Overview of the disease
Lupus erythematosus is a disease caused by the immune system mistakenly injuring normal tissues and organs. The cause of the disease is unknown, and it is related to a variety of factors such as genetics and endocrinology, etc. It manifests itself as pterontic erythema, joint pain, shortness of breath, palpitations and other symptoms.
What is Lupus Erythematosus?
Definition
Lupus erythematosus is an immune-mediated connective tissue disease.
Typical clinical manifestations are butterfly-shaped erythema on the face, but in addition to skin damage, it often involves multiple systems and organs.
Classification
Lupus erythematosus can be divided into cutaneous lupus erythematosus and systemic lupus erythematosus according to clinical manifestations and histopathologic features.
Cutaneous lupus erythematosus (CLE)
Acute cutaneous lupus erythematosus (CLE): both limited and generalized.
Subacute cutaneous lupus erythematosus: including erythema annulare, papulosquamous, and neonatal lupus erythematosus.
Chronic cutaneous lupus erythematosus: including limited and disseminated discoid lupus erythematosus, verrucous lupus erythematosus, swollen lupus erythematosus, deep-seated lupus erythematosus (lupus sebaceous membrane inflammation), tetanus-like lupus erythematosus, Blaschko’s linear lupus erythematosus and so on.
Systemic lupus erythematosus (SLE)
SLE is characterized by multiple systemic and organ involvement, recurrent relapses and remissions, and the presence of a large number of autoantibodies in the body. If left untreated, patients will experience irreversible damage to the involved organs, ultimately leading to death.
Morbidity
50~85% of patients with cutaneous lupus erythematosus are discoid lupus erythematosus, most common in middle-aged people aged 40~50 years old, the ratio of women to men is 3:1. 1.3%~5% of patients with discoid lupus erythematosus can progress to systemic lupus erythematosus.
Swollen lupus erythematosus occurs more often in young men.
The prevalence of SLE varies greatly geographically, with a global prevalence of (0-241)/100,000, a prevalence of (30-70)/100,000 in mainland China, and a female-to-male ratio of 9:1.
Questions you may be concerned about
How long can a lupus patient live?
The life expectancy of lupus erythematosus patients varies with the type of disease and severity of the disease. Most of them do not have any impact on their life expectancy after regular treatment, and need long-term follow-up observation.
Most cases of cutaneous lupus erythematosus involve the skin, and patients can survive for a long time with regular treatment to control the disease.
With the improvement of diagnosis and treatment, the survival rate of SLE patients has also increased dramatically, and the 5-year survival rate has exceeded 90%. The disease has changed from an acute and highly fatal disease to a chronic and controllable disease, but requires long-term follow-up observation.
Who is susceptible to lupus erythematosus?
People with a family history of the disease, exposure to infections or chemicals and other environments, and women are prone to get Lupus, as described below.
People with a family history of lupus;
Infections with specific viruses such as cytomegalovirus cause an autoimmune response that may progress to lupus erythematosus;
People with long-term exposure to chemicals, exposure to ultraviolet light, and long-term use of drugs such as isoniazid, hydralazine, and chlorpromazine;
Female patients have a higher incidence than men, especially women of childbearing age.
Can lupus erythematosus be completely cured?
Lupus erythematosus cannot be completely cured.
Lupus erythematosus is an autoimmune disease characterized by chronic recurrence. Symptoms and severity of the disease can be controlled by medication, but it cannot be cured completely.
Lupus erythematosus is mainly treated by medication to inhibit inflammation and regulate the immune response to improve the patient’s symptoms. Early detection and early diagnosis, combined with regular follow-up, can control the disease, minimize recurrence, avoid exacerbation and slow down the progression of the disease.
Etiology
The cause of the disease is still unclear, and is related to a variety of factors, such as heredity, sunlight, drugs, chemical agents and other factors.
Causes
The cause of lupus erythematosus is complex, and the pathogenesis is still not completely clear. Currently, it is believed to be related to a variety of factors such as heredity, sex hormones, and the environment.
Genetic factors
There is a tendency of family aggregation in the development of systemic lupus erythematosus, with a heritability of about 43%, and more than 90 susceptibility genes have been found.
