Systemic lupus erythematosus

Introduction to systemic lupus erythematosus The prevalence of this disease varies among races. Different strains of mice (NEB/NEWF, MRL1/1pr) spontaneously develop SLE symptoms after several months of birth. Family surveys show that first- and second-degree relatives of SLE patients About 10% to 20% may have the same kind of disease, some have hyperglobulinemia, a variety of autoantibodies and T inhibit cell dysfunction. The coincidence rate of single-oval twins is 24% to 57%, while that of double-oval twins is 3% to 9%. HLA typing showed that SLE patients were associated with HLA-B8, -DR2, -DR3, and some patients were associated with complement C2 and C4 defects, and even TNFa polymorphisms were significantly associated; the recent discovery of homozygous C2 gene deficiency, and -DQ The high frequency is closely related to DSLE; T cell receptor (TCR) is also associated with the susceptibility of SLE, and the low level of TNFa may be the genetic basis of lupus nephritis. All of the above tips SL have a genetic predisposition, but according to the Huashan Hospital survey of 100 SLE family members, it is a multi-gene inheritance, and environmental factors also play a significant role. basic knowledge The proportion of illness: 0.001% Susceptible people: good for young women Mode of infection: non-infectious Complications: uremia pneumonia sepsis pyelonephritis

Causes of systemic lupus erythematosus

Causes

The cause of this disease is unknown. Recent studies have confirmed that this disease is characterized by various immune response abnormalities. As for the factors that cause immune disorders, it may be multifaceted.

Systemic lupus erythematosus prevention

Population prevention

(1) Strengthening census and publicity: Strengthening the investigation of epidemiology of autoimmune diseases, establishing specialist counseling clinics, organizing patient associations, and discovering patients in time.

(2) Improve the level of diagnosis and treatment of medical staff: With the development of immunology and other basic medicines, the research on autoimmune diseases has also developed accordingly. Therefore, strengthening theoretical study and continuously improving the level of diagnosis and treatment of such diseases by medical personnel is Early detection and important measures for timely treatment.

(3) Laboratory testing techniques for developing and gradually popularizing autoimmune diseases: The histopathological changes of some autoimmune diseases are already obvious, but there is no typical clinical manifestation, so assistance with immunoassay techniques is needed, including detection of certain Autoimmune diseases have antibodies of particular diagnostic significance.

Personal prevention

(1) Primary prevention: Try to avoid inducing immune response factors, which is the key to preventing autoimmune diseases.

1 Maintain optimism: Modern immunological studies have shown that the body's immune function is also regulated by neurological and endocrine factors, so maintaining an optimistic mood is important to maintain the body's normal immune regulation.

2 adhere to exercise: physical exercise or participate in appropriate physical labor is an important way to enhance physical fitness and improve the body's ability to resist disease. Physical exercise can enhance the body's adaptability to external environmental changes, reduce the chance of disease, such as doing exercises, practicing Qigong, Take a walk, play Tai Chi, warm water or warm water bath.

3 Combination of work and rest: Traditional Chinese medicine has always advocated that there is a diet and a regular life. It is a major measure to keep fit and exercise. The rules of life, moderation and diet, moderate work and rest, can maintain energy and strength, avoid external causes. Invasion of disease factors.

4 to avoid predisposing factors: for example, when the summer comes, direct sunlight, it is easy to aggravate the skin damage of SLE patients, and even cause fluctuations in the whole body condition. Exposure and fatigue often make the condition change drastically, brain and heart damage occur, and can be applied when needed. Anti-radiation drugs such as 3% quinine ointment, compound titanium dioxide ointment, 10% ammonia benzoic acid ointment, etc., other exposures such as cold, X-ray, etc., can also cause the disease to intensify, can not be ignored.

5 prevention of infection: prevention of streptococcal infection is an important part of reducing the incidence of autoimmune rheumatic diseases, patients often show symptoms of respiratory infections such as sore throat, cough, etc., can be treated with appropriate antibiotics, should cure infections, such as chronic tonsillitis , sinusitis, chronic otitis media, dental caries and so on.

