1. What is systemic lupus erythematosus? What are its pathogenetic characteristics?
(1) Systemic lupus erythematosus (which sounds frightening) is translated from Western medical Latin – lupus, which has two meanings. Firstly, as the name suggests, it is more graphic, and the facial rash is similar to the facial scar of a wolf’s bite during a fight, where the wolf often tears the other side’s face with sharp teeth after biting it, and bites the face into a bloody mess. A large red scar is formed, with a depression in the middle and raised edges. 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, they often have a certain kind of organ damage as the main cause and are often admitted to other departments, such as those with abdominal pain and diarrhea to gastroenterology, those with headache and epilepsy to neurology, and those with cough and breathing difficulties to respiratory medicine, etc. It is easy to misdiagnose and miss diagnosis.
2. What is the disease and treatment situation of lupus in China?
(1) Epidemiological surveys show that the prevalence of SLE in China is about 7/100,000, i.e., 7 out of 100,000 people, and among female patients, it is close to 1/1000, with one woman 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 is much closer to that of the normal population, with the 10-year survival rate increasing from less than 10% to more than 90%.
3. Are there many types of SLE?
(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, the heaviest end being systemic lupus erythematosus, and the middle including disseminated discoid lupus erythematosus, subacute cutaneous lupus erythematosus, profound lupus erythematosus (lupus lipofuscinosis), ANA-negative systemic lupus erythematosus, and many other subtypes. 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 systemic lupus erythematosus, while 22% of disseminated discoid lupus erythematosus are transformed into systemic lupus erythematosus.
4. What are the causes of SLE? What is the pathogenesis?
The etiology and pathogenesis of SLE are not yet clear. Current research suggests that it is related to 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, which produce a large number of autoantibodies and combine with self-antigens in the body to form corresponding immune complexes, which are deposited in various parts of the body and cause acute and chronic inflammation and tissue necrosis with the participation of complement (e.g. lupus nephritis), or the antibodies act directly with tissue cell antigens to causing cellular damage (e.g. antigens on red blood cells and platelets combine with autoantibodies to cause hemolytic anemia and thrombocytopenia, respectively), resulting in multi-system damage of the body.
5.What factors are related to the development of SLE?
(1) Environmental factors: For example, sunlight (ultraviolet light) exposure and hair dyeing can aggravate the disease of lupus erythematosus or make it more active; women with high IQ and high work pressure are mostly seen in patients with lupus erythematosus, etc., all of which indicate that the environment plays a role in the development of lupus erythematosus.
(2) High levels of estrogen: women of childbearing age, where estrogen secretion is most intense, 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 working and living habits and actively guard against infections in order to avoid lupus erythematosus from bringing certain damage to oneself.
6.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 (by genetic disease, we mainly mean monogenic genetic diseases, i.e. genetic diseases controlled by a pair of alleles, including red-green blindness, hemophilia and albinism, etc.), but has some genetic predisposition.
(3) Evidence of a genetic predisposition includes.
(a) Blacks and Asians have higher rates of lupus than whites.
(b) The probability of one identical twin having the disease and the other having the disease is 25% to 70%, compared with 5% for heterozygotic twins.
(c) The probability of first-degree relatives of lupus patients is 1-16%;
(d) In families with more than two lupus patients, the most frequent relationship is between mother and daughter, followed by sisters, brothers and fathers and daughters.
(4) Its susceptibility is determined by several genes: including the leukocyte antigen (HLA)-class II molecules on the short arm of human chromosome 6 – DR2 and DR3.
7. How harmful is SLE?
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 this disease, it is a great blow to women who love beauty; on the other hand, patients often have a very fat face due to taking hormones, the so-called full moon face and buffalo back, and patients are reluctant to see others.
(2) Affects the quality of life: fever, depression, weakness, arthralgia, muscle pain, Raynaud’s phenomenon, mouth ulcers and lack of food and drink are often present during the active period.
(3) Different manifestations appear in different organ involvement: for example, headache, epilepsy, convulsions and mental abnormalities in neurological involvement, protein and blood in urine in renal involvement, abdominal pain and diarrhea, nausea and vomiting in digestive system involvement, etc.
8. What are the early symptoms of SLE? What are the characteristic manifestations?
(1) Early manifestations are not characteristic.
(2) It mostly develops in spring, and the first symptoms are mainly manifested as joint pain, rash and systemic manifestations.
(3) Joint symptoms are mainly in the small joints of both hands with pain and mild swelling, similar to rheumatoid, but of short duration, without deformity or bone destruction.
(4) The rash is mostly bright red butterfly-shaped erythema on the face (both cheeks and the bridge of the nose, while the nasolabial folds are not involved, with clear edges and a butterfly-like appearance) and frostbite like rash on the back of both hands (polymorphic erythema), which are also more characteristic manifestations. The former has tender edges, mostly without itching, and the rash 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 back of both hands is symmetrically distributed edematous erythema, not ulcerated, not itchy, but with burning pain, and exists all year round, while frostbite is good 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 antibody, double-stranded DNA antibody, anti-ENA antibody and complement should be done for suspected cases.
9.What tests should be done for SLE?
(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 kidney 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, electrocardiogram and fundus of the eye should be checked regularly.
10. What criteria do doctors use to diagnose lupus erythematosus?
Lupus is generally diagnosed according to the American or international diagnostic criteria for classification, such as the classification of lupus diagnostic manifestations proposed by the American College of Rheumatology in 1997, which has a total of 11 items. 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, either erythrocyte, hemoglobin, granular tubularity 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 x 109/L at least twice; lymphocytes < 1.5 x 109/L at least twice, thrombocytopenia < 100 x 109/L (except for drug effects).