Sex hormones
Systemic lupus erythematosus is most common in women of childbearing age, and pregnancy can trigger or aggravate the disease.
Ultraviolet light
Ultraviolet radiation can alter the chemical structure of DNA in skin tissues, making it more immunogenic and thus more immunogenic, thus triggering or exacerbating lupus erythematosus.
Drugs
Certain medications such as penicillin, isoniazid, and biological agents can also induce pharmacologic lupus.
Infections
Streptococcal and EBV infections can also trigger or exacerbate the disease.
Other factors
Cold stimulation, endocrine disorders, pregnancy, trauma, stress, pressure, overwork, etc. can also affect the occurrence and development of lupus erythematosus.
Symptoms
Clinical symptoms are varied. Early symptoms are often atypical and may include fever, fatigue and malaise. Most patients will develop a rash with butterfly-shaped erythema on the face, accompanied by joint pain.
Major Symptoms
There is individual variability in the clinical presentation of patients with lupus erythematosus, which may occur suddenly or progress gradually, may be mild or more severe, and may be intermittent or persistent.
The lesions are characterized by butterfly-shaped erythema on the cheeks of the face or red patches on other parts of the body. Sun exposure can cause the lesions to appear or worsen.
Systemic symptoms: fatigue and fever may be present.
Symptoms of various systems: headache, chest pain, dyspnea, arthralgia, Raynaud’s phenomenon (fingers and toes turn pale or greenish-purple in response to cold or pressure), etc.
Cutaneous lupus erythematosus
Acute Cutaneous Lupus Erythematosus (ACLE)
Mainly a skin manifestation in patients with SLE. Acute cutaneous lupus erythematosus sometimes presents with maculopapular lesions called lupus erythematosus herpetiformis (Lupus herpetiformis).
Limitations
Characteristics of the lesions: fused edematous erythema of the cheeks and dorsum of the nose (butterfly shape).
Site of onset: mainly on the face, may involve the forehead, neck, orbits and V-shaped area of the chest (exposed parts of the body).
Generalized
Symmetrically distributed fused macules and papules with purpura or petechiae, dark or bright red in color, may occur on any part of the body and may be accompanied by itching.
Subacute cutaneous lupus erythematosus
Subacute cutaneous lupus erythematosus usually develops on exposed areas of the upper trunk, such as the face, neck, chest, back of the shoulders and upper limbs.
Papular scaly type
Papules, erythematous spots or plaques of different sizes and shapes, covered with a thin layer of non-adherent scales, resembling psoriasis or furunculosis.
It is more likely to have renal involvement and develop into systemic lupus erythematosus.
Erythema annulare
Circumferential, polycyclic or arcuate, mildly elevated peripheral edematous erythema with smooth or scaly patches.
Generally, the condition is stable, the lesions are photosensitive, and there is no scar after healing, but there are pigmentation changes, and capillary dilatation can also be seen.
Neonatal lupus erythematosus
Lupus erythematosus neonatorum develops in the neonatal period due to the entry of antibodies from the mother’s body into the fetus through the placenta, and manifests as circular erythema of the skin, which subsides by itself in the first 4-6 months of life, but with cardiac damage left behind.
Chronic cutaneous lupus erythematosus
Discoid lupus erythematosus (DLE)
Discoid lupus erythematosus (DLE) is limited if the lesions only involve the scalp, face, ears, lips and mouth, and disseminated if the lesions involve the trunk, hands, feet and limbs.
Location: The lesions often involve the face, especially the back of the nose and cheeks, and may also involve the auricles, lips, and head, and scalp involvement may lead to permanent scarring alopecia.
Characteristics of lesions
Typical lesions are well-demarcated disk-shaped erythematous plaques or plaques, flat or slightly elevated, with adherent scales.
Peeling off the scales reveals underlying keratinous plugs and enlarged follicular openings.
Atrophy and hypopigmentation gradually appear in the center of the lesion, and hyperpigmentation in the periphery.
Other symptoms: no self-conscious symptoms or mild itching or burning sensation, a few patients may have low-grade fever, fatigue or arthralgia.
