REYNAUD DISEASE ( M. Raynaud , fr. doctor, 1834 — 1881; synonym symmetric gangrene of extremities) — the disease which is characterized by pristupoobrazny disorders of arterial blood supply preferential of brushes and feet. R.'s signs. for the first time in detail described M. Reynaud in 1862. It is tended to distinguish a Raynaud's disease as clinically isolated form angiotrofonevroza (see) and a syndrome (symptom complex) of Reynaud — similar disorders of local blood circulation, but secondary in relation to diseases, various by the nature, or the external damaging influences. Most often R.'s syndrome is observed at sclerodermas (see).
R.'s Aetiology. it is not specified, as well as at other angiotrofonevroza. Hereditary predisposition, constitutional deficiency of a vasculomotor innervation and its functional disturbances of not studied nature matter; a part is played, apparently, by psychogenic factors, injuries of c. N of page, hron. intoxication nicotine, alcohol. River. it is more often observed at young women, sometimes develops after inf. diseases, in some cases after influence of provocative factors (overcooling, unlimited insolation, overfatigue). Pay attention to rather high incidence among typists and pianists, and also to R.'s combination. with migraine (see).
General diseases of connecting fabric and related processes to them can be the cause of development of a syndrome of Reynaud (a scleroderma, a dermatomyositis, Hammen's disease — Rich); dysfunction of ovaries, diseases of a thyroid gland and adrenal glands; diseases of a nervous system (e.g., inflammation of ventral roots of spinal nerves; the diseases leading to a cervicobrachial syndrome (additional edges, a myopachynsis); obliterating defeats of vessels of extremities (see), cryoglobulinemia, etc. Can lead thermal defeats at chronic impacts of cold of type to development of a syndrome of Reynaud trench foot (see) or effects of sharp temperature variations; bruises of hands, in particular — vibration disease (see), intoxication certain poisons — an ergot, heavy metals (lead, mercury), chlorvinyl. The same exogenous influences at R. can act as the factors promoting its progressing.
R.'s Pathogeny. it is considerably connected with features of blood circulation in to skin (see), a cut at the person effectively participates in thermal control of an organism and defines a local thermal conditions in skin. The greatest value has it for fingers of hands and legs, brushes and feet where the vascular bed is adapted for providing the blood-groove many times surpassing local exchange requirements abounds with specific arteriovenous anastomosis (see), participating in local thermal control; has very extensive network of superficial veins. Arterial inflow and its distribution to shuntovy and capillary fractions, and also degree of a delay of blood in veins are controlled by the thermoregulatory centers of a hypothalamus through sympathetic nerves. The gleam of an arteriovenous anastomosis entirely is defined by a neurogenic impulsation; reduction of a blood-groove in fingers promotes their cooling, reduces fabric exchange, but does not lead to education those metabolites here, to-rye at ischemia in other areas effectively counteract neurogenic restriction of a blood-groove (bradikinin, collidine). Vessels of skin have hypersensitivity to sympathetic influences and action of catecholamines. The sympathetic control of blood circulation of distal departments of extremities which is extraordinary expressed, not having counteraction explains an originality and the local nature of changes at R., is the main reason that here the frustration unusual for other vascular areas are observed. Primacy of neurotrophic disturbances at R. is confirmed by strengthening of catecholamines in the blood flowing from distal departments of extremities, detection of structural changes in regional nodes of a sympathetic trunk, efficiency of the treatment directed to suppression of sympathetic influences: from use of ganglioblokator and physiotherapeutic procedures to sympathectomies (see).
Along with functional neurogenic mechanisms also local structural changes of vessels, their hyperreactivity, especially on temperature influences matter. Even after denervation of vessels remains patol. sensitivity of manual arteries to impact of cold. During an initial stage of an attack the spasm of arterioles, small arteries, an arteriovenous anastomosis prevails. In process of its permission the hyper tone of post-capillary sphincters is observed, there comes paresis of venules and veins (pallor of fabrics is replaced by their cyanosis and puffiness).
In patomorfol. to a picture P. the hypertrophy of smooth muscles of manual arteries is characteristic. Further there occur bone changes of dystrophic type in phalanxes of fingers, interphalangeal joints, ossifluence of nail tuberosity (tuberosity of distal phalanxes). The most rough local changes are observed at development necrosis (see) and gangrenes of soft tissues (see. Gangrene ), sometimes with involvement of bones (see. Osteonecrosis ). However these changes do not differ from observed at burns, freezing injuries, a leprosy, a scleroderma, back to tabes and nevrol. diseases. At various options of a syndrome of Reynaud reveal morfol. signs of the basic disease which led to its development.
