Moderator (continued by Qingping): What is systemic lupus erythematosus? What are its pathogenetic characteristics? Liu Xiangyuan: (1) Systemic lupus erythematosus (which sounds scary) is translated from the Western medical Latin – lupus, which has two meanings, firstly, the rash on the face is similar to the facial scar of a wolf bite in a fight. The rash on the face is similar to the facial scar of a wolf bite. The second meaning is that lupus is as cunning as wolves, with dangerous attacks, easy to recur, and persistent and unpredictable. (2) In fact, “lupus erythematosus” is not only skin damage, but also various organs of the body such as the brain, heart, lungs, joints, kidneys, blood and muscles can be involved, and many autoantibodies (rheumatic immune diseases with the most autoantibodies) appear in the blood. (3) The onset of the disease is characterized by the prevalence of young women, and the more aggressive and beautiful women seem to be the more likely to get it (competitive, sentimental, and love to take the bull by the horns, customarily called lupus character). It is much more common in women than in men, with women being 7 to 10 times more likely than men. It manifests as unexplained joint pain (especially in the finger joints, sometimes confused with rheumatoid), fever, mouth ulcers, hair loss, photosensitivity, and foam in the urine. Some people have a history of trauma or hair dye before the onset of the disease, etc. Because of the involvement of various organs, often with a certain organ damage as the main focus, they are often admitted to other departments, such as abdominal pain and diarrhea to the gastroenterology department, headache and epilepsy to the neurology department, cough and breathing difficulties to the respiratory department, etc. It is easy to misdiagnose and miss the diagnosis. Moderator (Qingping): What is the prevalence and treatment of lupus in China? Liu Xiangyuan: (1) Epidemiological surveys show that the prevalence of SLE in China is about 7/100,000, i.e. 7 out of 100,000 people, while among female patients it is close to 1 in 1000, with one lady in 1000 people. According to the above estimates, the number of SLE patients in China exceeds 1 million, which is the highest in the world. (2) Patients with SLE are no longer considered incurable; rather, it is a chronic lifelong disease similar to hypertension and diabetes. Since the 1980s, the proportion of SLE patients diagnosed early has greatly increased, and the life expectancy of patients has greatly approached that of the normal population, with the 10-year survival rate increasing from less than 10% to more than 90%. Moderator (Qingping): Are there many types of lupus erythematosus? Liu Xiangyuan: (1) Yes, lupus erythematosus can be divided into many types, similar to the spectrum, and is a spectrum disease, with the mildest end being limited discoid lupus erythematosus and the heaviest end being systemic lupus erythematosus, with many subtypes including disseminated discoid lupus erythematosus, subacute cutaneous lupus erythematosus, profound lupus erythematosus (lupus lipofuscinosis), and ANA-negative systemic lupus erythematosus. SLE can be further classified as lupus nephritis, neuropsychiatric lupus, lupus pneumonia, lupus myocarditis, and lupus hepatitis according to the different organ tissues involved. (2) The disease, treatment and prognosis of each type of lupus are different, except for SLE, which is more severe and has a poorer prognosis, but the rest are better; (3) Each type of lupus can be transformed into each other, for example, 6.5% of limited discoid lupus with abnormal serology are transformed into SLE, while 22% of disseminated discoid lupus are transformed into SLE. Moderator (Qingping Xuan): What are the causes of SLE? What is the pathogenesis? Xiang-Yuan Liu: The etiology and pathogenesis of SLE are not yet clear. Current research suggests that it is related to both intrinsic factors such as genetics and sex hormones, as well as environmental factors and drugs. The interaction of various factors, such as genetic quality, environmental factors and estrogen level, leads to the reduction of T suppressor cell function and excessive activation of B cells, resulting in the production of a large number of autoantibodies, which combine with self-antigens in the body to form corresponding immune complexes and precipitate in various parts of the body, causing acute and chronic inflammation and tissue necrosis (e.g. lupus nephritis) with the participation of complement, or antibodies acting directly with tissue cell antigens to (e.g. antigens on red blood cells and platelets combine with autoantibodies to cause hemolytic anemia and thrombocytopenia, respectively), resulting in multi-system damage to the body. Moderator (Qingping Continued): What factors are related to the development of SLE? Xiang-Yuan Liu: (1) Environmental factors: For example, sunlight (ultraviolet light) exposure and hair dyeing can aggravate lupus erythematosus or