Deep-seated lupus erythematosus (lupus sebaceous meningitis)
Site of incidence: Prevalent in the face, upper arms (especially the deltoid region), buttocks and femur.
Characteristics of lesions: subcutaneous nodules or plaques with normal or dark purple-red surface skin, which form localized skin depressions after fading.
Accompanying symptoms: a few patients may be accompanied by systemic symptoms such as low-grade fever, arthralgia and malaise.
Frostbite-like lupus erythematosus
Frostbite-like lupus erythematosus is characterized by purplish-red patches on the cheeks, back of the nose, auricles, hands, feet, knees and elbows.
It mostly occurs in cold and humid environments, and most patients with this type have photosensitivity and Raynaud’s phenomenon.
The lesions usually do not itch significantly and do not fade with warming.
Verrucous lupus erythematosus
Pathogenesis: It occurs on the extensor side of the upper limbs, hands and face.
Lesions are characterized by hypertrophy in the form of warts, and the surface is often covered with a thick layer of adherent and tightly packed scabs, similar to hypertrophic lichen planus.
Swelling lupus erythematosus
Location: Facial and limb.
Characteristics of lesions: raised edematous erythema, or annular or semicircular doughnut-like plaques, with smooth surface, no scales and follicular keratosis, and high skin temperature.
Blaschko’s linear lupus erythematosus.
Site of incidence: Prevalent in the head and face.
Characteristics of lesions: linear distribution of erythema, subcutaneous nodules or limited non-scarring alopecia.
Systemic lupus erythematosus
Clinical manifestations are varied, and the natural course of the disease is characterized by alternating exacerbations and remissions.
Fever, arthralgia and erythema pteronyssinus on the face are the most common early symptoms of the disease, and with the progression of the disease, most patients gradually develop multi-system damage.
Fever: the fever type is variable, some are low fever, some can reach 39℃~40℃, or irregular fever. Some patients may be accompanied by enlarged lymph nodes.
Joint muscle: joint swelling and pain, muscle pain, but muscle weakness is not obvious. Good invasion of finger, toe, knee, wrist joints, joint symptoms in the active period of the disease aggravated.
Skin mucosa: edematous butterfly-shaped erythema on the cheeks and bridge of the nose, often aggravated by sunlight, as a typical skin lesion. There may be ulcers on the mucous membranes of the mouth and nose.
Nails: vasculitis-like damage such as purplish-red macules, petechiae, capillary dilatation and punctate atrophy of fingertips at the distal and periungual areas of the limbs and the ends of the fingers (toes).
Hair: frontal hairline hair is dry, jagged, fine and easily broken (lupus hair).
Kidney: there may be foamy urine (proteinuria) and other manifestations of nephritis, nephrotic syndrome, renal insufficiency, and uremia.
Respiratory system: can have cough, cough sputum, dyspnea and other symptoms such as pleural effusion, pleurisy, alveolitis (hemorrhage), interstitial lung disease.
Neurological system: it can be manifested as depression, oligophrenia, or even dementia, or there can be manifestations of lupus encephalopathy such as epileptiform seizures, headache, delirium, convulsions, hemiparesis, or coma which can lead to rapid death.
Digestive tract symptoms: loss of appetite, nausea, vomiting, abdominal pain, diarrhea and other intestinal and mesenteric vasculitis manifestations.
Other symptoms: dry mouth, dry eyes, anemia, chest pain, palpitations, etc.
Consultation
Usually consult the Department of Rheumatology and Immunology. When joint pain and pteronyssinus erythematosus occur, prompt medical attention is recommended.
Department of Medicine
Department of Dermatology and Venereology
If erythema appears on the surface of the skin in various parts of the body, or if there is fever or joint pain, it is recommended that you consult a doctor promptly.
Department of Rheumatology and Immunology
If you have erythema of the skin, especially butterfly-shaped erythema of the face, fever, joint pain, etc., you may consult the Department of Rheumatology and Immunology.
Emergency Department
Headache, delirium, convulsions, hemiplegia, coma, or chest pain, dyspnea, etc. require immediate consultation at the Emergency Department.
