. Diagnosis is based on the detection of elevated plasma ACTH levels and low plasma cortisol levels. Treatment depends on the cause, but generally consists of hydrocortisone and sometimes other hormones.
ICD-10 code
E27.1 Primary adrenal insufficiency
E27.2 Addison's crisis
Epidemiology
Addison's disease develops in 4 people per 100,000 per year. Observed in all age groups, with equal frequency in men and women, is more often clinically manifested during metabolic stress or injury. The onset of severe symptoms (adrenal crisis) may be preceded by acute infection(common cause, especially with septicemia). Other causes include trauma, surgical intervention and Na loss due to increased sweating.
Causes of Addison's disease
About 70% of cases in the United States are associated with idiopathic adrenal atrophy, probably caused by autoimmune processes. Other cases result from destruction of the adrenal glands by granulomas (eg, tuberculosis), tumor, amyloidosis, bleeding, or inflammatory necrosis. Hypoadrenocorticism can also be caused by the administration of drugs that block the synthesis of glucocorticoids (for example, ketoconazole, the anesthetic etomidate). Addison's disease can be combined with diabetes mellitus or hypothyroidism in polyglandular deficiency syndrome.
Pathogenesis
There is a deficiency of mineralocorticoids and glucocorticoids.
Mineralocorticoid deficiency results in increased Na excretion and decreased K excretion, mainly in urine, but also in sweat, saliva, and from the gastrointestinal tract. As a result, low concentrations of Na and high concentration K in plasma. The inability to concentrate urine in combination with electrolyte imbalance leads to severe dehydration, plasma hypertonicity, acidosis, decreased circulating blood volume, hypotension and ultimately circulatory collapse. However, in cases of adrenal insufficiency caused by impaired ACTH production, electrolyte levels often remain within normal limits or are moderately altered.
Glucocorticoid deficiency contributes to hypotension and causes changes in insulin sensitivity and disturbances in carbohydrate, fat and protein metabolism. In the absence of cortisol, essential carbohydrates are synthesized from proteins; as a result, hypoglycemia and a decrease in glycogen reserves in the liver are observed. Weakness develops, partly due to insufficient neuromuscular function. Resistance to infection, injury, and other types of stress also decreases.
Myocardial weakness and dehydration reduce cardiac output, circulatory failure may develop. A decrease in plasma cortisol levels leads to increased production ACTH and an increase in the level of beta-lipotropin in the blood, which has melanocyte-stimulating activity and, together with ACTH, causes hyperpigmentation of the skin and mucous membranes characteristic of Addison's disease. Therefore, secondary adrenal insufficiency, which develops as a result of hypofunction of the pituitary gland, does not cause hyperpigmentation.
Symptoms of Addison's disease
Early symptoms and signs include weakness, fatigue, and orthostatic hypotension. Hyperpigmentation is characterized by diffuse darkening of exposed and, to a lesser extent, covered parts of the body, especially pressure points (bone protrusions), skin folds, scars, and extensor surfaces. Black pigment spots are often observed on the forehead, face, neck and shoulders.
Areas of vitiligo appear, as well as bluish-black discoloration of the nipples, mucous membranes of the lips, mouth, rectum and vagina. Anorexia, nausea, vomiting and diarrhea are common. There may be a decrease in tolerance to cold and a decrease in metabolic processes. Dizziness and syncope are possible. Gradual onset and nonspecific early symptoms often lead to an incorrect diagnosis of neurosis. Later stages of Addison's disease are characterized by weight loss, dehydration, and hypotension.
Adrenal crisis is characterized by profound asthenia; pain in the abdomen, lower back, legs; peripheral vascular insufficiency and, finally, renal failure and azotemia.
Body temperature may be low, although severe fever is common, especially if the crisis is preceded by an acute infection. In a significant number of patients with partial loss of adrenal function (limited adrenocortical reserve), adrenal crisis develops under conditions of physiological stress (eg, surgery, infection, burns, serious illnesses). The only signs may be shock and fever.