(10) Immunological abnormalities: antiphospholipid antibodies; positive anti-double-stranded DNA antibodies; positive anti-Sm antibodies; false-positive syphilis serologic test.
(11) positive antinuclear antibodies. 2009 saw the promulgation of a new international diagnostic criterion for lupus, with diagnosis being made in two cases, the first being confirmed by renal pathology confirming the presence of lupus nephritis plus positive ANA or anti-ds-DNA; the other being ≥4 of the following indicators (containing 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.
11. What are the treatments for lupus erythematosus?
They are divided into general treatment and drug treatment.
(a) 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.
(ii) Drug therapy.
(1) Non-steroidal anti-inflammatory drugs: suitable for those with low fever, joint symptoms, rash and pericarditis and pleurisy, and cautiously used 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, caution for those with 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. In severe cases, 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
12.What do I need to pay attention to in life and diet when I have SLE?
(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 charlatan doctors, go to regular hospitals for treatment and follow medical advice.
(2) Rest and activity problems: rest more during the activity period, to ensure 8-10h sleep at night, daily lunch break, with the improvement of the disease, gradually increase the amount of activity, in order not to feel tired.
(3) Makeup and sunlight 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 sunlight from 11:00 p.m. to 3:00 p.m.; wear protective hats and long-sleeved clothes, put UV filters in windows (most patients are allergic to UVB, some are allergic to UVA and even visible light, glass protects 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 SPF≥50, UVA PA (++)~(++++), sunscreen should contain physical sunscreen ingredients such as titanium dioxide and 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 pharmaceutical 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, alcohol consumption mainly due to interaction with certain liver-damaging drugs leading to liver damage.
(b) Adjustment according to organ involvement and other manifestations: the appearance of hair loss do not eat cauliflower (aggravate the process of hair loss?) (c) avoid the application of food or drug allergic to certain foods or drugs; those with photosensitivity to avoid using foods or drugs that may increase photosensitivity such as mud snails, parsley, celery, figs, mushrooms and smoked foods, western drugs such as sulfonamides, tetracyclines, hydrochlorothiazide and estrogen, and herbal medicines 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 (isoniazid can inhibit this enzyme). If renal damage and severe proteinuria, without renal failure, should be high protein diet, do not eat spinach (because of the oxalate, can increase proteinuria and tubular urine), if there is renal failure, must limit protein intake, to supplement animal high-quality protein, less beans and soy products; swelling 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 traditional 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.
13.Can lupus be cured?
Lupus erythematosus is an autoimmune disease, because there are too many causes, not caused by a single factor, so it cannot be cured, but it can be controlled and not developed, and under the condition of taking a small amount of drugs to maintain the non-development, it can dance with wolves and coexist with the disease for a long time.
14.Is SLE easy to relapse? Relapse performance? What are the recurrence factors?
Yes, it is easy to relapse. Recurrence manifestations.
(1) Fever of unknown origin, i.e. not explained by cold, pharyngeal, pulmonary and urinary tract infections, etc.
(2) reappearance of a new rash or an accompanying vasculitis-like rash on the ends of the fingers (toes) or other areas.
(3) Recurrence of joint swelling and pain.
(4) Significant hair loss, excluding hormonal causes.
(5) Fresh ulcers of the mouth and nose.
(6) Development of pleural or pericardial effusion.
(7) Increased proteinuria.
(8) Significant leukopenia or thrombocytopenia or anemia.
(9) Presence of neurological symptoms, such as headache, vomiting, and convulsions.
(10) Increased titer of anti-double-stranded DNA antibody.
(11) Increased blood sedimentation of 50 mm/hour or more.
(12) Decrease in complement, especially 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 and 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 about half of the patients have aggravation or relapse 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, if there is an antiphospholipid syndrome or nephrotic syndrome, if there is a history of hypercoagulability or thrombosis), or if the disease is unstable or has not been stabilized for a long time, especially in lupus nephritis. If pregnancy is planned, immunosuppressants such as cyclophosphamide and mycophenolate should be avoided and, if necessary, azathioprine <2 mg/(kg.d) should be used, 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 hormone is small (e.g. prednisone <15mg/d) and no immunosuppression, as prednisone ≥20mg/d can increase 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, routine blood and urine, creatinine clearance, 24h total protein and calcium, anti-platelet antibodies and antiphospholipid antibodies if platelets are decreased. Lupus patients are prone to miscarriage in the first three months of pregnancy, so they should take medication and avoid trauma and injury; the disease is likely to worsen in the second three months of pregnancy and after delivery (about 50%), so they should be monitored closely. 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, including 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) Others: sudden discontinuation or rapid reduction of hormones; seeking medical help everywhere, taking medication indiscriminately, stopping medication that can control the disease; overexertion, little rest; irregular life; violent emotional fluctuations.
15.What are the prevention methods for SLE?
(1) Prevent the above recurrence factors, such as preventing exposure to sunlight, 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 and vitamins, and eat more fruits and vegetables.
(3) Increase physical exercise appropriately. Patients in the active stage should pay attention to bed rest, and after the condition is stabilized, they can participate in some appropriate social activities and engage in some work that they can do, but they should not overwork, and appropriate physical exercise and abstain from daily sex life are conducive to the treatment of the disease.