6 Avoiding drug abuse: At present, the number of iatrogenic diseases has increased, which has caused widespread concern in the medical community. Since many pharmacological pharmacological effects are not fully understood, drug abuse can easily cause substances with hapten properties to enter the body. Causes immunopathological damage, produces various autoimmune diseases, such as penicillin, penicillamine, chlorpromazine, contraceptives, etc., easily induces SLE, while hydralazine antihypertensive drugs, anticonvulsants such as phenytoin, proca Because of amines, isoniazid, etc., after long-term use of these drugs, symptoms similar to SLE can occur, and autoantibodies can be found in the body, but the symptoms can resolve spontaneously shortly after stopping the drug.

(2) Secondary prevention:

1 early diagnosis: early manifestations of SLE, repeated intermittent fever, joint swelling and pain, fatigue, weakness, serological examination of gamma globulin increased, or detection of autoantibodies, such patients should be alert to autoimmune diseases, further examination is required, also Try to use drugs such as lymphokines that increase cellular immune function, such as transfer factors and interferons, to achieve inhibition, eliminate the role of antigens, and perform diagnostic treatment.

2 early treatment:

(3) Level 3 prevention:

1 patients should be birth control, avoid pregnancy: pregnancy and childbirth can induce systemic lupus erythematosus, is also an important factor to aggravate the condition, and the chance of spontaneous abortion and stillbirth is several times higher than normal.

2 adhere to treatment: especially for the use of hormones and immune modulators should be reviewed regularly.

3 TCM syndrome differentiation: See the Treatment section.

Systemic lupus erythematosus complications Complications uremia pneumonia sepsis pyelonephritis

1. Allergies: This disease is prone to drug allergy, and the performance is heavier. Once allergic, it is not easy to reverse or the condition deteriorates. Sometimes the disease is in a relatively stable period, and the sensitized drugs can cause acute exacerbations such as persistent high fever. Commonly used drugs that cause allergies are: penicillins, cephalosporins, sulfonamides, estrogens, procainamide, phenytoin, etc., so lupus patients disable the above drugs.

Some cases are prone to allergies to some foods. The harm to the disease is basically similar to the above drugs, especially for animal meats, such as dog meat, horse meat, and mutton. Therefore, patients should be cautioned to avoid worsening the disease.

2. Infection: It is a common complication, and it is also the most common cause of death and the main cause of deterioration of the disease. The cause of infection is related to long-term immunosuppressive agents, uremia and low immune function of the disease itself. Pneumonia, pyelonephritis and sepsis are The most common complications, the pathogens can be Staphylococcus aureus, Nocardia, Escherichia coli, Proteus, tuberculosis, cryptococci and viruses.

Systemic lupus erythematosus symptoms Common symptoms Blood abnormalities Thrombocytopenia Skin purpura Kidney damage Fatigue Light allergy Wind scaly hyperthermia

Common ones are as follows:

(1) Vascular lesions: Necrotic vasculitis manifested as small blood vessels (small arteries or arterioles). Immunofluorescence has DNA, C 3 and immunoglobulin deposits on the vessel wall.

(2) Skin pathological changes: Skin pathological changes are epidermal atrophy, basal cell or dermis liquefaction denaturation or necrosis, with edema of the dermis and epidermal junction. Immunofluorescence showed deposition of immunoglobulin, (IgG), (IgM), (IgA) and complement C 3 , C 4 , C 1 q at the junction of epidermis and dermis.

(3) Renal lesions: Patients with systemic lupus erythematosus with abnormal renal and urinary examinations have 100% renal lesions and glomeruli, renal tubules, interstitial and blood vessels when performing biopsy and immunofluorescence. The characteristic change is that the hematoxylin and the glomerular basement membrane change in a coil. Immunofluorescence showed a "full house red" phenomenon, (multiple immunoglobulins and complement deposition were seen in the glomerular tubules and stroma).

(4) Heart changes: Half of patients with systemic lupus erythematosus involve the heart, including pericarditis, myocarditis, heart valves, and endocardial lesions. Mainly manifested as non-bacterial spastic endocarditis or Libran - Sacks endocarditis.