make it more active; women with high IQ and high work pressure are more common among lupus erythematosus patients, etc., which indicate that the environment plays a role in the development of lupus erythematosus; (2) High level of estrogen: women of childbearing age with the highest secretion of estrogen are the most common. (3) Genetic factors: Lupus patients may carry certain lupus-causing genes on their chromosomes and inherit them to the next generation. It is more common for identical twins to have lupus at the same time. (4) Certain drugs (accounting for 10%), such as the anti-arrhythmic drug procainamide, the antihypertensive drug hydrazidazine, the anti-tuberculosis drug isoniazid, hair dyes, smoking and foods such as celery, mushrooms and bean sprouts, are related to the onset of lupus erythematosus. Therefore, it is important to maintain good work and living habits and actively guard against infections in order to avoid certain damage caused by lupus erythematosus to oneself. Moderator (Continued by Qingping): Is SLE hereditary? How big a role does genetic factor play in SLE? (1) Lupus is caused by immune abnormalities after the interaction of genetic factors (about 20%) and environmental factors (about 80%), i.e. those with lupus genetic factors will trigger the disease once they encounter certain precipitating conditions; (2) Lupus is not a genetic disease (the so-called genetic diseases mainly refer to monogenic genetic diseases, i.e. genetic diseases controlled by a pair of alleles, including red-green blindness, hemophilia and (3) Evidence of genetic predisposition includes: (a) blacks and Asians have lupus more often than whites; (b) one of the identical twins has the disease and the other one has the disease with a probability of 25%-70%, while the probability of heterozygotic twins is 5%; (c) first-degree relatives of lupus patients have the disease with a probability of 1%-16%; (d) there are more than two lupus patients in the family, and the relationship between them is The most frequent relationship is between mother and daughter, and the others are sisters, brothers and fathers in order. (4) Its susceptibility is determined by several genes: including DR2 and DR3, the leukocyte antigen (HLA)-II class molecules on the short arm of human chromosome 6. Moderator (continued by Qingping): How harmful is SLE? Liu Xiangyuan: It is very harmful and can involve all tissues and organs of the body, and can be life-threatening in serious cases. (1) Affecting the image of patients: On the one hand, if rashes and hair loss appear on the face due to the activity of the disease, it is a big blow to women who love beauty; on the other hand, patients often have fat faces due to taking hormones, the so-called full-moon faces and buffalo backs, and patients are reluctant to see others. (2) Affecting the quality of life: during the active period, there are often fever, mental depression, weakness, joint pain, muscle pain, Raynaud’s phenomenon, mouth ulcers and unthinking diet, etc.; (3) Different manifestations of different organ involvement: for example, headache, epilepsy, convulsions and mental abnormalities in the nervous system, protein and blood in the urine in the kidney involvement, abdominal pain and diarrhea, nausea and vomiting in the digestive system involvement, etc. Moderator (Qingping Xuan): What are the early symptoms of SLE? What are the characteristic manifestations? Liu Xiangyuan: (1) Early manifestations are not characteristic. (2) Most of the symptoms occur in spring, and the first symptoms are mainly arthralgia, rash and systemic manifestations; (3) Joint symptoms are mainly pain and mild swelling in small joints of both hands, similar to rheumatoid, but of short duration, without deformity and bone destruction; (4) The rash is mostly bright red butterfly-shaped erythema on the face (cheeks and nasal bridge on both sides, but the nasolabial folds are not involved, with clear edges and a butterfly-like appearance) and rash. (4) The rash is mostly bright red butterfly-shaped erythema on the face (both cheeks and nasal bridge, but the nasolabial folds are not involved, the edges are clear, and the shape is like a butterfly) and frostbite rash on the back of both hands (polymorphic erythema), which is also more characteristic. The former rash is tender at the edges, mostly without pruritus, and deepens in color and becomes more edematous after sun exposure. In contrast, allergic dermatosis does not involve the nasal bridge, and the erythema has no tenderness at the edges, and there is significant itching. The frostbite like rash on the backs of both hands is symmetrically distributed edematous erythema, not ulcerated, not itchy, but with burning pain, and exists all year round, while frostbite occurs in winter, often ulcerated, with obvious itching. (5) Systemic symptoms: such as low-grade fever, hair loss, fatigue and anemia. (6) There may be manifestations of multiple organ damage: blood cells, liver, kidneys, heart, brain