A clinical picture
the II—IV fingers of brushes and feet Are surprised preferential; later also other parts of a body (a nose, ears, a chin) which are exposed to cooling can suffer. For R., unlike Reynaud's syndrome, strict symmetry of manifestations, earlier involvement of hands, than legs, staging in progressing of a disease is characteristic.
Usually during R. allocate 3 stages.
In I stages the short-term attacks of ischemia of fingers lasting for several minutes are observed. Due to the cooling of hands (washing by a cold water, rinsing of linen), less often under other circumstances (nervousness, smoking) there comes sudden numbness of fingers or their distal departments. Fingers become cold, alabaster-white (a symptom of «a dead finger»), burning, the aching pains appear, to-rye can become hardly transferable even at moderate cooling.
These symptoms at the termination of impact of cold pass in a few minutes. Warming of hands promotes improvement of a state even if the attack was provoked not by thermal external influence. A recurrence of attacks with gradual shortening of the mezhpristupny periods (during these periods no aberrations are noted) and transition of a disease to the following stage is characteristic.
II stage it is characterized by increase in duration of attacks to an hour and more, their emergence sometimes without the noticeable external reasons, development during the termination of an attack of a phase of cyanosis, sometimes with small puffiness of fabrics.
In III stages attacks lead to profound local changes: on skin of fingers bubbles with hemorrhagic contents are formed, there are necrotic changes, superficial ulcers, dystrophic changes of nails (see tsvetn. fig. 1 — 3). A possibility of a massive necrosis of fabrics and gangrene at R. at the I—II stages it is called into question; they are more characteristic of Reynaud's srshdrom at considerable changes of vessels (a scleroderma, arteritis) or are caused by deep disturbances of rheological properties of blood in a vascular bed of extremities at their cooling (cryoglobulinemia).
the Diagnosis of a disease and Reynaud's syndrome is supposed on the basis of characteristic complaints and the description of a typical attack. Initial diagnosis becomes simpler the fact that the symptom of «a dead finger» usually easily is reproduced by means of COLD test.
Radiodiagnosis disease and Reynaud's syndrome consists in definition of extent of trophic changes in bones and surrounding soft tissues, studying of passability and structure of regional arteries.
Rentgenol. studying of soft tissues is carried out by means of a screenless X-ray analysis by «soft» radiation on a special film. Initial rentgenol. manifestations are expressed in emergence of hypostasis of hypodermic cellulose on the affected fingers, a smazannost of its borders, homogenization and decrease in transparency of its shadow on the roentgenogram. In the subsequent symptoms of fibrosis in the form of rough restructuring of soft tissues are defined. Hypodermic cellulose gets rough cellular tyazhistoye a structure, its contours on roentgenograms are not differentiated. On side surfaces of interphalangeal joints the additional soft and woven educations caused by thickenings of folds of skin are defined. Owing to a smorshchennost of skin its shadow on the roentgenogram has ischerchenny character and a gear outside contour. Occasionally in soft tissues dense shadows of calcifications are defined.
Bone manifestations of R. and Reynaud's syndrome are characterized by dystrophic changes in a look osteoporosis (see), ossifluence (see), and also osteonecrosis (see). At the same time patol. changes are usually localized and are more expressed in distal phalanxes of fingers of a brush. At early stages of osteoporosis there is a depression of the trabecular drawing of a bone, thinning of its compact substance, is preferential in the bases of distal phalanxes.
Further osteoporosis extends to other sites of a brush. Quite often cystic zones of reorganization of a bone tissue develop. There is a concentric atrophy of distal phalanxes leading to reduction of their volume, thinning and a point of nail tuberosity. More exact quantitative assessment of osteoporosis is possible by methods rentgenogrammetriya (see) and densitometries (see).
Parallel to osteoporosis the phenomena of ossifluence develop, to-rye originally affect nail tuberosity, and then extend to a considerable part of a phalanx or all phalanx in general (fig.). Owing to the expressed consolidation of soft tissues can develop anchylosis (see) or an incomplete dislocation in interphalangeal joints. In hard cases find an osteonecrosis.
Angiography (see) would have limited value for R.'s diagnosis., but helps to reveal organic forms of disturbances of a blood-groove; in late stages of R. the stenozirovaniye of manual arteries is noted.
Tool methods of a research have auxiliary diagnostic value. Are applied skin thermometry (see) and termografiya (see), reografiya (see), kapillyaroskopiya (see), pletizmografiya (see), an oscillography (see. Oscillography arterial ).
