Chronic adrenal insufficiency
Endocrinology / / May 24, 2016
adrenocortical insufficiency may occur or acute or chronic form.
Chronic adrenal insufficiency is divided into primary and secondary.Primary adrenal insufficiency is the result of the defeat of the pathological process itself adrenal glands, while the secondary failure depends on a reduced secretion of ACTH by the anterior pituitary disorders due to pituitary or hypothalamic region.
Primary chronic adrenocortical insufficiency was described in 1855 by Thomas Addison English clinician, after whom the disease was named.
disease affects equally both sexes, although some authors believe that women suffer from chronic adrenal insufficiency.The disease occurs mainly in the 3-4th decade of life, but sometimes the age of the patients is younger or older.
etiology. cause of chronic primary adrenal insufficiency is considered mainly tuberculosis and adrenal atrophy them.The statistics of previous years indicate that in most cases the cause of chronic adrenal insufficiency was fibro-case
Over the past decades have seen an increase in the incidence of chronic adrenal insufficiency, which are based on the atrophy of the cortex, the so-called idiopathic atrophy, selective cortical necrosis.Currently, up to 50% of chronic adrenal insufficiency due to primary atrophy of the adrenal cortex.Bilateral tuberculous process in the adrenal glands is now less than 50% of the disease, due to a decrease in the frequency and severity of tuberculosis.However, some clinicians believe that the tuberculous etiology of chronic adrenal insufficiency is still ranked first in frequency, accounting for 61,7-78% of all cases.
Tuberculosis of the adrenal glands is a consequence of hematogenous dissemination of tuberculosis.TB process usually active, progressive and eventually destroys the whole gland.The lack of a history of tuberculosis does not exclude tuberculous nature of adrenal lesions.
atrophic process mainly affects the cortex and medulla rarely captures.This destructive process that destroys the adrenal cortex.There is speculation that the primary atrophy of the adrenal glands caused by some chronic, sluggish flowing inflammation of unknown etiology (infectious and toxic Soffer theory, etc.), Or caused by toxic destructive agent, the nature and character of which is also unknown (Kiefer, Brenner et al.).
Research Anderson and Blizzard revealed the presence of serum antibodies to the adrenal tissue in cases of idiopathic Addison's disease.According to these researchers, primary atrophy of the adrenal cortex is the result of an autoimmune process.
However, we can agree with Miescher, autoserotherapy that is probably a secondary phenomenon, a reaction to the inflammatory process.
third most common cause of chronic adrenal insufficiency is amyloidosis.Amyloid degeneration of the adrenal glands occurs as a manifestation of the general amyloidosis.
Tumors of the adrenal cortex can also be the cause of their chronic disease.Mostly it metastasized breast cancer or metastases of bronchogenic cancer.
observed isolated cases of Addison's disease caused by purulent infection, measles, typhus, leukemic infiltration of the adrenal glands or the pituitary gland, blastomycosis, particularly common in North America, disseminated coccidioidomycosis.
In rare cases, chronic adrenal insufficiency can cause a parasitic infestation.
a result of treatment with cytostatic agents (mielosan derivatives of mustard gas, etc.) Can also be Addison's disease.
Vascular lesions, mainly as thrombosis and hemorrhage in the adrenal tissue or observed infections pregnancy toxemia, result in these glands massive fibrosis and ultimately lead to chronic adrenal insufficiency.
pathogenesis. The pathogenesis of most symptoms of the disease is reduction of production cortex of the adrenal cortisol and aldosterone.data on the biological action of corticosteroids Accumulated now allow attributed to the failure of cortisol gastrointestinal disturbances, weakness, cardiovascular disorders, lowering fasting blood sugar and spontaneous hypoglycemia, decreased tolerance to water stress, neutropenia, eosinophilia, lymphocytosis, and a number of otherdisorders.Since aldosterone deficiency associated violations of salt metabolism, dehydration, hypotension.Weighting of all symptoms during the crisis due to the increase in utilization of glucocorticoid tissue under stress.
pigmentation depends on increased production of pituitary melanocyte-stimulating hormone, or melanophore.Recent research demonstrated that also possesses ACTH melanocyte activity.
pathogenetic significance of damage adrenal medulla ns.