Diagnosis of Addison's disease
Based clinical symptoms and signs suggest adrenal insufficiency. Sometimes the diagnosis is assumed only upon detection characteristic changes electrolyte levels, including low Na (5 mEq/L), low HCO 3 (15-20 mEq/L), and high level blood urea.
Research results that suggest Addison's disease
Research
Laboratory studies, starting with plasma cortisol and ACTH levels, confirm adrenal insufficiency. Increased level ACTH (>50 pg/ml) with low cortisol levels [
If ACTH and cortisol levels are within the normal range and clinical observations suggest adrenal insufficiency, especially in patients preparing for surgery, provocative tests should be performed. If time does not permit (eg, emergency surgery), the patient should be given hydrocortisone empirically (eg, 100 mg intravenously or intramuscularly) and challenge tests performed subsequently.
Addison's disease is diagnosed in the absence of an increase in cortisol levels in response to the administration of exogenous ACTH. Secondary adrenal insufficiency is diagnosed using a stimulation test with prolonged ACTH, an insulin tolerance test, and a glucagon test.
The ACTH stimulation test involves the administration of a synthetic analogue of ACTH in a dose of 250 mcg intravenously or intramuscularly. (According to some authors, if secondary adrenal insufficiency is suspected, the study should be done with a low dose of 1 mcg IV instead of 250 mcg, since these patients develop a normal response at higher doses.) Patients taking glucocorticoids or spironolactone should skip the appointment. Normal plasma cortisol levels before injection range from 5 to 25 mcg/dL (138 to 690 mmol/L), doubling within 30 to 90 minutes to reach at least 20 mcg/dL (552 mmol/L). Patients with Addison's disease have low or low-normal levels that do not rise above 20 mcg/dL within 30 minutes. A normal response to a synthetic ACTH analogue can be observed in secondary adrenal insufficiency. However, because pituitary insufficiency can cause adrenal atrophy, it may be necessary to give the patient 1 mg of long-acting ACTH intramuscularly once daily for 3 days before testing if pituitary disease is suspected.
A prolonged ACTH stimulation test is used to diagnose secondary (or tertiary - hypothalamic) adrenal insufficiency. A synthetic analogue of ACTH is administered at a dose of 1 mg intramuscularly, and cortisol levels are determined at intervals over 24 hours. Results within the first hour are similar to those of the short test (determination within the first hour), but in Addison's disease there is no further increase after 60 minutes. In secondary and tertiary adrenal insufficiency, cortisol levels continue to rise for 24 hours or more. Only in cases of prolonged adrenal atrophy is it necessary to administer long-acting ACTH to stimulate the adrenal glands. Usually carried out first short test, if there is a normal response, further research is considered.
Differential diagnosis
Differential diagnosis of Addison's disease
Hyperpigmentation can be observed with bronchogenic carcinoma, salt intoxication heavy metals(for example, iron, silver), chronic dermatoses, hemochromatosis. Peutz-Jeghers syndrome is characterized by pigmentation of the mucous membranes of the cheeks and rectum. A combination of hyperpigmentation and vitiligo is often observed, which may indicate Addison's disease, although it also occurs in other diseases.
The weakness that develops in Addison's disease improves with rest, in contrast to neuropsychiatric weakness, which is worse in the morning compared to physical activity. Most myopathies can be differentiated by their distribution, lack of pigmentation and characteristic laboratory signs.
Patients with adrenal insufficiency develop fasting hypoglycemia due to decreased gluconeogenesis. In contrast, patients with hypoglycemia caused by insulin hypersecretion may experience attacks at any time, often have increased appetite with increased body weight, and also have normal function adrenal glands It is necessary to differentiate low Na levels in patients with Addison's disease from those in patients with heart and liver diseases (especially those taking diuretics), hyponatremia in the syndrome of impaired ADH secretion, and salt-wasting nephritis. These patients are not prone to the appearance of hyperpigmentation, hyperkalemia with increased blood urea levels.