Preparation for medical treatment
How to get to the doctor: registration, preparation of documents, common problems
Tips
Take a picture of your previous rash (lesion) on your cell phone before you go to the doctor, as this may give the doctor more information.
If you have lesions on your face, do not wear makeup before the visit.
Preparation List
List of symptoms
Are there erythema or papules on the face or trunk extremities? Is there butterfly-shaped erythema on the face?
Is the skin damage more pronounced in light-exposed areas?
Are there symptoms of hair loss?
Are there symptoms of mouth ulcers?
Are there any accompanying symptoms such as fatigue, fever, joint pain or muscle pain?
If any of these symptoms are present, how long have they been present?
List of medical history
Any long-term use of drugs such as isoniazid, hydralazine, chlorpromazine, etc.?
Any long-term exposure to chemicals?
Any other autoimmune diseases such as scleroderma, dry syndrome, etc.?
Any family history of lupus erythematosus?
Checklist
Test results of the last six months, which can be brought to the doctor’s office
Routine blood test, urine test, blood sedimentation test
Immune-related tests such as antinuclear antibody, complement test, etc.
Skin histopathology
Medication list
Medications used in the last 3 months, if there is a box or package, you can bring it to the doctor.
Topical glucocorticosteroids: mometasone furoate cream, dieldrin cream, halometasone ointment
Topical calcineurin inhibitors: tacrolimus ointment, pimecrolimus gel
Intravenous glucocorticoids: prednisone, methylprednisolone, etc.
Immunosuppressants: Methotrexate, Matemacrolate, etc.
Other drugs: hydroxychloroquine, thalidomide, aminoglutethimide, etc.
Diagnosis
Diagnosis is mainly based on symptoms and laboratory tests, such as blood tests for immune-related markers, and sometimes tissue biopsy.
Diagnosis is based on
Medical history
Family history of lupus erythematosus may be present.
Clinical manifestations
Butterfly-shaped erythema on the cheeks of the face or red patches on other parts of the body. Exposure to sunlight may cause the lesions to appear or worsen.
Systemic manifestations such as fatigue and fever may be present.
There may be headache, chest pain, dyspnea, arthralgia, Raynaud’s phenomenon.
Laboratory Tests
Autoantibody test
More than 80% of patients with acute cutaneous lupus erythematosus are positive for antinuclear antibodies (ANA), and may also be positive for anti-Sm antibodies, anti-double-stranded DNA (dsDNA) antibodies, anti-Ro/SSA antibodies (anti-Ro antibodies), and anti-La/SSB antibodies (anti-La antibodies).
More than 90% of patients with subacute cutaneous lupus erythematosus are positive for antinuclear antibodies, and 70% to 90% are positive for anti-Ro antibodies (anti-SSA antibodies) and anti-La antibodies (anti-SSB antibodies).
Patients with chronic cutaneous lupus erythematosus may be positive for antinuclear antibodies at low titers, and 1% to 3% are positive for anti-Ro antibodies (anti-SSA antibodies); anti-double-stranded DNA antibodies are present in <5%.
The main antibodies detected in SLE patients are as follows.
Antinuclear antibodies: in the active phase of SLE, the positivity rate can be 80% to 90% or more, or even (required) 100% positive.
Anti-double-stranded DNA antibodies: the hallmark (highly specific) antibodies of SLE, positive in 40% to 70% of patients, correlating with disease activity, especially with kidney damage. It can be used as an indicator to monitor changes in SLE and drug efficacy.
Anti-Sm antibody: the hallmark antibody of SLE, positive in 30% of patients, with the same clinical significance as anti-double-stranded DNA antibody, but not related to disease activity.
SLE patients may also be detected with a variety of antibodies, including anti-U1 ribonucleoprotein (U1RNP) antibody, anti-Ro antibody, anti-La antibody (anti-SSA antibody, anti-SSB antibody), anti-cardiolipin antibody, anti-beta glycoprotein 1 antibody, lupus anticoagulant, anti-human immunoglobulin, and so on.
Other Laboratory Tests
Blood routine, erythrocyte sedimentation rate (hematocrit), urine routine, kidney function and other tests may be performed.
Patients with acute cutaneous lupus erythematosus may have anemia, leukopenia, thrombocytopenia, elevated erythrocyte sedimentation rate, proteinuria and hematuria.