Along with symptoms, the pathogenesis of which is reduction of adrenocortical function, in patients with Addison's disease symptoms may occur related to the pathological process that caused the damage to the adrenal glands.For example, tuberculosis intoxication can cause fever, accelerated erythrocyte sedimentation rate, shifts from the white blood cells, loss of appetite, weakness, decline of power and others.
Fibrous-cheesy tuberculosis affects both adrenal glands, involving in pathological process not onlycortex, brain and its substance.The adrenal glands while increasing in size, reaching a weight of up to 60 at the expense of cheesy masses, infiltrates of epithelioid and giant cells, fibrous tissue.The cheesy masses are often lime deposits.Residues of the adrenal glands are found in the form of foci of cortical tissue, where there are signs of recovery.
In primary adrenal atrophy, decrease in size.Atrophic and degenerative changes are particularly pronounced in the beam and reticular areas of the cortex.The process is characterized by a massive infiltration of the adrenal cortex by lymphocytes and plasma cells, the development of fibrosis.Among cell round infiltration and fibrous tissue hyperplastic islets are hypertrophied cortical cells.
When adrenal amyloidosis large - up to 30-40, the amyloid deposits occur in the cortex and in the medulla, but subcapsular departments usually saved unaffected adrenal tissue sites than, apparently, explains the relatively rare development of chronic diseaseadrenal amyloidosis in general.
Chronic adrenal insufficiency often found changes in the thyroid gland.They are expressed in varying degrees lymphoid infiltration and fibrosis gland tissue.Sometimes lymphoid infiltration expressed very sharply.In such cases, thyroid tissue is almost completely atrophied.Among the epithelial cells of follicles observed degenerative changes and a group of cells.It is accompanied by a significant decrease in thyroid function, up to severe myxedema.Primary myxedema with Addison's disease was first described Falta and Schmidt.Such changes are more common in idiopathic adrenal atrophy.
Anatomical changes in the other endocrine glands in chronic adrenal insufficiency less common.
changes in the kidney are rare.Guttman found in the study of 566 autopsies of chronic adrenal insufficiency, collected literature filed changes in the kidney only 10%.Anatomical changes in the kidneys reduced mainly to dystrophic lesions of tubules in chronic adrenal insufficiency.
Often there is a chronic degeneration of the liver to regenerate areas of nodular type.
Sorkin points to frequent liver disease in chronic adrenal insufficiency.He found nuclei vacuolation of liver cells, fatty change them, leukocyte infiltration in the portal field, atrophy parenchyma.
Heart with Addison's disease often reduced in size.Histological changes in the heart muscle with Addison's disease are characterized by degenerative processes in it - fatty degeneration, the disappearance of the individual muscle fibers of varying degrees karyolysis, ie the disappearance of the nuclei, but no leukocyte infiltration...
Patients with chronic adrenal insufficiency complain mainly the sharp weakness, fatigue, lack of appetite, weight loss;often have nausea, vomiting, constipation, diarrhea exchangeable, sometimes severe abdominal pain.
These complaints are joined pigmentation of the skin and mucous membranes, and hypotension.
Thorn results table of the most common symptoms in patients with chronic adrenal insufficiency: fatigue, easy fatigue sets in - 100% of the cases;Weight Loss - 100% of the cases;pigmentation - 94% of cases;anorexia - 90% of cases;nausea - 84% of cases;vomiting - 81% of cases;abdominal pain - 32%;constipation - 28% of cases;diarrhea - 21% of cases;increased need for soli- 19% of cases;muscle pain - 16% of cases.
Often hypoglycemia observed in a variety of clinical manifestations, hypotension.
The first symptoms are often fatigue and anorexia, although it may at first appear pigmentation or hypotension.
Loss of appetite (anorexia) can be so pronounced that one kind of food causes in patients with nausea and even vomiting.
These complaints are often joined by severe abdominal pain, sometimes of uncertain nature and localization, often concentrated in the lateral parts, in adrenal location.These pains are sometimes accompanied by abdominal wall strain, rigidity of his muscles, nausea, vomiting, prostration, and create a picture of acute peritonitis.If the pain focus mainly on the right half of the abdomen, they can simulate acute appendicitis or cholecystitis.This syndrome "acute abdomen" due, apparently, inflammation around adrenal tissue.
Besides acute pain, sometimes there are constant, aching pain in the epigastric region.
