Idiopathic edema

Idiopathic edema (synonyms: primary central oliguria, central oliguria, cyclic edema, antidiabetes insipidus, psychogenic or emotional edema, in severe cases - Parhon syndrome). The vast majority of patients are women of reproductive age. No cases of the disease have been registered before the onset of the menstrual cycle. In rare cases, the disease may debut after menopause. Isolated cases of the disease in men have been described.

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Causes of idiopathic edema

The name "idiopathic edema" indicates that the etiology of this disease is unclear. It should be noted that emotional stress, long-term use of diuretics, and pregnancy play a leading role in the onset of idiopathic edema. The listed etiological factors apparently contribute to decompensation of the constitutionally conditioned defect of the central regulatory link of water-salt balance.

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Pathogenesis of idiopathic edema

The pathogenesis of the disease is still unclear. It is believed that the disease is based on hormonal dysregulation of a central nature. Increased secretion of antidiuretic hormone plays a significant role along with increased sensitivity of the renal tubules to this hormone. The role of excessive secretion of aldosterone is also noted. The role of estrogens in the form of a violation of the cyclic rhythm of estrogen secretion with relative hyperestrogenism in the second phase of the menstrual cycle due to progesterone deficiency has also been identified. A number of researchers point to the pathogenetic role of the orthostatic factor and the role of increased transudation of fluid from the vascular bed. Hormonal dysfunction underlying the disease is a consequence of a violation of the central mechanisms of regulation of water-salt balance, mainly the hypothalamic-pituitary link.

Symptoms of idiopathic edema

The main symptoms of idiopathic edema are periodically occurring edema with oliguria. The edema is soft and mobile, most often located on the face and paraorbital areas, on the hands, shoulders, shins, ankles. Hidden edema is also possible. Clinical manifestations vary depending on the severity of the disease: there is a mild form with minor edema of the face and ankles, as well as a severe form, in which pronounced edema tends to generalize. With generalized edema, its distribution depends on gravity. Thus, upon awakening, edema is most often localized on the face, after taking a vertical position and by the end of the day it descends to the lower parts of the body.

Depending on the clinical course, two forms of the disease are distinguished - paroxysmal and permanent. Some predominance of the paroxysmal form is reflected in the name of this syndrome - periodic, or cyclic, edema. The paroxysmal form of the disease is manifested by periodic edema with oliguria and high relative density of urine, which are replaced by periods of polyuria, when the body gets rid of excess water. Periods of oliguria are usually long - from several days to a month. Then they can be replaced by periods of polyuria, usually shorter. The duration of polyuria can be measured in hours, when up to 10 liters of urine are excreted in half a day, and days, when the amount of urine excreted daily during the week is 3-4 liters.

Cycles of the disease (oliguria - polyuria) appear at different intervals. Factors that provoke the onset of an edematous attack may be emotional stress, heat, premenstrual period (the second, luteal phase of the cycle), pregnancy, change in diet, climatic conditions. In the permanent phase of idiopathic edema, the edema is constant, monotonous, and not periodic. In severe clinical cases, at the height of edema with an increase in body weight due to fluid, as a rule, by more than 10 kg, symptoms of water intoxication may develop. They are manifested by headache, dizziness, shortness of breath, adynamia, confusion. The period of edema decline with pronounced polyuria may be manifested by symptoms of dehydration. During a period of longer polyuria, general weakness, loss of appetite, thirst, vegetative manifestations are characteristic, usually in the form of tachycardia, a feeling of interruptions in the heart area, cardialgia. Thirst is an obligatory sign of the disease and, along with oliguria, the main mechanism for the formation of edema.

Positive water balance with fluid retention in the body leads to rapid weight gain. Fluctuations in body weight with and without edema range from 1 to 14 kg. Rapid weight gain of 1 kg or more per day necessarily indicates fluid retention in the body, and not an increase in fat content. This is an important diagnostic sign that should be remembered, since with hidden edema, patients often complain of obesity with periods of rapid fluctuations in body weight.

Idiopathic edemas are often combined with other neuroendocrine disorders: obesity, dysfunction of the sex glands in the form of amenorrhea or oligomenorrhea, hirsutism, bulimia, decreased libido, sleep disorders. Emotional and personal disorders, as a rule, are clearly presented in the form of astheno-hypochondriacal disorders. Vegetative disorders are obligatory signs, manifested by permanent and paroxysmal disorders. Permanent vegetative disorders are extremely diverse: they can be observed as increased dryness, as well as increased moisture of the skin, as a pronounced decrease, as well as a significant increase in blood pressure, tachycardia, sweating, decreased skin temperature. Paroxysmal vegetative disorders are detected only with pronounced psychopathological manifestations and can be either sympathoadrenal or mixed in nature.

Neurological examination, along with radiological and electroencephalographic examination, does not reveal any pathognomonic signs. Scattered microsymptomatology and signs of dysraphic status are revealed.

Skull radiographs often reveal compensated intracranial hypertension, hydrocephalic skull shape, and frontal hyperostosis. EEG is extremely diverse: along with normal bioelectrical activity of the brain, signs of involvement of the upper brainstem structures are often detected. The fundus shows retinal vascular dystonia with a tendency to narrowing of small arteries. It should be remembered that at the height of intense edema (weight increase up to 10 kg), congestion is possible in the fundus, which completely disappears with the disappearance or significant reduction of edema.

Differential diagnosis of idiopathic edema

The diagnosis of idiopathic edema syndrome is made by excluding other pathological conditions that can contribute to fluid retention in the body (heart failure, kidney pathology, liver cirrhosis with ascites, narrowing of venous and lymphatic vessels, dysproteinemia, allergic and inflammatory diseases, hypothyroidism).

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Treatment of idiopathic edema

Treatment of idiopathic edema should begin with discontinuing diuretics, especially chlorothiazide. A long-term diet with limited salt is recommended. A positive effect is achieved by using large doses of Veroshpiron - up to 6-9 tablets per day. In some cases, a positive effect is achieved by using Bromocriptine (Parlodel) 1/2 tablet (1.25 mg) 3-4 times a day for six months. A significant place among therapeutic measures is occupied by differentiated psychotropic therapy, carried out in individually selected doses depending on the severity of psychopathological manifestations.

Often it is necessary to combine drugs with antidepressant and neuroleptic effects. Among neuroleptics, drugs such as melleril (sonapax), teralen are preferable, among antidepressants - pyrazidol, amitriptyline, azafen. Among vegetotropic drugs, anaprilin in a dose of 40-60 mg, divided into 4 doses, has a positive therapeutic effect. The main principle of therapy is its complexity.