In patients with subacute cutaneous lupus erythematosus, a few may have leukopenia, elevated erythrocyte sedimentation rate and proteinuria.
Laboratory tests for chronic cutaneous lupus erythematosus are mostly normal, with a few patients having anemia, leukopenia, thrombocytopenia, and elevated erythrocyte sedimentation rate. Urine routines are rarely abnormal.
Systemic lupus erythematosus may have anemia, leukopenia, increased erythrocyte sedimentation rate; routine urinalysis may have proteinuria, hematuria, and tubular urine in the case of renal damage, and 24-hour urinary protein quantification is an important indicator for determining the activity of lupus nephritis.
Pathologic examination
Skin histopathology shows hyperkeratosis, follicular keratosis, epidermal atrophy, basal cell liquefaction; connective tissue mucoid edema, fibrin-like degeneration and necrotizing vasculitis.
Frozen tissue specimens were treated with fluorescent antigen and stained, and then placed under a fluorescence microscope for observation, i.e., direct immunofluorescence (DIF).
More than 90% of the skin lesions of SLE patients are positive, and 50% to 60% of normal-looking skin is also positive, especially the positive rate of normal skin in exposed areas can reach 70% to 80%.
Whether normal skin is positive or not is a reliable method to distinguish SLE from cutaneous lupus erythematosus.
Other tests
Involvement of internal organs in SLE patients may present with corresponding abnormalities in lung function, chest X-ray, electrocardiogram, ultrasound, magnetic resonance imaging of the head and cerebrospinal fluid examination.
Differential Diagnosis
Differential of cutaneous lupus erythematosus
Acute cutaneous lupus erythematosus should be differentiated from rosacea, seborrheic dermatitis, drug rash, dermatomyositis and other cutaneous vasculitides.
Subacute cutaneous lupus erythematosus should be differentiated from psoriasis, polymorphic sun rash, tinea corporis, erythema annulare, and second-stage syphilis.
Chronic cutaneous lupus erythematosus should be differentiated from lichen planus, nodular disease, scleroderma, common warts, lymphoma, cutaneous lymph node infiltration, and stage III syphilis.
Similarities: The similarities between these diseases and cutaneous lupus erythematosus are mainly characterized by the presence of red patches on the skin.
Differences: Direct immunofluorescence (DIF) of the skin lesions is helpful. In general, a positive DIF at the lesion supports the diagnosis of cutaneous lupus erythematosus, but a negative DIF does not rule out the diagnosis.
Differentials of systemic lupus erythematosus
SLE should be differentiated from dermatomyositis, erythema multiforme, acute rheumatic fever, rheumatoid arthritis, and drug rash.
Similarities: The above diseases may present with red skin patches and multi-system and multi-organ damage similar to SLE.
Differences: Hematologic abnormalities, autoantibodies, and biopsies of affected kidneys in SLE patients can help to differentiate them.
Treatment
Adrenocorticotropic hormone with immunosuppressants is the main treatment option. Commonly used drugs include cyclophosphamide, merti-macrolide and cyclosporine.
Cutaneous lupus erythematosus is usually treated with a stepwise treatment model, including general, localized to systemic therapy.
The principle of SLE treatment is early, individualized and standardized treatment, and the goal of treatment is to control the disease activity, achieve remission or low disease activity state, prevent and reduce recurrence, reduce drug toxicity, maximize the delay of disease progression, reduce organ damage and improve the prognosis.
Patients need to follow the doctor’s instructions to avoid self-adjustment of medication.
General treatment
Avoid adverse stimuli, such as sun protection, cold protection, smoking cessation, and avoiding trauma.
Diet, pay attention to vitamin D supplementation, try to avoid high salt diet, as well as avoid photosensitive food, such as celery.
Be careful with photosensitizing drugs, such as quinolones and tetracycline antimicrobials.
Localized treatment
Glucocorticoid
Weak and medium potent preparations are chosen for thin and tender skin, strong or super potent preparations are chosen for hypertrophic and warty lesions, and intradermal injection of glucocorticosteroids can also be used.
