Vasculitis

Introduction

Introduction to vasculitis

Vasculitis is an infiltration of inflammatory cells around the blood vessel wall and blood vessels, accompanied by vascular damage, including inflammation of cellulose deposits, collagen fibrosis, endothelial cells and myocyte necrosis, also known as vasculitis. The pathogenic factors acting on the blood vessel wall directly cause the primary vasculitis, and the symptoms and signs on the basis of which are vasculitis diseases; the adjacent vascular lesions caused by the blood vessel wall are secondary vasculitis, but this It is only one of its lesions, so it is not a vasculitis disease. In the so-called collagen vascular disease, vascular injury is one of the important associated lesions, and it should be a generalized vasculitis disease. Vasculitis is not uncommon. Vasculitis lesions not only involve the blood vessels themselves, but also the tissues and organs that the blood vessels control. Some of them can be fatal. Since the application of corticosteroids, the prognosis has changed a lot. The type, size and function of blood vessels are different, and the clinical signs and symptoms of vasculitis are also different.

basic knowledge

The proportion of illness: 0.001%

Susceptible people: no special people

Mode of infection: non-infectious

Complications: allergic vasculitis Pulmonary vasculitis

Pathogen

Cause of vasculitis

Most of the causes are unknown, and a few causes are more clear, such as serum diseases, drug allergies and infections. Hepatitis B virus has been proven to be the cause of a variety of long-term vasculitis, and then found Chinese cytomegalovirus, herpes simplex virus, adult T-cell leukemia The virus can cause vasculitis.

The occurrence of primary vasculitis is mainly caused by immune abnormalities, while the research is more humoral immunity, and less research on cellular immunity. It is sensitized both endogenous and exogenous, with nuclear antigen, cryoglobulin and denaturing immunoglobulin in the endogenous; multi-drug and infectious factors in the exogenous antigen, but In most diseases, the cause is still not easy to determine.

Prevention

Vasculitis prevention

1. One egg at most one per day.

2, milk up to half a catty.

3. Eat more green vegetables.

4, kelp, seaweed, fungus eat more.

5, soy products + coarse grains to eat.

6, the spirit should relax, do not think about their patients every day.

7. To properly communicate with people.

8, absolutely can not smoke, drink, low-salt diet as well.

Complication

Vasculitis complications Complications Allergic vasculitis Pulmonary vasculitis

The lesion may have thrombosis or aneurysmal expansion, and the healing lesion may have fibrous tissue and endothelial cell proliferation, which may lead to stenosis of the lumen.

Symptom

Vascular inflammation symptoms Common symptoms Kidney damage Liver function impaired Breathing difficulty Acute inflammatory cells Infiltrating cerebral ischemia

Symptom characteristics

1, the lungs: diffuse, interstitial or nodular lesions, can have cough, shortness of breath, difficulty breathing, etc.;

2, kidney: the appearance of hematuria, proteinuria, high incidence, often early renal dysfunction;

3, liver: liver discomfort, liver function damage;

4, cardiovascular: the emergence of no pulse, bilateral limb blood pressure difference increased;

5, the nervous system: due to intracranial blood supply vascular disease, causing cerebral ischemia symptoms, intracranial vasculitis can cause intracranial hemorrhage or nodular lesions. Supply of vascular lesions of peripheral nerves, which can cause neuropathy and neurological sensation and dyskinesia;

6, nasopharyngeal: in Wegener granulomatosis (one of vasculitis), may have nasopharynx granulomatous lesions, nasal congestion, nose bleeding and other symptoms;

7, skin: a lot of vasculitis will appear rash, skin vascular changes, and sometimes rash is the only clinical manifestation. In allergic purpura, there may be skin bleeding spots and bleeding spots. Some manifest as nodular lesions, which appear after mosquito bites, and are found in allergic vasculitis and Behcet's disease. In addition, there are joint pains, gastrointestinal symptoms and the like.

