Raynaud's syndrome
Introduction
Introduction to Reynolds syndrome Raynaud's syndrome is a pathophysiological change characterized by cold or emotional factors that characterize the pale skin, cyanosis and flushing of both hands. This sign is caused by the paroxysmal sputum of the arteries. It is more common in young women, and it occurs in both hands and fingers, but it can also involve both feet and toes. There is no "Renault syndrome" in Chinese medicine, but there are similar records in the literature regarding its clinical manifestations. For example, in the Han Dynasty Zhang Zhongjing's "Treatise on Febrile Diseases", there are "hands and feet cold, pulse fine desires, angelica Sini soup main. If there is a long-term person in the person, plus Wu Hao, ginger soup master. "This disease should belong to the pulse of TCM syndrome, cold, cold limbs, cold hands and feet. basic knowledge Sickness ratio: 5% Susceptible people: more common in young women Mode of infection: non-infectious Complications: insomnia
Cause
Cause of Raynaud's Syndrome
Cold stimulation (40%)
A considerable number of patients with Raynaud's syndrome live in the colder climate of northern Europe, North America, the United Kingdom, northern China and other places. The typical incidence of Raynaud's syndrome is often after the cold stimulation, the frequency and extent of the attack and the coldness of the climate. Related.
Mental factors (25%)
Patients with Raynaud's syndrome are mostly sympathetically excited, and some patients are also induced in the case of high mental stress. It may be due to the disordered state of the patient's vasomotor nerve center, which makes the peripheral arterial smooth muscle tension increase and sensitive to stimulation. Increased in degree.
Endocrine factors (20%)
Renault syndrome is more common in women, and the symptoms of the patient are aggravated during menstruation and reduced during pregnancy, so consideration may be related to the level of secretion of sex hormones.
Occupational factors (10%)
Among workers who have been engaged in vibration work (such as steam drills and chainsaws) for a long time, the incidence of Raynaud's syndrome can be as high as 50%. Studies have shown that when the vibration frequency is 125 Hz, the impact tension on the distal finger artery is quite large. There is a manifestation of subendothelial fibrosis. In addition, among the employees in the frozen food industry, the incidence of Raynaud's syndrome is also significantly increased.
A significant proportion of patients with idiopathic Raynaud's syndrome will show primary disease during follow-up (Table 1). The ischemic changes in the distal extremities caused by these diseases are mainly arteriosclerosis or chronic arterial inflammation, local thrombosis. Caused by peripheral arterial occlusion, rather than simple arterial spasm.
Pathogenesis
The typical triad of Raynaud's syndrome is pale, cyanotic and flushing. The pale fingers are mainly caused by the slow perfusion of capillaries due to the strong spasm of small arteries, resulting in reduced or lack of blood flow in the skin's blood vessels. Accumulation of metabolites may cause the capillaries to include a small expansion of the venules, a small amount of blood flowing into the capillaries, causing cyanosis after rapid deoxygenation; the vasospasm of the extremities is relieved, and a large amount of blood enters the dilated capillaries, ie, reactive hyperemia occurs. The color changes to flushing; when the blood flow is restored, the capillary perfusion is normal, the episode stops, and the skin color returns to normal.
The pathogenesis of vasoconstriction during the onset of Raynaud's syndrome has been studied for a century. Renault believes that Raynaud's syndrome is caused by abnormal neurological function, but this theory is denied by Lewis. Lewis repeatedly uses local anesthetics to block patients with Raynaud's syndrome. The autonomic and body wall nerves, but not effectively prevent the onset of Raynaud's syndrome, he believes that the onset of Raynaud's syndrome is due to local vascular function defects, caused by high blood vessel wall response under cold stimulation.
When the contractile force of the artery is greater than the expansion force of the transarterial lumen, the blood flow in the artery is terminated. Lewis's study shows that when the Raynaud's syndrome occurs, the blood flow of the finger artery is completely interrupted, causing the pressure of the finger artery to be interrupted by 0.667. About kPa (5mmHg), clinical observations show that there are two pathophysiological mechanisms of arterial occlusion and arterial spasm caused by the onset of Raynaud's syndrome.