The duration of treatment of topical glucocorticosteroids should not be too long, especially the strong and super-strength glucocorticosteroids, which should not be used continuously for more than 2 weeks, and can be considered to be repeated intermittently for a longer period of treatment.
Calcium-modulated phosphatase inhibitors
They are effective in subacute cutaneous lupus erythematosus and acute cutaneous lupus erythematosus, and slightly less effective in discoid lupus erythematosus.
Commonly used preparations: tacrolimus, pimecrolimus cream, etc.
Vitamin A-acid preparations
Can be used in discoid lupus erythematosus with obvious keratinization.
Commonly used preparations: tazarotene gel and retinoic acid cream.
Systemic therapy
Antimalarials
Antimalarials are the first line of systemic therapy.
They are effective in discoid lupus erythematosus, swollen lupus erythematosus and subacute lupus erythematosus by more than 80%.
It can be used concurrently with NSAIDs in the treatment of SLE patients, and can reduce skin and joint symptoms, as well as improve pleurisy and mild pericarditis.
Commonly used preparations: hydroxychloroquine. It is contraindicated in children younger than 6 years of age, and the minimum effective dose should be used in children 6 years of age and older. Continuous treatment with hydroxychloroquine is recommended for pregnant patients.
Watch for ocular adverse reactions.
Glucocorticoids
Disseminated discoid lupus erythematosus, recalcitrant discoid lupus erythematosus, acute cutaneous lupus erythematosus, and some subacute cutaneous lupus erythematosus require systemic treatment with glucocorticoids.
Glucocorticoids are important drugs in the treatment of SLE, with small to moderate doses for patients with mild and moderate disease. High-dose therapy or shock therapy is required for lupus nephritis and lupus encephalopathy.
In cutaneous lupus erythematosus, intermediate doses are usually used, and long-term glucocorticoid maintenance therapy is not recommended for those without systemic involvement.
Commonly used agents: prednisone, methylprednisolone, etc.
Non-steroidal anti-inflammatory drugs (NSAID)
Mainly used for the treatment of systemic lupus erythematosus muscle and joint symptoms and fever and other systemic symptoms. When there are symptoms of digestive tract irritation, it can be combined with oral mucosal protection drugs (proton pump inhibitors).
Commonly used agents: aspirin, indomethacin, diclofenac sodium, ibuprofen, loxoprofen sodium, celecoxib, etoricoxib, and so on.
Immunosuppressants
Lupus encephalopathy and lupus nephritis often require the concomitant application of immunosuppressants and large amounts of prednisone (glucocorticosteroids, such as prednisone, methylprednisolone, etc.).
Cyclophosphamide is one of the effective drugs in the treatment of severe SLE.
It is less commonly used in the treatment of patients with cutaneous lupus erythematosus. Glucocorticoids may be added or switched to immunosuppressive drugs when they are ineffective or have been used for too long to be tapered.
In patients with cutaneous lupus erythematosus without systemic involvement, mercaptopurine, cyclosporine and cyclophosphamide are not recommended.
Commonly used agents: methotrexate, azathioprine, cyclophosphamide, morphimecrolide, 2-chlorodeoxyadenosine, tacrolimus, and leflunomide.
Thalidomide
Can be used to treat relapsing or refractory cutaneous lupus erythematosus. Recommended in combination with hydroxychloroquine.
A small number of patients may develop symptoms of peripheral neuropathy after taking it, and should be discontinued as soon as they occur.
With pregnancy planning, pregnancy is prohibited, and should be strictly contraceptive during the drug and within 6 months of stopping the drug.
Aminophenyl sulfone
Mainly used in the treatment of lupus erythematosus herpetiformis, but also used in discoid lupus erythematosus and subacute cutaneous lupus erythematosus when conventional drugs are not effective.
Recommended for use in combination with hydroxychloroquine. Glucose-6-phosphate dehydrogenase activity and HLA-B related gene examination are recommended before treatment to avoid acute hemolytic reaction and aminoglycoside syndrome.
Plant extracts
Such drugs have some immunosuppressive and/or immunomodulatory effects.
Commonly used preparations: tretinoin, kunming shanhaitou, total white peony glycosides, etc.