tissue, etc. Immunological tests including anti-nuclear antibodies, double-stranded DNA antibodies, anti-ENA antibodies and complement should be done for suspected cases. Moderator (Continued Qingping): What tests should be done for SLE? Liu Xiangyuan: (1) Routine examination: blood and urine routine, liver and kidney function, blood sedimentation and C-reactive protein. (2) Immunological examination: anti-nuclear antibody, anti-double-stranded DNA antibody (associated with disease activity and renal damage), anti-Sm antibody (high specificity, a marker antibody), anti-ribosomal P antibody (high specificity, a marker antibody), anti-nucleosome antibody (associated with disease activity), immunoglobulin and complement level, etc. (3) Additional items should be added according to different organ involvement and drug use: for example, 24h urine should be kept for protein quantification and creatinine clearance for those with kidney damage, and blood lipids, electrolytes and blood glucose should be checked for long-term hormone use. For long-term use of hydroxychloroquine, regular electrocardiogram and fundus examination should be performed. Moderator (Qingping): What criteria do doctors use to diagnose lupus erythematosus? Xiang-Yuan Liu: Lupus is generally diagnosed according to the American or international classification and diagnostic criteria, such as the classification and diagnostic manifestations of lupus proposed by the American College of Rheumatology in 1997, which has a total of 11 articles, of which 4 or more can be diagnosed with a sensitivity and specificity of 96%. These include (1) zygomatic erythema; (2) discoid erythema; (3) photosensitivity; (4) oral ulcers; (5) non-erosive arthritis, (6) plasmacytosis; (7) renal lesions: proteinuria >0.5 g/dl or 3+; cellular tubularity, which may be erythrocytic, hemoglobin, granular tubular, or mixed tubularity. (8) Neurological abnormalities: convulsions and psychosis (exclude drugs or metabolic disorders such as those caused by uremia or electrolyte disturbances); (9) Hematologic abnormalities: hemolytic anemia with reticulocytosis; leukocytes <4×109/L, at least 2 times; lymphocytes <1.5×109/L, at least 2 times, thrombocytopenia <100×109/L (exclude drug effects); (10) Immunologic abnormalities: antiphospholipid antibodies; positive anti-double-stranded DNA antibodies; positive anti-Sm antibodies; false-positive syphilis serologic test (11) positive anti-nuclear antibodies. 2009 saw the promulgation of a new international diagnostic criteria for lupus, with two conditions for diagnosis. the first condition is confirmed by renal pathology confirming the presence of lupus nephritis plus positive ANA or anti-ds-DNA; the other condition is ≥4 of the following indicators (including at least 1 clinical and 1 immunological): 11 clinical conditions, with non-scarring alopecia in the first 8 of the above replacing photosensitivity, plus three in hematology. And immunological abnormalities were 6, for the above 11 + 3 of 10 + reduced complement + positive Coom'b test. Hematologic and immunologic abnormalities and renal biopsy were emphasized. Moderator (Continued Qingping): What are the treatment options? Liu Xiangyuan: It is divided into general treatment and drug treatment: (1) General treatment: psychological and spiritual support, avoidance of sunlight or ultraviolet radiation, prevention and control of infection or other comorbidities and selection of appropriate exercise according to the condition. (2) Drug therapy: (1) non-steroidal anti-inflammatory drugs: for those with low fever, joint symptoms, rash and pericardial and pleurisy, and caution for those with hematologic lesions. (2) Anti-malarial drug hydroxychloroquine, which is a basic drug, effective for rash, low fever, arthritis, mild pleuritis and pericarditis, mild anemia and blood leukocyte count reduction and combined with dry syndrome, use with caution if there is ophthalmia. Long-term application is useful for reducing hormone dose, lowering blood glucose and lipids, and maintaining remission to prevent relapse. The main adverse reactions are cardiac conduction disorders and retinal pigmentation, and electrocardiogram and ophthalmic examination should be performed regularly. (3) Glucocorticosteroids: choose different doses and dosage forms according to the condition: take prednisone as an example, small dose is suitable for active SLE patients without important organ damage; medium dose is suitable for those with high fever or mild damage to one important organ; large dose is suitable for those with malignant high fever or serious damage to one or more important organs, take it in divided doses if there is fever, take it in the morning if there is no fever, and gradually reduce the dose after the condition is stabilized. The dose should be gradually reduced for maintenance. For patients with severe disease, mega-dose shock therapy can be used, usually methylprednisolone is used for 3-5 days, and then changed to regular amount of