(5) Others: There are fibrin-like substances deposited in the synovial membrane and serosa, with cell proliferation and small vessel cellulose necrosis. Neurological disorders include diffuse neurocytopenia, small vasculitis, microinfarction, necrosis, and hemorrhage. The pathological changes of the lungs are more common with interstitial pneumonia and diffuse pulmonary fibrosis, and there are coil-like lesions in the pulmonary capillaries.

Systemic lupus erythematosus examination

1. Blood routine and erythrocyte sedimentation rate: The most common are different degrees of anemia, mostly positive cell pigmented anemia, a small number of hemolytic anemia, anti-erythrocyte antibodies, about 15% of patients with positive Coomb test, white blood cells more (2.0 4.0)×109/L, in which neutrophils or lymphocytes are decreased, and lymphocyte reduction is significantly related to the activity of SLE. When there is secondary infection, white blood cells can be elevated. If there is no infection, no white blood cells are even high fever. Elevated, 1/3 of patients have mild thrombocytopenia, and the erythrocyte sedimentation rate increases during active period.

2. Urine routine: There may be different degrees of proteinuria, hematuria and tubular urine or pyuria.

3. Biochemical examination: there may be elevated transaminase and abnormal turbidity test. When there is renal insufficiency, BUN and creatinine may increase. However, protein electrophoresis indicates that gamma globulin is elevated. This change is usually related to disease activity. Parallel can be reduced as the condition improves, so it can also be used as an indicator of dynamic observation of the condition.

4. Immunological examination

(1) Lupus cells: LE cells, because the anti-nuclear protein antibodies present in the serum of patients act on the damaged nuclei, causing the nuclear proteins to change, forming a circular, unstructured uniform body, which is The phagocytic phagocytosis forms LE cells, which are stained with Wright. It can be seen that a large unstructured smog-like purple-red uniform body is contained in a large granulocyte, and the nucleus of the neutrophil is squeezed aside. If the homogenate is not phagocytized and surrounded by many neutrophils, a so-called "rosette" can be formed. The test method is to take the patient's blood to make it coagulate, and keep it at 37 ° C for 2 h, mashing. Blood clots, sedimentation by centrifugation, taking the pale yellow upper white blood cell smear for Wright's staining, and examining under the light microscope, the positive rate of this method is 50%-80%, and the positive rate is related to the examination time, the number of times, whether it is treated, etc. In the active stage and late stage, the positive rate is high, it should be carried out several times, even dozens of examinations can be found to find LE cells. LE cells are not specific for the diagnosis of SLE, but also can be seen in dermatomyositis, scleroderma, Rheumatoid arthritis Acute leukemia, due to the complicated method, by checking the influence of drugs and affect individual people and positive rate is low, more and more people are not used by the current use of simple and reliable antinuclear antibody Instead, both have the same clinical significance.

(2) Antinuclear antibody spectrum:

1 anti-nuclear antibody (ANA): is a general term for antibodies to various nuclear components, SLE ANA positive rate of up to 95%, its titer is higher, ANA greater than 1:80 has a great diagnostic significance for SLE, ANA to SLE also No specificity, can also be seen in other connective tissue diseases, such as scleroderma, Sjogren's syndrome, etc., but its positive rate and titer is low, peripheral and homogenous types are more common in SLE, have a certain diagnostic significance.

2 anti-dsDNA antibody, high specificity for SLE, the positive rate is 50% to 80%, and the antibody titer decreases with the remission of the disease.

3 anti-Sm antibody, high specificity, the positive rate of SLE patients is 20% to 30%, this antibody has nothing to do with SLE activity.

4 other autoantibodies, anti-RNP antibodies, anti-SSA antibodies, anti-SSB antibodies, anti-histone antibodies, etc., can be positive, a variety of autoantibodies can occur in SLE.

(3) Antiphospholipid antibodies include anticardiolipin antibodies, lupus anticoagulants, and biological false positive reactants (ie, false positive syphilis assays), all of which are directed against substantially the same phospholipid antigen, and antibodies inhibit hemagglutination in blood vessels, However, patients with this antibody not only have no bleeding tendency, but are prone to thrombosis of arteries and veins. In addition, habitual abortion and thrombocytopenia often occur, and the above symptoms and antiphospholipid antibodies together constitute an antiphospholipid syndrome.