Contraindicated with pregnancy planning, during pregnancy, lactation, and in children.
Vitamin A acid
Mainly used in the treatment of chronic cutaneous lupus erythematosus, especially for warty lupus erythematosus.
Commonly used preparations: avitaminic acid, isotretinoin.
Retinoic acid is fat-soluble, taking the drug with meals can promote absorption, and the dosage can be adjusted according to the clinical effect and adverse reactions after 2~4 weeks of treatment, and the course of treatment is generally from several weeks to several months.
Retinoids have a clear teratogenic effect and are contraindicated in women with a pregnancy plan or during pregnancy.
Biological agents
Biological agents are mainly used in patients with systemic lupus erythematosus; they are not recommended for patients with cutaneous lupus erythematosus without systemic involvement.
Commonly used agents: intravenous human immunoglobulin, rituximab, belimumab, tetracycline, etc.
Stem cell transplantation
Stem cell transplantation is used in some patients with refractory SLE. It is not recommended for patients with cutaneous lupus erythematosus without systemic involvement.
Other treatments
In severe cases such as lupus nephritis, plasma exchange may be used.
High-dose intravenous injection of human blood gammaglobulin may be considered for patients with hemolytic anemia or thrombocytopenia and for SLE with unsatisfactory efficacy of treatment with glucocorticoids.
Special treatment for women of childbearing age
Women of childbearing age should pay attention to contraception during the course of treatment, and SLE patients should prepare for pregnancy at least 6 months (preferably one year) after stabilization of the disease; if pregnancy occurs unexpectedly during the active period of the disease, it is best to terminate the pregnancy at an early stage of the pregnancy.
Throughout the pregnancy, closely observe the changes of the disease and laboratory indicators, and ask experienced rheumatologists and obstetricians and gynecologists for guidance during the perinatal period, so as to avoid serious consequences.
Continued use of hydroxychloroquine and low-dose aspirin is recommended for SLE patients during pregnancy.
Non-steroidal anti-inflammatory drugs (NSAIDs), glucocorticoids (low-dose prednisone), mercaptopurine, cyclosporine, and tacrolimus are permitted during pregnancy in patients with SLE, but there is a low risk of causing fetal damage.
Biologic agents should be used with caution in pregnancy in patients with SLE, and cyclophosphamide, mertiomaxolide, methotrexate, and leflunomide are contraindicated.
Breastfeeding is encouraged for most women with SLE, and medication use during breastfeeding requires individualized discussion of medication risks.
Prognosis
There is no cure, but the disease is manageable, and early and timely treatment and control is key. Pregnancy can be considered if the disease has been stable for more than six months, there is no damage to vital organs, and the doctor assesses that treatment with teratogenic medications can be discontinued.
Cure
Skin lesions of cutaneous lupus erythematosus can mostly disappear after treatment. Some patients with chronic cutaneous lupus erythematosus may have atrophic scarring and hyperpigmentation or depigmentation, and individual patients with discoid lupus erythematosus may have skin lesions for a long time.
Most patients with chronic cutaneous lupus erythematosus and subacute cutaneous lupus erythematosus have a good prognosis due to the lack of vital organ involvement.
The prognosis of acute cutaneous lupus erythematosus and systemic lupus erythematosus depends on the degree of vital organ involvement.
With the continuous improvement of diagnosis and treatment, the survival rate of SLE has improved substantially, with more than 90% of patients surviving for more than 5 years, and 80% to 90% of patients surviving for more than 10 years.
Harmfulness
Lupus erythematosus occurs on the face or exposed parts of the body affecting aesthetics, and patients may experience psychological stress, affecting their daily life and work.
The main causes of death in SLE patients are severe damage to multiple organs and infections, especially lupus nephritis and lupus encephalopathy, which have a high mortality rate.
SLE tends to aggravate during pregnancy or puerperium, especially patients with renal lesions or with hypertension are prone to pregnancy hypertension and cardiac insufficiency, and even death.
Daily
Avoid ultraviolet rays, wear a hat and sunglasses when going out, insist on the use of sunscreen, pay attention to rest, do not stay up late, exercise regularly, follow the doctor’s instructions for review, and avoid other organ damage.