hormone, which can be repeated if necessary. (4) Immunosuppressants: cyclophosphamide (CTX), morte-macrolide, azathioprine, methotrexate, cyclosporine A, vincristine; 5. Other: high-dose immunoglobulin shock, plasma replacement and mesenchymal stem cell infusion: for patients with severe disease, who cannot be controlled or tolerated by conventional treatment, or who have contraindications Moderator (continued Qingping): When you have SLE, what are your life and diet What should be noted? Xiang-Yuan Liu: (1) Attitude: build up confidence, do not listen to biased beliefs, keep a happy mood, avoid emotional ups and downs and sentimentality, engage in recreational activities, but do not be exhausted, do not blindly believe in charlatans, go to regular hospitals for treatment and follow medical advice. (2) rest and activity problems: rest more during the active period, ensure 8-10h sleep at night, need lunch break every day, gradually increase the amount of activity as the condition improves, to the extent that you do not feel tired; (3) makeup and sun exposure problems: avoid using cosmetics containing aromatic amines, avoid hair dyeing, eyebrow tattooing or breast augmentation, avoid over-decorating houses, and ventilate for a long enough time. Avoid exposure to daylight from 11:00 p.m. to 3:00 p.m.; wear protective hats, long-sleeved clothes, put UV filters in windows (most patients are allergic to UVB, some are allergic to UVA and even visible light; glass can protect those allergic to UVB, but those allergic to UVA are only partially protected, and UVB and UVA1 can cause cutaneous lupus), and use broad-spectrum sunscreen that protects against both UVA and UVB. Sunscreen (UVB index SPF≥50, UVA index PA (++)~(++++), sunscreen should contain physical sunscreen ingredients such as titanium dioxide, zinc oxide. At least 20min before the sun to wipe a sufficient amount, sun longer or wet, should repeat wipe, sunscreen duration is SPF multiplied by 15 ~ 20 minutes, skin care products with mild non-irritating, non-allergenic, moisturizing function-based, active period without makeup, stable condition with medicinal products (both cosmetics and topical drug characteristics). (4) dietary issues: (a) general principles: high protein, low fat, low salt, low sugar, rich in vitamins and calcium, no smoking and no alcohol, smoking increases damage to the blood vessel wall and reduces the efficacy of hydroxychloroquine, drinking alcohol mainly due to the interaction with certain liver-damaging drugs lead to liver damage; (b) adjust according to organ involvement and other manifestations: the appearance of hair loss do not eat cauliflower (aggravate the process of hair loss?) ; allergic to a certain food or drug to avoid the application; have light allergy to avoid with may increase photosensitive food or drugs such as mud snail, parsley, celery, figs, mushrooms and smoked food, Western drugs such as sulfonamides, tetracyclines, hydrochlorothiazide and estrogen, and Chinese medicine containing osteopontin. The former contains 20-carboxy-5enoic acid metabolized into prostacyclin, which inhibits platelet agglutination and aggravates bleeding, and the latter contains histidine, which produces histamine, and histamine clearance depends on monoamine oxidase (which is inhibited by isoniazid), and excessive histamine produces allergic reactions. If there is severe proteinuria due to renal damage and no renal failure, high protein diet should be consumed without spinach (because it contains oxalate, which can increase proteinuria and tubular urine); if there is renal failure, protein intake must be limited, mainly animal high quality protein supplementation, less beans and soy products; swelling is obvious, high blood pressure and low urine volume limit water and salt; (c) according to the drugs taken: long-term use of hormones should pay attention to control fat intake. Eat more cucumbers and tomatoes, and limit staple foods and sweets. (d) According to the view of Chinese medicine: lupus is mostly Yin deficiency and internal heat, so do not eat seafood (hairy food), mutton, dog meat, venison, cinnamon and spicy food, etc. Moderator (Qingping): Can lupus erythematosus be cured? Liu Xiangyuan: It can't be cured, mainly because lupus erythematosus can be controlled more stably, but it can't be cured completely. Lupus erythematosus is an autoimmune disease, because there are too many causes, not caused by a single factor, so it can't be cured, but it can be controlled not to develop, and under the condition of taking a small amount of drugs to maintain not to develop, it can dance with wolves and live with the disease for a long time. Moderator (Continued Qingping): Is SLE very easy to relapse? What are the manifestations of relapse? What are the recurrence factors? Liu Xiangyuan: Yes, it is easy to relapse. Relapse manifestations: ① Fever of unknown cause, i.e., cannot be explained by cold, pharyngeal, pulmonary and urinary tract infections, etc.; ⑦ Re-appearance of new rash or