Treatment of Addison's disease
Normally, the maximum excretion of cortisol occurs early in the morning, the minimum at night. Consequently, hydrocortisone (an analogue of cortisol) is prescribed at a dose of 10 mg in the morning, 1/2 of this dose at lunch and the same amount in the evening. Daily dose usually 15-30 mg. Nighttime use should be avoided as it may cause insomnia. Additionally, it is recommended to take 0.1-0.2 mg of fludrocortisone once a day to replace aldosterone. The most the easy way adequacy of dose selection is achieving normal level renina.
Normal hydration and lack orthostatic hypotension indicate adequate replacement therapy. In some patients, fludrocortisone causes hypertension, which is corrected by dose reduction or administration of antihypertensive non-diuretic drugs. Some clinicians prescribe too low doses of fludrocortisone in an attempt to avoid prescribing antihypertensive drugs.
Concomitant illnesses (eg infections) are potentially dangerous and should be treated with care great importance; During illness, the dose of hydrocortisone should be doubled. If nausea and vomiting occur during oral administration of hydrocortisone, a switch to parenteral administration. Patients should be taught when to take supplemental prednisone and how to emergency situations administer hydrocortisone parenterally. The patient should have a filled syringe with 100 mg of hydrocortisone. In case of adrenal crisis, a bracelet or card indicating the diagnosis and dose of glucocorticoids can be helpful. If salt loss is severe, as in hot climates, the dose of fludrocortisone may need to be increased.
With concomitant diabetes mellitus, the dose of hydrocortisone should not exceed 30 mg/day, otherwise the need for insulin increases.
Emergency care for adrenal crisis
Help must be provided urgently.
ATTENTION! In adrenal crisis, delay in glucocorticoid therapy, especially in the presence of hypoglycemia and hypotension, can be fatal.
If the patient is acutely ill, confirmation with an ACTH stimulation test should be delayed until the condition improves.
100 mg of hydrocortisone is injected intravenously over 30 seconds, followed by an infusion of 1 liter of 5% dextrose in 0.9% saline containing 100 mg of hydrocortisone over 2 hours. Additionally, 0.9% is administered intravenously saline before correction of hypotension, dehydration, hyponatremia. During rehydration, serum K levels may decrease, requiring replacement therapy. Hydrocortisone is administered continuously at 10 mg/hour for 24 hours. When high doses of hydrocortisone are administered, mineralocorticoids are not required. If the condition is less acute, hydrocortisone can be administered intramuscularly at 50 or 100 mg. Within 1 hour after administration of the first dose of hydrocortisone, blood pressure should recover and improve general condition. Inotropes may be required before glucocorticoids are effective.
During the second 24-hour period, a total dose of hydrocortisone of 150 mg is usually prescribed if the patient's condition has significantly improved, and on the third day - 75 mg. Maintenance oral doses of hydrocortisone (15-30 mg) and fludrocortisone (0.1 mg) are taken daily thereafter as described above. Recovery depends on treatment of the underlying cause (eg, trauma, infection, metabolic stress) and adequate hormonal therapy.
For patients with partial adrenal function who develop a crisis in the presence of a stress factor, the same hormonal therapy is necessary, but fluid requirements may be much lower.
Treatment of complications of Addison's disease
Sometimes dehydration is accompanied by a fever above 40.6 °C. Antipyretics (eg, aspirin 650 mg) may be administered orally with caution, especially in cases with a drop in blood pressure. Complications of glucocorticoid therapy may include psychotic reactions. If psychotic reactions are observed after the first 12 hours of therapy, the dose of hydrocortisone should be reduced to the minimum level that allows maintaining blood pressure and good cardiovascular function. May be temporarily required antipsychotic drugs, however, their use should not be prolonged.
When treated, Addison's disease does not usually reduce life expectancy.
It is important to know!
Symptoms of ectopic ACTH production syndrome include varying degrees of hypercortisolism. In case of rapid progression tumor process and high production of hormones by the adrenal cortex, a typical Itsenko-Cushing syndrome develops.
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