Addison's Disease

ADDISON'S DISEASE (morbus Addisonii), first described in 1855 by the English physician Thomas Addison. The most characteristic signs of A., or bronze disease, are the gradual appearance of bronze-brown discoloration of the skin and mucous membranes, progressive weakness, emaciation, disturbance of digestive organs (dyspeptic phenomena, constipation, more rarely diarrhea), decrease in muscular strength and blood pressure, pain in the lumbar region, in muscles and joints.--Etiology. The disease A. b. is closely connected with hypofunction of the adrenal glands, and statistical data show that in more than 80% of cases the cause of the disease is tuberculous damage to the adrenals; more rarely, an acute inflammation of the adrenal glands, their suppuration, destruction of the adrenals due to hemorrhage, atrophy and sclerosis of the adrenals, thrombosis of the adrenal veins, malignant neoplasms or syphilitic damage to the adrenals serve as the etiological factor. The presence of a hypernephroma can sometimes also be accompanied by symptoms of A. b. Thus, it can be considered established that in most cases the basis of A. b. is the loss of adrenal gland tissue; the question of which part of the adrenals - the cortical or medullary substance - has the main significance is not yet fully resolved at the present time. Besides pathological processes in the adrenal glands, in some cases the cause of the development of purely functional A. b. can also be a disease of the nervous apparatus regulating the activity of the chromaffine system. In the development of A. b., disturbance of the activity of the entire endocrine apparatus and various intoxications, especially toxins of tuberculosis, diphtheria and typhus, have significance. Among the possible etiological factors for the development of the symptom complex of A. b., trauma, air contusion, poisoning by suffocating gases and even psychic influences are also mentioned.-Path-anatomical data in A. b. in the vast majority of cases come down to changes in the adrenal glands, usually both, more rarely one. Tuberculosis, cancer, sarcoma, gummatous infiltrations, suppurations, acute and chronic inflammations, atrophy, sclerosis, hemorrhages, thrombosis of vessels, sometimes hypernephromas - these are the path-anatomical changes in the adrenal glands that are most frequently encountered in A. b. Various changes (small-cell infiltration, atrophy, degeneration of fibers, etc.) in the solar plexus, in the nodes and trunks of the sympathetic nerve are also often noted. Sometimes in A. b. only the mentioned change in the nervous apparatus is found with normal adrenal glands. Very rarely are cases of A. b. encountered in which no changes are found either in the adrenal glands or in the sympathetic system. Similarly, cases of complete destruction of the adrenal glands by a pathological process without symptoms of A. b. are sometimes observed; the latter is explained by the possibility of compensatory activity of the paraganglia (see). In many cases, insufficient involution of the thyroid gland and status thymico-lymphaticus are found, which can be considered especially favorable soil for the development of the A. b. syndrome. Microscopic examination of a skin section in A. b. gives a picture of a sharp increase in the amount of melanin pigment in the cells of the Malpighian layer, with an abundant number of pigment-containing wandering cells - chromatophores - in the underlying connective tissue. The melanin pigment does not give a reaction to iron, and on the basis of the latest research, it can be assumed that it is a product of protein breakdown, for example, of tryptophan, and possibly is formed from adrenaline itself. Clinical picture. The first signs of A. b. are most often rapid fatigue after any, even insignificant, physical exertion, weakness, insomnia, depressed mood and digestive phenomena in the form of complete absence of appetite, then nausea, vomiting and constipation, in severe cases replaced by looseness (up to almost uncontrollable diarrhea). The pulse, due to the absence of normal vascular tone, is usually soft and small, blood pressure is significantly lowered, noises in the heart are often noted, due to weakening of the physiological tone. In the blood, phenomena of anemia with relative lymphocytosis and mononucleosis are mostly noted. The most characteristic symptom of A. b. is the brownish-brown pigmentation of the skin, especially sharply expressed on areas exposed to light or friction (face, neck, edges of eyelids, hands), and on areas that are normally pigmented (linea alba, breast nipples, sexual organs, folds around the anus). Pigmentation of the mucous membranes of the lips and oral cavity (cheeks, palate, tongue) is also very characteristic, and sometimes also of the mucous membrane of the vagina and rectum. On the palms and soles only the lines of folds are pigmented; nails and sclerae rarely change color. From the side of the nervous system, dizziness, decrease in intelligence and memory are noted, sometimes convulsive phenomena, fainting and even comatose state. Reflexes_ch are usually normal, excitability of the autonomic nervous system is lowered. Temperature can be lowered, but febrile periods are also observed. The sugar content in the blood is lowered, absence of adrenaline glycosuria and abnormally high tolerance to grape sugar are noted. A. b. sometimes proceeds acutely, accompanied by peritoneal symptoms and a whole series of violent phenomena resembling typhoid disease, but most often the course of A. b. is chronic, accompanied by short-term or prolonged remissions, sometimes even lasting for several years. The average duration of the disease is about 2-3 years, but in individual cases the disease can last even up to 10 years.-In differential diagnosis, skin pigmentations occurring in some forms of cirrhosis of the liver (cirrhose bronzee), pigmentations in diabetes (bronze diabetes), in tuberculosis, in chronic malaria, in malignant anemia and malignant neoplasms, pigmentations in cases of chronic arsenic poisoning (arsenic melanosis) and silver (argyria), and finally, changes in skin color in pellagra, scleroderma, amyloid degeneration and some endocrine diseases (Basedow's disease), in which pigmentation resembling that in Addison's disease is sometimes observed, should be kept in mind. The prognosis in most cases is unfavorable. Therapy, due to the fact that in A. b. there is hypofunction of the adrenal glands, consists in the application of organotherapy, mainly in the form of adrenaline or preparations of adrenal glands, which do not always give positive results, however. Adrenaline is prescribed either per os, in the form of a basic solution of hydrochloric acid adrenaline in amounts up to 30 drops per day, or, more often, subcutaneously in doses of ¹/₄-1 mg per day. Fresh adrenal glands of a sheep (daily 1-2 glands in finely chopped form) and tablets from dried glands, e.g., Merck's adrenaline tablets, containing 0.1 g of gland each, 3-4 times a day, or so-called suprarenal tablets of 0.3 three times a day, are also used. In the literature, reports of successful use of thyroidin in some cases of A. b. are encountered. In cases with clearly expressed tuberculous etiology, a course of treatment with tuberculin can be added to the treatment with preparations from the adrenal glands, which usually does not give encouraging results, just as it does not give a significant therapeutic effect and irradiation of the adrenal gland area. In cases where only one of the adrenal glands is affected by the tuberculous process, good results can be expected from the extirpation of the affected adrenal gland. More favorable results can be expected from the application of antiluetic therapy in cases with luetic character of adrenal gland damage. In recent years, attempts have been made to transplant adrenal glands in A. b.; the results are in general little encouraging. In cases of sharp decline in strength and decrease in tone, droplet enemas or subcutaneous infusions of physiological NaCl solution with adrenaline (1 mg of adrenaline per 300-400 cubic cm of physiological NaCl solution) act well. In severe diarrhea, enemas with adrenaline (20 drops of 1% solution per 250 cubic cm of water) can be prescribed. As general strengthening means - preparations of calcium, iron and arsenic subcutaneously.

D. Russian.

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