Daily Management
Diet management
Light diet, small meals.
Diet should be given with high quality protein, high vitamin, low sugar, low salt, low fat, high calcium diet, such as egg and milk, fresh dark-colored vegetables (spinach, broccoli, kale, etc.), fresh fruits and so on.
For patients with lupus nephritis who develop renal insufficiency, pay attention to a low-protein diet and control water intake.
Avoid foods that induce or aggravate this disease, eat less seafood, spicy foods, such as fish, shrimp, chili peppers, etc. Avoid drinking coffee, strong tea, etc.
Avoid photosensitive foods, such as parsley, leeks, mushrooms and celery.
Avoid alcohol. Alcohol may have an effect on the efficacy of certain medications or aggravate existing symptoms.
Skin management
Avoid direct sunlight on the skin. Outdoor activities should be minimized during the summer months, and when outdoor activities are necessary, good sun protection should be used to reduce ultraviolet radiation on the skin, such as wearing a long coat, long pants, an umbrella or a wide-brimmed hat, and applying shading agents (sunscreens).
Cotton underwear should be worn, not chemical fiber clothing, to reduce friction on the skin.
Keep the skin clean, the water temperature is not easy to be too hot when bathing, do not scratch the scalp with force when washing hair, and should not use shower gel and shampoo to stimulate the skin lesions when there are skin lesions.
For patients with skin lesions on the scalp, face and other parts of the face, try to avoid the application of cosmetics, hair dyes, etc., so as not to cause the disease to recur or aggravate.
Life management
Have a regular daily life, ensure good sleep and sufficient rest, and avoid too much pressure.
Go to public places as little as possible, avoid overwork and colds.
During the stable period of the disease, exercise appropriately under the guidance of the doctor to strengthen the body’s resistance.
Female patients of childbearing age should not get pregnant during the acute or active period, pay attention to contraception, and get pregnant under the guidance of a doctor during the stable period of the disease.
Do not use health supplements on your own, the ingredients of health supplements may interact with the drugs you are taking. Health supplements are not a substitute for medication. If you want to use them, please consult your doctor first.
Psychological adjustment
Family members and friends should be concerned about and considerate of the patient, and create a kind, gentle and harmonious interpersonal environment.
Family members should help patients to build up confidence and courage to overcome the disease, so that they can take the initiative to cooperate with the treatment and care.
Patients should have correct knowledge of the disease and actively cooperate with the treatment.
Disease monitoring
Fatigue, fever, stomach pain, severe headache, and the appearance of new or worsening skin lesions in patients with lupus erythematosus often indicate disease activity.
Watch out for adverse effects of medications, and prompt ophthalmologic consultation is recommended if vision loss or blurred vision occurs. Hydroxychloroquine should be discontinued if retinopathy develops.
Follow-up and review
Patients with lupus erythematosus should be followed up regularly.
Routine laboratory tests, such as routine blood and urine tests, will be performed during the follow-up visits.
Indicators related to disease activity, such as erythrocyte sedimentation rate, complement, antinuclear antibody, and anti-double-stranded DNA antibody, should be reviewed every 6 to 12 months to assess whether the disease is stable, as well as to assess whether cutaneous lupus erythematosus is likely to develop into SLE.
For patients with active SLE, it is recommended that disease activity be assessed at least once a month; for stable SLE, it is recommended that disease activity be assessed every 3 to 6 months.
Those taking glucocorticoids should have their blood pressure, blood glucose, blood electrolytes, and bone mineral density checked regularly, and magnetic resonance imaging (MRI) of the hip joint should be performed if necessary.
Have an eye exam within the first year of taking hydroxychloroquine, and perform an eye exam once a year for those with abnormalities or age 60 years or older.
Prevention
The pathogenesis of lupus erythematosus is unclear and there are no effective preventive measures. The following measures can be taken to avoid recurrence or exacerbation of the disease.
Avoid sun exposure, infection, trauma, stress, overwork and other triggers of lupus erythematosus activity.
Prevent the occurrence of influenza and pneumonia by receiving influenza vaccine and pneumonia vaccine under the guidance of a doctor.