vasculitis-like rash at the ends of fingers (toes) or other parts of the body; ③ Re-occurrence of joint swelling and pain; ④ Obvious hair loss, excluding hormonal causes; ⑤ Fresh ulcers in the mouth and nose; ⑥ Appearance of pleural fluid or pericardial effusion, ⑦ Increased proteinuria; ⑧ Leukocytopenia or thrombocytopenia or anemia (9) significant; (10) neurological symptoms, such as headache, vomiting, and convulsions; (11) increased titer of anti-double-stranded DNA antibodies; (12) increased sedimentation of more than 50 mm/hour; (13) decreased complement, especially decreased complement C3. Recurrence factors are mainly the following: (1) exposure and ultraviolet radiation: when it is really difficult to avoid, sunshades should be used for activities in the sun, or wear a wide-brimmed hat, long-sleeved clothes, long pants, and sunscreen on the skin. (2) Drug-induced: such as sulfonamides, botaxone, hydrazinepyridazine, procainamide, chlorpromazine, phenytoin sodium, isoniazid, oral contraceptives, etc., can make patients with lupus in remission enter the active phase. (3) Cold, flu and infection: avoid going to crowded public places as much as possible; those in stable stage can be vaccinated (not suitable for active stage), such as influenza virus and pneumococcal vaccination once a year, etc. Usually wash your mouth diligently, change your toothbrush regularly, wash your vulva diligently, rinse with furacilin or alkaline solution, change your underwear diligently, and disinfect your underwear often under sunlight. Do not eat unhygienic food. Seek medical attention promptly when various infections occur. (4) Pregnancy and childbirth: Pregnancy has a great impact on lupus erythematosus, and more than half of the patients have aggravation or relapse of the disease in the last trimester of pregnancy and several months after delivery. The most serious one is kidney damage. Pregnancy and contraceptive use should not be used during the first 2 years of the disease (especially if the disease is unstable, has antiphospholipid syndrome or nephrotic syndrome, has a history of hypercoagulability or thrombosis), and pregnancy should not be avoided if the disease is unstable or has not been stabilized for a long time, especially if lupus nephritis is present. If pregnancy is planned, immunosuppressants such as cyclophosphamide and mycophenolate should be avoided, and azathioprine <2 mg/(kg.d) should be used if necessary, and functional mercaptopurine methyltransferase should be measured before use. The timing of pregnancy is when there is no significant organ involvement (Cr <2mg/dl, urine protein <0.5g/d), the disease has been controlled for more than 1 to 3 years (at least 6 months), the amount of hormones is small (e.g. prednisone <15mg/d) and no immunosuppression is used, as prednisone ≥20mg/d increases the risk of eclampsia and gestational diabetes. Pre-pregnancy testing for ANA, anti-ds-DNA, anti-SSA and SSB antibodies, lupus anticoagulant, C3, C4, CH50, blood electrolytes, liver function, blood and urine count, creatinine clearance, 24h total protein and calcium, anti-platelet antibodies and antiphospholipid antibodies in case of reduced platelets. Lupus patients are prone to miscarriage in the first three months of pregnancy, so they should insist on taking medication and avoid trauma and injury; they are prone to aggravation in the second three months of pregnancy and after delivery (about 50%), so they should be closely monitored. Frequency of follow-up for pregnant women with lupus: once every 4-6 weeks in the first 20 weeks of pregnancy, once every 2 weeks from 20 to 28 weeks of pregnancy, and once a week after 28 weeks of pregnancy, with follow-up of changes in disease, physical examination, blood routine, blood biochemistry, urine routine, anti-ds-DNA antibody, complement C3 and C4, CH50, uric acid and anti-cardiolipin antibody, etc. (5) Other: sudden discontinuation or rapid reduction of hormones; seeking medical help everywhere, taking drugs indiscriminately, stopping the use of drugs that can control the disease; overwork and little rest; irregular life; drastic emotional fluctuations. Moderator (Qingping Continued): What are the preventive methods for SLE? Xiang-Yuan Liu: (1) Prevent the above recurrence factors, such as preventing sun exposure, not dyeing hair, preventing cold and cold, fatigue, excessive intercourse, abortion, childbirth, trauma, mental stimulation, etc. (2) Eat a light diet, avoid tobacco, alcohol, spicy and stimulating food, greasy and fried food, milk and its products can be skimmed milk instead of whole milk. Avoid eating fava beans and other beans because they contain saboura ammonia, which can contribute to the deterioration of lupus, replenish calcium, take fish (except for those with reduced platelet bleeding), fish oil, vitamins, and eat more fruits and vegetables, (3) increase physical exercise appropriately. Physical exercise, abstain from daily sex life, which is conducive to the treatment of the disease.