Patients with occlusive Raynaud's syndrome due to proximal arterial occlusion, decreased intra-arterial dilatation force, normal vasoconstriction response caused by cold and emotional stimulation, can terminate the blood flow of the finger artery, the onset of Raynaud's syndrome, many disorders can lead to Arteriolar occlusion, the most common cause of which is arteritis associated with atherosclerosis and autoimmune connective tissue disease. Normal arterial systolic sputum-finger arterial pressure changes between 1.33 and 2.00 kPa (10 to 15 mmHg). Abdominal arterial pressure below 4.00 kPa (30 mm Hg) or two-finger arterial pressure difference > 2.00 kPa (15 mm Hg), suggesting significant arch or finger artery occlusion, plethysmographic studies indicate: arterial occlusive disease and arterial cold sensitivity There is a quantitative relationship, and patients with a significant decrease in pressure in the arterial lumen due to occlusion of the arch and finger artery are prone to Raynaud's syndrome.
The pathophysiological mechanism of Raynaud's syndrome caused by arterial spasm is still not fully understood. Patients with spastic Raynaud's syndrome have no significant arch and finger artery occlusion. Normal arterial pressure is normal at room temperature. Arterial angiography studies show that under normal circumstances, Renault synthesis The patient's finger artery has no obvious lesions, but under cold stimulation, the blood flow of the finger artery can be completely interrupted. Krahnbahl et al studied the hemodynamics of the finger artery under cold stimulation in patients with spastic Raynaud syndrome, when the temperature dropped. Before 28 °C, the patient's arterial pressure decreased little. When the temperature dropped to 28 °C, sputum suddenly occurred and the arterial blood flow was interrupted.
Clinical and experimental studies have shown that the adrenergic nerve activity is enhanced in patients with spastic Raynaud's syndrome. The arteriovenous shunt is significantly reduced when the normal finger is cold, and the blood flow of the capillaries is not significantly changed. The patients with spastic Raynaud's syndrome at room temperature. Under cold stimulation, arteriovenous shunt and capillary flow were significantly reduced. After sympathetic blockers, patients with spastic Reynolds syndrome had significantly increased arteriovenous shunt and capillary flow at room temperature and cold stimulation. Studies have shown that increased adrenergic nerve activity may be the main factor in the pathophysiology of spasticity Renault syndrome.
Changes in -adrenergic receptors in vascular smooth muscle cells may be associated with repeated cold stimuli, which may also be related to the pathophysiological mechanisms of patients with spastic Raynaud syndrome. Keenan and Porter's studies have shown that circulating platelets in patients with spastic Raynaud's syndrome Among them, the level of 2-adrenergic receptor is significantly higher than that of patients with occlusive Raynaud syndrome and normal people. The serum of normal humans is incubated with the serum of patients with sputum Raynaud syndrome. The level of 2-adrenergic receptors on normal human platelets is obvious. The decline, while the control group did not have this change, these studies show that the regulation of receptors is carried out by increasing the synthesis of cellular receptors, although receptors on the platelet membrane and receptor levels on vascular smooth muscle are still needed in humans. Further quantitative studies, but experimental and clinical studies support the direct link between the two, and believe that changes in receptor levels are the most fundamental abnormalities in many factors leading to vasospasm.
Many other factors are also thought to be involved in the pathophysiological processes of spasticity of Raynaud's syndrome, such as changes in blood viscosity, abnormal serum proteins, increased serum serotonin levels, and changes in vascular shear stress. It is believed that vaso-related peptides, such as calcium-related peptides, endothelin, etc., are also involved in the onset of spasticity of Raynaud's syndrome, but are not a major factor in most patients.
Prevention
Reynolds syndrome prevention
1. Keep warm in winter, prevent local cold, and drink a small amount of wine.
2. Do not smoke, avoid the use of various vasoconstrictor drugs and -blockers.
3. The skin should be kept clean, avoid trauma, and promptly treat various diseases that can cause vascular damage.
4. Keep indoors warm and disinfect regularly.
5. Avoid unnecessary emotional excitement and mental stress.
6. If there is ulcer or gangrene in the affected area, pay attention to the cleansing of the skin, if necessary, with the drug fumigation and topical application.
7. If you have both systemic symptoms such as fever, aversion to cold, and body aches, you should control infection and symptomatic treatment in a timely manner.
