Polyglandular Syndromes

POLYGLANDULAR SYNDROMES, a term used in endocrinology to denote pathological processes in which there is involvement of several endocrine glands, provided that the time of onset and the degree of involvement of these glands are approximately the same. In this, polyglandular syndromes differ from other endocrine diseases in which a number of glands become diseased sequentially, but where it is always possible to isolate the primarily affected gland, which, in view of the correlative connections between endocrine glands, caused the involvement of other glands as well. Polyglandular syndromes were first described in 1907 by Claude and Gougerot under the name "pluriglandular endocrine insufficiency"; it is more correct to speak of polyglandular syndromes, since along with insufficiency, one or another gland may also exhibit increased activity. Falta proposed retaining the name "polyglandular insufficiency" for those cases in which there is functional insufficiency; for those cases in which there are anatomical changes, he proposed using the expression "multiple sclerosis of the glands," since here, for the most part, there is fibrous sclerosis of the connective tissue alongside atrophy of the parenchyma. Falta also admits the existence of polyglandular hyperplasia of glands with increased activity, under which he classifies gigantism. In polyglandular syndromes, 3 or 4 glands are most often involved, namely the thyroid and sex glands, the adrenal glands, and the pituitary gland. This so-called thyro-genito-suprarenal, or thyro-genito-hypophyseo-suprarenal syndrome, occurs in men twice as often as in women. Symptomatically, the phenomena of late eunuchoidism come to the fore, with atrophy of the sex glands, loss of secondary sexual characteristics, and a decrease in sexual functions; the presence of severe anemia, graying, and cachexia gives these patients an older appearance than that of ordinary eunuchs. Along with the noted phenomena, there are hypothyroid symptoms: wrinkled, dry skin, puffiness, severe hair loss on the head (up to the eyelashes), and brittleness of the nails and teeth. Almost always, there is observed severe adynamia (less often dark pigmentation of the skin), apathy, forgetfulness, sometimes a tendency toward depression, insomnia, headaches, joint pain, gastric achylia, polyuria, monocytosis, and eosinophilia in the blood. In rarer cases, the involvement of the parathyroid glands and the pancreas is noted. The involvement of the adrenal glands and the pituitary gland makes polyglandular syndromes very serious in prognostic terms; patients usually live no more than 5-6 years. More favorable in this regard is thyro-sexual insufficiency, a syndrome described by Borchardt, with phenomena involving only two glands. The etiology is very diverse, but in all probability non-specific; tuberculosis and syphilis are noted; individual cases of polyglandular syndromes develop following a suffered acute infection. It should be admitted that polyglandular syndromes develop predominantly in individuals with a congenital insufficiency of the endocrine apparatus. Therapy is generally unsuccessful; polyglandular organotherapy does not lead to the goal. Symptomatically, it is possible to achieve an effect from the use of individual organ preparations.