(4) SLE other autoantibodies: antibodies against erythrocyte membrane antigens are positive for anti-human globulin test, anti-granulocyte antibodies, anti-platelet antibodies and anti-lymphocyte antibodies, antibodies to Golgi, ribosomes, for cell framework Ingredients, microfiber and other antibodies, about 1/3 of cases are positive for rheumatoid factor.

(5) Immunoglobulin: Most patients have elevated IgG and IgM, which is caused by the presence of various autoantibodies in the body.

(6) Complement: Half of the patients have hypocomplementemia, serum total complement (CH50), and decreased C3 content, which may indirectly reflect the increase of circulating immune complexes, which is related to the disease activity. The increase of complement decomposition products C3a and C5a also represents the disease activity. There are many methods for detecting circulating immune complexes, but none of them is satisfactory.

5. Immunopathological examination

(1) Renal biopsy: The pathological diagnosis provided by this method is valuable for the treatment of lupus nephritis and prognosis. It should include at least light microscopy, fluoroscopy, electron microscopy if necessary, and identification by immunohistochemistry. The subtype of lymphocytes and the composition of the sediment.

(2) Skin lupus belt test: The patient's skin was examined by direct immunofluorescence technique, and a localized immunoglobulin precipitation band appeared at the junction of epidermis and dermis, which was yellow-green and immunoglobulin (mainly IgG) and complement. The conjugate of the epidermal dermal junction, the positive rate of SLE patients is 50% to 70%. If the material is taken from the lesion site, all kinds of skin diseases are positive, so the normal skin should be taken from the exposed site to improve the specificity of the test. Sexuality, in the treatment, the immunofluorescence band can disappear with the relief of the disease, so this can be used as one of the indicators to judge the efficacy.

6. X-ray examination: Chest X-ray examination is mainly based on interstitial pneumonia. There are small inflammatory shadows and discoid atelectasis in both lungs, pleural effusion, etc., and general heart enlargement caused by myocarditis. And pericardial effusion.

Diagnosis and diagnosis of systemic lupus erythematosus

The disease should be associated with other connective tissue diseases, bacterial or viral infectious diseases, histiocytosis X, malignant reticuloendotheliosis, thrombocytopenia, hemolytic anemia, various types of kidney disease, hepatitis, myocardial-pericardium Inflammation, differentiation of nervous system diseases. Especially need to identify with lupus syndrome and neonatal lupus syndrome.

Lupus syndrome

The most common of these is systemic lupus erythematosus caused by drugs. This syndrome can be seen in some symptoms and signs similar to SLE, and laboratory results, sometimes difficult to distinguish. The following conditions are helpful in identifying: taking medication history, gender differences are not obvious, clinical symptoms are mild, visceral involvement, kidney disease, butterfly erythema, mouth ulcers, hair loss, and white blood cells, thrombocytopenia, and hypocomplementemia are rare. Anti-Sm antibody and anti-n-DNA (FARR) antibody were negative. The most important feature is that the clinical symptoms and laboratory signs disappear after stopping the drug, and then reappear when the drug is used. Sometimes antinuclear antibodies exist for a longer period of time and generally have a good prognosis.

Neonatal lupus erythematosus syndrome

The disease is seen in infants under 6 months. Most of the mothers of the children suffer from SLE or other connective tissue diseases, and there are RO antigen (Sjogren's syndrome A antigen) and La antigen (Sjogren's syndrome B antigen) in the serum. Children with symptoms after birth, mainly for congenital conduction block, lupus-like dermatitis, auto-hemolytic anemia, RO and La antigen positive in vivo. In addition, often accompanied by congenital heart disease, various defects and endocardial fibroelastosis, white blood cells and thrombocytopenia. The typical manifestations of lesions are scaly and ring-shaped erythema, which are found in the exposed parts, ie at the top of the head, neck and eyelids, and appear to be discoid erythema. The disease is a self-limiting disease, the blood abnormalities are improved within 6 weeks, and the skin lesions can disappear within 6 months. In addition to children with heart disease, the general prognosis is good. It is not known why puberty can become a SLE.

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