Differential diagnosis disease and Reynaud's syndrome it is most difficult. R.'s recognition. find possible, as a rule, not earlier than after 2 years' observation, as a result of an exception of all other diseases, to-rye can cause development of a recurrent ischemic syndrome of distal departments of extremities, especially sclerodermas. At a system scleroderma Reynaud's syndrome is observed more than in 70% of cases, sometimes for several years advancing emergence of other symptoms, patognomoyaichny for this disease. However, as a rule, at general diseases of connecting fabric already at early stages out of attacks the tsianotichnost of fingers remains and there are signs of organic disturbance of blood circulation. Early allocation of the cases of a syndrome of Reynaud caused by professional influences (a vibration disease) is urgent. In some cases to diagnose R. detection of a vasospasm of an eyeground in the period of an attack helps. Tool researches are most informative by comparison of data during the mezhpristupny period and during an attack, and also at use functional and pharmakol. tests. Consider that at R., unlike similar secondary forms of pathology (Reynaud's syndrome), during the mezhpristupny period not decrease is noted, and it is rather an increase in indicators of a blood-groove, vasodilatation according to a kapillyaroskopiya, but in response to cold test there comes the vasomotor spasm and sharply the blood stream decreases. An angiography in the conditions of active pharmakol. tests helps to distinguish functional changes from organic. In differential diagnosis with a cryoglobulinemia biochemical and immunological data — increase matter at the last a caption of Cold hemagglutination, as a rule, higher than 1:30 000 (a normal indicator 1:64), increase in content of immunoglobulins of type M (see. Immunoglobulins ), sometimes the M-gradient on an electrophorus gram of proteins, positive test of Koombs (see. Koombs reaction ).
the Help in the period of an ischemic attack usually comes down to local thermal procedures, offer the patient hot drink, tea. In the absence of effect apply antispasmodics (Platyphyllinum, Dibazolum, a papaverine, Nospanum), alpha adrenoblockers (phentolamine) or ganglioblokator (Gangleronum), tranquilizing means. At systematic treatment find application Isobarinum (Octadinum), Methyldopum, Reserpinum, can be used Mydocalmum, komplamin (Xantinoli nicotinas). Methods of a physiotherapeutic and resort therapy — the general for various angiotrofonevroz (see).
At Reynaud's syndrome the leading place is taken by treatment of the main disease.
Surgical treatment of a Raynaud's disease is directed to elimination or reduction of vasculomotor influences of sympathetic department of century of N of page by intervention on a sympathetic trunk, its nodes, connecting branches, vegetative educations! in vessels.
Already in 20 — the 30th 20 century were reported about positive effect of such operations for century of N of page as gangliectomy (see), ramicotomy (see), sympathectomy (see), desimpatization. In process of accumulation of the facts began to avoid interventions on a star-shaped (cervicothoracic) node at angiospastic defeats of vessels of upper extremities in connection with the coming cosmetic defect as Bernard's syndrome — Horner (see. Bernard — Horner a syndrome ). More attention was paid by ramicotomies (to a section of connecting branches between a spinal cord and a sympathetic trunk, between nodes of a sympathetic trunk and somatic nerves), to especially preganglionic, deprived of shortcomings cervical gangliectomy. However not always the sufficient effect of the isolated interventions on branches of a sympathetic trunk forced surgeons to complicate and combine interventions. At unsuccessfulness of conservative therapy it is reasonable to begin surgical treatment with blockade (novocaine, lidocaine, Trimecainum, quinine-urea) sympathetic educations (a star-shaped node, nodes of a sympathetic trunk Th2 — Th3, L1 — L2). The short-term desimpatization of vegetative educations allows to estimate medical effect and to specify indications to operation. At damage of upper extremities by the most effective consider a preganglionic ramicotomy (C6 — Th1), the isolated gangliectomy or their combination with a periarterial sympathectomy. As R.'s manifestations. quite often have generalized character and are combined with other vegetative frustration, the gangliectomy of the Th3 node of a sympathetic trunk across Ognev is of interest. This operation improves blood circulation both in lower, and in upper extremities. Operation in early terms of a disease (before development of organic lesions of vessels) allows to reach higher effect of treatment, than in late stages.
At Reynaud's syndrome caused by tumors in the field of a brachial plexus, an additional cervical edge, osteochondrosis, gangl an ionite of the toxi-infectious nature, primary defeat of arterial vessels, surgical treatment consists in elimination of the reason of a syndrome of Reynaud — removal of the center of a compression of nervous roots or vessels, reconstructive operations on vessels.
The forecast and Prevention
the Forecast at R. rather favorable at adequate treatment; at Reynaud's syndrome it depends on the nature of the main disease, on opportunities of its treatment and extent of organic changes in a vascular bed.
Prevention of ischemic attacks at R. consists in the prevention of coolings of extremities taking into account that even at a temperature of 1.5 ° perhaps maximum vasoconstriction. It is necessary to avoid also long stay in conditions of high humidity of the environment, adverse professional effects, overfatigue, especially emotional, to refuse smoking and to exclude other intoxications.
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E. E. Gogin; I. P. Korolyuk (rents.), V. S. Mikhaylovsky (hir.).