Clinical manifestation

1. The main performances are:

1 multiple system damage;

2 active glomerulonephritis;

3 ischemic or blood stasis symptoms and signs, especially seen in young people;

4 bulging sputum and other nodular necrotizing rash;

5 multiple mononeuritis and unexplained fever. Tissue biopsy has diagnostic significance for this disease, and other tests include angiography and autoantibody testing.

2, skin type allergic vasculitis, symptoms of vasculitis This disease only affects the skin, mostly in young adults. Generally, there are symptoms such as fatigue, joint muscle pain, and a few cases may have irregular fever, but there may be no such symptoms. Skin damage can be pleomorphic, with erythema, nodules, purpura, wheal, blood blisters, papules, necrosis and ulcers. The most common under the knees is the most common lesions in the lower part of the lower leg and the back of the foot. The appearance of more skin lesions is characterized by purpura-like rash, which does not fade. It is due to the infiltration and exudation of inflammatory cells in the blood vessel wall. Therefore, this ecchymosis is high and can be touched. It is the disease. feature. Some begin with subcutaneous nodules, such as soybeans to broad beans and jujube size, and light red has tenderness. Some begin to be completely like purplish-like skin lesions, and some skin lesions begin to change like erysipelas, some resemble plaque-like changes, and some resemble polymorphic erythema. Wind damage, pimples, etc. may be associated with the development of skin lesions. Due to the severe inflammatory reaction, blood blisters, necrosis and ulcers occur on purpura and purpura rash, and some nodular lesions can also cause ulceration and pain. Edema is the weight of the ankle and the back of the foot. It is more obvious in the afternoon, accompanied by two lower limbs soreness.

There are many types of skin lesions, but almost all have purpura or nodules. Pustules can also occur when extraneous multinucleated leukocytes are extravasated to surrounding tissues. Skin lesions can occur in any part, such as the back, upper limbs, buttocks, etc., symmetrically distributed. Skin lesions have conscious pain, itching or burning sensation, and some have no symptoms and tenderness. After the skin lesions, there is pigmentation, and if there is an ulcer, there may be atrophic scars. In the acute onset of the lesion, the lesion appeared in batches, distributed widely, with edema of the calf, and the condition was heavier. Impaired chronic menstruation, repeated episodes last for months or years. The lesions are lighter in 2-4 weeks. Some skin lesions merge with each other and expand into a large lesion. It is more common in the knees, elbows and hands, and it seems to be persistent erythema.

3, systemic allergic vasculitis, symptoms of vasculitis, the disease is affected by multiple organs, the condition is heavier. Diffuse exudation and hemorrhagic lesions are mostly within the organs due to the involvement of small blood vessels in the organs, especially in the posterior capillaries. Most of the organ involvement is acute, usually with headache, irregular fever, discomfort, fatigue, joint and muscle pain. The course of the disease is different, the severity is different. If it is exposed to the antigen once, it will be about 3-4 weeks. If the antigen is repeatedly contacted repeatedly, the disease will recur and the course will last for several months or years. The prognosis of this disease depends on the extent of the affected organs and lesions. Pleomorphic skin lesions occur in 67% of patients, but tend to be palpable. 75% of patients had non-specific fever, and about 2/3 of them had joint pain and joint swelling. The lesion can invade the mucous membrane, causing nasal discharge, hemoptysis, and blood in the stool. One third of patients have kidney involvement, proteinuria and hematuria, and severe renal failure is the leading cause of death. Invasion of the intestine may have abdominal pain, fatty spasm, blood in the stool, acute cholecystitis and other gastrointestinal symptoms. There may be pancreatitis and diabetes.