Complication
Reynolds syndrome complications Complications insomnia
Often accompanied by emotional irritability, depression, sadness, suspicious, insomnia, multiple dreams, pain in the body and other symptoms.
The disease is long and repeated and frequently reported by the author, which is characterized by thinning of the skin of the fingers, tightening, toughness, joint failure or stiffness, and even rest pain and finger ulcers.
Symptom
Symptoms of Reynolds Syndrome Common Symptoms Skin on the part of the body... Skin pale skin pain Burning skin Blue-purple changes the skin on the back of the hand Redness and swelling Skin elasticity Poor nails appear blue-purple hand Skin purple finger cyanosis or necrosis
1. Symptoms: The onset is slow, usually after the cold. Especially after the finger touches the low temperature, it occurs frequently in winter. When the attack occurs, the skin color of the finger turns white, and then the purple cicada, often starts from the fingertips and then spreads the whole finger or even the palm. Accompanied by local cold, hemp, needle-like pain or other abnormal feeling, while the wrist pulse is normal, the attack lasts for a few minutes and then relieves itself. The skin turns into flushing with burning, tingling, and then turns into normal color. Local warming, sputum, swinging upper limbs, etc. can often terminate the attack. The affected fingers are often symmetrical. The little finger and the ring finger are often the first to be affected. Later, the other fingers are affected, the thumb is not tired, the lower limbs are rare, and the seizure interval is divided. There are no other symptoms in the hands and feet, and in some patients with severe illness, the seizures are persistent and almost intermittent.
2. Typical episodes can be divided into 3 phases
(1) Pale: This is an early manifestation, sudden occurrence, small arteriolar spasm of the extremities, resulting in slow capillary perfusion of tissue, decreased blood flow in the blood vessels of the skin, when blood flow is severely reduced or blood flow in capillaries is stagnant. At the time, there will be a "dead finger" phenomenon, which is characterized by a symmetrical occurrence in the two-finger arteries. In severe cases, the palmar artery may be involved. If the hand and foot are simultaneously ill, the condition of the hand is always heavier than the foot, occasionally the tip of the nose. In the case of buccal and auricular auricular disease, it is rare that the lingual artery also has a paralysis phenomenon. The onset usually starts from the fingertip end, and the posterior direction develops toward the root or palm of the finger, but rarely exceeds the wrist bone area. The skin pale begins with a finger. The order is mostly 4th, 5th, 3rd, and 2nd fingers. Because the blood circulation of the thumb is rich, the color of the skin can be changed only when the condition is severe. The hand has a cold feeling and the local temperature is lowered. Or numbness, acupuncture, cumbersome and stiff, etc. If the attack lasts, the sensory function may decrease; if the motor function is affected, it may cause movement disorder or hyperhidrosis.
(2) Cyanosis: After a few minutes, the small arteries are relieved, and the small veins are still in a state of paralysis, which hinders blood flow. At this time, hypoxic paralysis occurs in the nipple venous plexus and capillaries, blood flow is slow or stagnant, resulting in blood. The amount of oxygen in the medium is reduced, and the amount of reduced hemoglobin is increased, so that the cyanosis phenomenon occurs after the skin is pale, and the symptoms are generally mild.
(3) flushing: When the patient is in a warm environment, after the cold stimulation is relieved and the vasospasm disappears, the fine arteries, capillaries and venules expand and the blood flows in, and the skin becomes flushed. At this time, the local temperature increases. There may be mild burning pain and fighting pain. When the blood perfusion is normal, the attack stops and the skin color returns to normal.
The above-mentioned typical episodes are more common in Raynaud's disease. The Raynaud phenomenon is similar to the early clinical manifestations of Raynaud's disease, but it is often less typical, and some may not be continuous changes of the above regularity, such as cyanosis without pale or pale without bruising. Or no flushing and return to normal by pale and/or bruising, which is less common or seen in advanced cases.
Light cases last only a few minutes after each episode, returning to normal after local warming, no abnormalities in the skin between the two episodes, severe seizures lasting longer or frequent episodes, even in summer or rainy weather can cause seizures, and It must be warmed by the whole body and the sense of chills can be restored to normal. This patient is generally cold, and often has central nervous system disorders, such as easy to be excited or emotional, suspicious.