One of permanent chronic adrenal insufficiency symptoms is weight loss, which may be in some patients significant.Significant factors affecting weight loss patients are chronic dehydration and sodium loss, gastrointestinal disturbances, drastically reduce appetite and adversely affect the intestinal absorption, as well as an active tuberculous process.
skin pigmentation and mucous membranes - common symptom may appear long before other symptoms of the disease (5-10 years old) with chronic adrenal insufficiency pigmentation.The intensity of the pigmentation is not associated with the severity of the patient's condition, but its vibrations still to some extent an expression of the deterioration or improvement.
the treatment of chronic insufficiency of the adrenal corticosteroid pigmentation does not disappear completely, but there is a slight bleaching of skin color, which apparently caused by a decrease in blood ACTH and melanocyte stimulating hormone.
pigmentation caused by melanin deposition in the papillary layer of the skin and mucous membranes.It can be quite different - from tan to brown with intense shade of bronze, and therefore the disease is also called "bronze disease."Pigmentation may be diffuse, but usually it is particularly pronounced in the field of friction from clothing, in scars, on the ground of previous skin burns, skin folds, on the lines of palms of the hands, on the extensor surfaces of the fingers, less stop, especially in the interphalangeal joints, on the nipple of the breast.
Figure: Chronic adrenal insufficiency.The patient was 29 years old.Pigment deposition on the face, hands, and particularly in the breast nipple.
pigmentation often happens on the oral mucosa - on the gums, tongue, hard and soft palate, on the inner surface of the cheeks, as well as in the genital area and around the anus.Pigmentation is sometimes uneven and alternates with vitiligo or leucoderma, ie, skin discoloration spots of various sizes -.. From large to very small.In the latter case it appears mottled skin.Occasionally pigmentation is on the nails in the form of small spots, sometimes occupies the entire nail.The literature describes isolated cases of Addison's disease without skin pigmentation.Pigmentation of the mucous membranes are often absent, although its presence is very important diagnostic sign.
Subcutaneous adipose tissue may be underdeveloped, but in some patients who were obese before the onset of the disease, even after weight loss is always occurring in chronic adrenal insufficiency, there remains a significant amount of fat.
more or less marked atrophic changes observed in skeletal muscle.Muscle strength is reduced.
Specific changes in the skeleton are missing.
an important role in the clinical picture of chronic adrenal insufficiency playing dysfunction of the cardiovascular system.Hypotension in chronic adrenal insufficiency can be one of the first symptoms of the disease, appearing well before the development of the clinical picture.The degree of hypotension is different, for the most part it varies between 100 / 60-80 / 50 mm Hg.Art., giving a large scale during the day.But if the disease was preceded by hypertension, the blood pressure numbers are normal.With an increase in blood pressure usually falls adrenal insufficiency, especially diastolic, reaching very low, sometimes not determined numbers for crises.
Chronic adrenal insufficiency is often orthostatic hypotension and a decrease in pulse pressure.
pigment deposition on the dorsum of the foot, especially in the interphalangeal joints,
in patients with chronic insufficiency of the adrenal cortex.
Chronic adrenal insufficiency.The patient was 28 years old.The deposition of pigment in the interphalangeal joints of the hand back of the hand and the palmar creases.
lesions of the heart muscle are shown on the ECG low voltage of all of the teeth, especially the lower izoelektrichnym T wave in all leads.Often lengthening of the interval S - T, and sometimes negative T1, T2, T3 and T in the chest leads, prolongation of PQ and QRS.First degree heart block (P - Q = 0,20 or more) was found in 60% of patients with Addison's disease.In the treatment of chronic adrenal insufficiency cortisone comes a significant shortening of the interval P - Q.
ECG changes before and after treatment can be delivered in connection with a change in the potassium blood levels (before treatment - a high level of potassium, it is normalized after treatment).Bradycardia, often observed in chronic adrenal insufficiency, especially during periods of worsening condition of patients, can also be attributed in part due to a significant and sustained increase in serum potassium.
If untreated Addison's disease is not observed severe heart failure that is likely to be explained by the large common asthenia, in which patients are in complete rest.However, the slightest movement causes a significant shortness of breath and tachycardia.
patients with chronic insufficiency of the adrenal cortex is often achlorhydria up to 50%, which can be attributed to insufficient cortisol production.Some patients develop chronic gastritis or gastric ulcer or duodenal ulcer.