Chest X-ray showed pneumonia and nodular shadows, pleurisy or pleural effusion. It can invade the nervous system, such as headache, diplopia, delusion, confusion, and even cerebral thrombosis and spasm, difficulty in swallowing, sensory and / or motor dysfunction. Heart damage is myocardial infarction, heart rhythm disorder and pericarditis. Renal cortical ischemia may produce severe hypertension. The most common ocular manifestations of systemic vasculitis are scleral inflammation and retinal hemorrhage. Painful swelling of the epididymis and testicles may be a manifestation of vasculitis. It has been suggested that the diagnosis of systemic vasculitis for renal biopsy, and direct immunofluorescence tests can often help to confirm the diagnosis.

Examine

Vasculitis examination

Blood biochemical examination

Skin type allergic vasculitis generally has no significant changes. Systemic allergic vasculitis may have anemia, temporary reduction of platelets, leukocytosis, 1/3 of patients with eosinophilia, generally 0.04 ~ 0.08, a few up to 0.56. There are proteins and red blood cells in the urine, and occasionally Granular tube type. The heavy BUN can be raised. More than half of patients may have an increase in erythrocyte sedimentation rate. Total complement and complement C3, C4 can be reduced. IgG, IgA content increased, IgM decreased, and its changes were consistent with the disease. The liver function of the patient may be abnormal. The circulating immune complex is positive. The following tests also have implications for the disease, such as antinuclear antibodies, syphilis serum tests, wet factors, cryoglobulin, and HBsAg. Attention should also be paid to potential infections and tumors, while paying attention to the observation of connective tissue diseases.

2. Histopathological examination

The change was mainly based on the severity of the lesion, the length of the disease and the condition at the time. In general, there are inflammatory cell infiltration around the capillaries and small blood vessels of the dermis, and there are many neutrophil infiltration and many scattered nuclear fissures formed by the disintegration of neutrophils, called nuclear dust, and also organized cells and eosinophils. Infiltration, deposition of fibrin bundles with strong eosin staining around the blood vessels. In the section, due to the deposition of fibrin and the obvious edema, the collagen around the blood vessel is obscured, called fibrin-like degeneration. Vascular endothelial cells swell, such as severe, can cause vascular lumen obstruction. Inflammatory cells also invade the blood vessel wall. The blood vessel wall is mainly invaded by neutrophils, making the blood vessel wall unclear. At the same time, there may be eosinophils and a small number of mononuclear cells. Fibrin bundle deposition and vascular necrosis. More red blood cell extravasation is common.

3. Electron microscopy

It can be found that the veins of the capillaries are invaded, especially the blood vessels of 8-30 m in size. In the early stage, vascular endothelial cells were swollen, fissures and phagocytic cells were active between endothelial cells, and the basement membrane was thickened. Neutrophils begin within the interstitium of the blood vessels. Heavy platelets condense in the lumen and pass between endothelial cells.

4. Direct immunofluorescence examination

It can be found that the vascular basement membrane has IgA antibodies, and the dermis and subcutaneous tissues have IgM and IgG antibodies and complement C3 deposition. Medical teaching. The breeding network can find the destruction of tissue components in its fixed parts, mainly found in the fibrin-like necrosis area.

Diagnosis

Vasculitis diagnosis and identification

Differential diagnosis

(1) Allergic purpura occurs in children and adolescents, and most of the skin lesions in the lower extremities with ecchymosis and siltation are associated with joint pain. Platelets are normal, urine can have proteinuria and hematuria, and occasionally gastrointestinal bleeding symptoms.

(2) Young women with papular necrotic tuberculosis are common. There are scattered central necrotic solid papules near the joints of the extremities or the buttocks. There is atrophic scar after the healing. The tuberculin test is strongly positive, and the histopathology has tuberculosis.

(3) skin-type nodular polyarteritis is mostly in the lower limbs, there are subcutaneous nodules distributed along the small arteries, conscious pain and obvious tenderness, skin histopathological manifestations of arteritis and small arterial necrosis.

(4) should also be differentiated from hyperglobulinemia, acute acne-like lichen-like pityriasis and nodular vasculitis.