3. Signs
(1) Excitation test: 1 cold water test: immersing the finger in cold water of about 4 ° C for 1 min, can induce typical seizures, 2 fist test: after 1 min of fist clenching in both hands, the fingers can be released under bending, and seizures can occur.
(2) Finger skin temperature recovery: After 15 minutes of normal test, the endothelial temperature returned to normal, and the patient exceeded 20 minutes.
Examine
Examination of Raynaud's syndrome
(1) Incentive test:
1 cold water test: immersing the finger (toe) in cold water at about 4 ° C for 1 minute, can induce the above typical episode,
2 clenching test: After two and a half minutes of fisting in two hands, the above changes can also occur after releasing the finger in a bent state.
3 Soak your hands in water at 10 to 15 °C, and expose your body to a cold environment.
(B) refers to the measurement of arterial pressure: the measurement of the arterial pressure is as accurate as the arterial angiography by photoplethysmography. If the arterial pressure is lower than the brachial artery pressure >5.33 kPa (40 mmHg), it is indicated as obstructive.
(C) the relationship between temperature and finger arterial pressure: normal, with a decrease in temperature, only mild arterial pressure drops; sputum type, when the temperature drops to the trigger temperature, the arterial pressure drops suddenly; obstruction type, refers to arterial pressure It gradually decreases with decreasing temperature, but the arterial pressure is significantly lower than normal at normal temperature.
(IV) Determination of temperature recovery time: measured by photoplethysmography, after immersion in ice water for 20s, the average time for the finger temperature to return to normal is 5 to 10 minutes, while the intrinsic patients are often extended to more than 20 minutes.
(5) refers to angiography and hypothermia (after immersion in ice water) refers to angiography: in addition to the diagnosis, this method can also identify the existence of organic changes in the arteries of the extremities. This method is especially suitable for patients with ischemic ulcers. .
(6) A wrinkle microcirculation examination: It is helpful to distinguish whether it is Raynaud's disease or secondary Raynaud's sign. The microcirculation changes are different in the three different stages of the intermittent period and the attack period. Abnormally seen, the lighter has microvascular torsion and twisted shaped tube sputum (in morphological changes) occasionally slight granulocyte-like blood cell aggregation; severe red blood cells ooze around the capillaries, occasionally small bleeding points, inside the tube The blood flow is slowly stagnant. For example, the Raynaud phenomenon caused by connective tissue disease can be seen that the dome is significantly enlarged or the microvascular caliber is extremely expanded to form a "giant tube", and there are layered bleeding points around the tube.
(7) Others: blood antinuclear antibodies, rheumatoid factor, immunoglobulin electrophoresis, complement, anti-DNA antibody, cryoglobulin and Coombs test; determination of nerve conduction velocity of upper limbs helps to discover carpal tunnel syndrome; hand X Line examination helps to find rheumatoid arthritis and finger calcification.
Sometimes to find the primary disease, should be related to laboratory tests and other auxiliary examinations, such as suspected and autoimmune connective tissue disease, should check serum anti-nuclear antibodies, anti-asDNA antibodies (SLE specific antibodies), anti-wire Dot antibody (specific antibody to CREST syndrome), anti-Scl-70 (specific antibody to PSS), rheumatoid factor, immunoglobulin, complement, cryoglobulin assay, Coombs assay, anti-RNP antibody (for mixed connective Tissue disease is specific).
Diagnosis
Diagnosis and diagnosis of Raynaud's syndrome
diagnosis
1. With the typical Raynaud's sign, that is, in the case of cold stimulation or emotional agitation, the skin of the extremities has a regular color change, from pale to hairy flushing normal.
2. Most of the symmetry is good for women aged 20 to 40.
3. Even in a small number of critically ill patients, the finger (toe) artery is occluded, while the upper limb paralysis, ulnar artery, lower extremity paralysis and dorsal artery pulsation are good.
4. Severe patient finger (toe) occurs skin dystrophies, skin elasticity is reduced, shallow ulcers and gangrene are limited to the fingertips.
5. Patients with Raynaud's disease generally have no abnormalities when they are examined. The Renault sign is accompanied by the clinical manifestations of a certain primary disease. Further laboratory tests and auxiliary tests can be performed to establish the primary disease.
6. For patients with a lack of typical episodes, one or two of the auxiliary examinations may be used to determine the diagnosis.
Differential diagnosis
Care should be taken to distinguish from other vascular dysfunction diseases characterized by skin color changes.
(1) Hand and foot cyanosis: It is a vasospasm disease caused by autonomic dysfunction. It is more common in young women. The skin of the hands and feet is symmetrical and even purple, and the cold can make the symptoms worse, often accompanied by skin scratches or sweating of hands and feet. Such as autonomic dysfunction, its pathological changes are persistent arteriolar arteries and capillary and varicose veins, need to be differentiated from Raynaud's syndrome, patients with hand and foot carbuncle have no typical skin color changes, the range of sputum is more extensive, involving The entire hand and foot can even involve the entire limb. The purpura lasts for a long time. Although the cold can make the symptoms worse, in the warm environment, the symptoms can not be relieved or disappeared immediately. Emotional hormones and mental stress generally do not induce the disease.
(2) reticular bluish: mostly female, due to small arterial spasm, capillaries and veins without tension expansion, the skin is persistent reticular or spotted purpura, lesions mostly occur in the lower limbs, occasionally involving the upper limbs, trunk and On the face, the affected limb is often accompanied by chills, numbness and abnormal feeling. The blemishes are obvious when the cold or the limbs are drooping. After the warm environment or the raised limbs, the markings are alleviated or disappeared. Clinically, it can be divided into marble-like skin spots. There are three types of reticular leukoplakia and symptomatic reticular leukoplakia.
(3) erythema extremity pain: the cause is still unclear, the pathological changes are symmetry of the extremities, aging of the vasodilatation, more common in young women, rapid onset, both feet at the same time, occasionally involving both hands, symmetry Paroxysmal severe burning, when the temperature of the foot exceeds the critical temperature (about 33-34 ° C), such as the foot in the warm bedding, the pain can occur, mostly burning, can also be stinging or pain The limbs are drooping, standing, and exercising can induce pain episodes, raise the affected limbs, rest or expose the feet to the outside of the bedding, and the pain can be alleviated. When the symptoms occur, the skin color of the feet is flushed and bloody, and the skin temperature rises. Sweating, increased back and posterior tibial artery pulsation, according to the present characteristics, easy to be similar to Raynaud's syndrome, a small number of erythematous limb pain can be secondary to polycythemia or diabetes.
(4) Frostbite syndrome is more common in areas with low temperature and humidity, especially in early winter and early spring. It is more common in children and adolescents. The predilection is in the hands, feet, ears, nose, and frostbite. The patient is sensitive to cold. The skin is red and swollen, followed by a linear small lump in the purple-red circle, pain, local congestion after heat, itching, and even blisters, ulceration, slow healing, often leaving atrophic scars, the disease often relapses year after year.
(5) cryoglobulinemia This disease is an immune complex disease, about 15% of patients with Raynaud's phenomenon as the first symptom, mainly characterized by skin purpura, hemorrhagic skin lesions intermittently in the lower limbs, often left after disappearing Pigmentation, severe cases of ulcers in the external iliac crest, a few may have gangrene gangrene, ulcers also found in the nose, mouth, throat, tracheal mucosa and ears, about 70% of patients have multiple joint pain, 50% of patients have kidney damage, followed by Hepatosplenomegaly, nervous system damage, etc., blood cryoglobulin increased, C3 complement decreased, RF positive, increased gamma globulin.
(6) The intrinsic carpal tunnel syndrome is caused by the compression of the median nerve in the carpal tunnel. The main manifestation is the burning pain of the finger. After the activity, the numbness can be relieved, the finger pain is reduced or the feeling disappears, and the muscle muscle atrophy. But there is no intermittent skin color change, no symmetry.
In addition, it should distinguish between idiopathic and secondary, secondary secondary to various systemic diseases, such as: thromboangiitis obliterans can be secondary to Raynaud's phenomenon, patients are almost all male, longer course, dorsal artery The symmetry beat is weak or disappears, and the upper stage of scleroderma can also be followed by the Raynaud's upper arm, and the facial, neck and chest skin and subcutaneous tissue are obviously changed.
