Systemic lupus erythematosus in children

Introduction to Pediatric Systemic Lupus Erythematosus Systemic lupuserythematosus (SLE) is a multi-system autoimmune disease characterized by extensive vasculitis and connective tissue inflammation, anti-nuclear antibodies (ANA), especially anti-dsDNA and anti-Sm antibody. The clinical manifestations of children with SLE are very complicated. In addition to the common manifestations of fever and rash, the clinical manifestations are different due to different organs. They often involve multiple systems such as urinary, nervous, cardiovascular, and blood, often at moderate to severe levels. Multiple organ damage. The process is heavy and potentially fatal, and the prognosis of children with SLE is more severe than that of adults. However, with the progress of diagnosis and treatment, the current prognosis is significantly improved, and the 10-year survival rate is about 80%. basic knowledge The proportion of illness: the probability of illness in infants and young children is 0.003% Susceptible people: children Mode of infection: non-infectious Complications: urinary tract infection, pyelonephritis, cholecystitis, folliculitis

Causes of systemic lupus erythematosus in children

The exact etiology and pathogenesis are still unclear. The pathogenesis seems to be related to heredity, immunity, sex hormones and environmental factors. It is based on the genetic susceptibility, and the external environment stimulates the body's immune dysfunction and immune regulation disorder. Sexual disease.

Genetic factors (30%)

The disease is related to human leukocyte antigen. Chinese people are closely related to HLA-DR2. Relatives of the children may have the same disease. The incidence rate of single-oval twins is 24%, and double eggs are 2%. In recent years, HLA has been found. - Class II alleles are associated with certain autoantibodies present in SLE patients, and 96% of patients with high dSdNA antibodies have HLA-DQBI*0201 (linked to DR3 and DR7) or DQBI*0602 (linked to DR2 and DRw6) or DQB1 *0302 (linked to the DR4 haplotype), anti-phospholipid antibodies and anti-Sm antibodies have also been found to be closely related to certain alleles, and some complement components, such as C2, C4, C1 genetic defects are also prone to the disease.

Immunization (20%)

Peripheral blood lymphocytes decreased, inhibitory T cells (CD8+) could not lower B cells to produce immunoglobulin (Ig), T cells produced increased IL-6, stimulated B cell proliferation, IL-2 decreased, B cell function was active , blood Ig is increased in polyclonal, there are a variety of autoantibodies in the body, such as anti-nuclear antibodies (ANA), anti-DNA antibodies, cold-reacting IgM anti-lymphocyte antibodies, IgG anti-neuro antibodies, anti-phospholipid antibodies, anti-lipid antibodies and Rheumatoid factor, etc., autoantibodies can cause acute hemolytic anemia, hypoleukocytosis, vasculitis and coagulopathy (thrombocytopenia, etc.), and the corresponding large number of immune complexes can cause nephritis, heart and central nervous system diseases. In this disease, the complement system is activated, blood complement is reduced, and kidney tissue often has complement components, especially Clq, suggesting that the activation is a classic pathway.

Hormone (20%)

The disease occurs in women, the incidence of women is 5 to 9 times that of men. Pregnancy and oral contraceptives can aggravate the condition, suggesting that there is an estrogen-mediated immune regulation disorder in the disease, and testosterone levels are often reduced in children's blood. SLE children Serum follicle stimulating hormone (FSH), luteinizing hormone (LH) and prolactin were higher than normal.

Environmental factors (25%)

Virus-like inclusion bodies can be found in the endothelial cells of patients, and anti-viral antibodies can be detected in the glomerular eluate. Both of these diseases are related to viral infections. Clinically, infections can be induced to induce the disease. Herpesvirus can increase serum Sm antibody in patients, but the role of infection in the pathogenesis of SLE is still unclear. Various viral antibodies can be detected in the blood of patients, especially anti-Rubella virus, Epstein-Barr virus and paramyxovirus antibodies are elevated. , may suggest polyclonal B-cell activation, but not specific immune response, UV irradiation can induce or aggravate the condition, UV can cleave nDNA of skin tissue cells, and induce keratinocytes to produce IL-1, IL-3, IL-6 And tumor necrosis factor, can also attenuate macrophage clearance of antigen and inhibit T cell activation, etc., certain drug allergies and strong psychological mood and stress can aggravate the condition.

Pediatric systemic lupus erythematosus prevention

(1) Avoiding light and eliminating fatigue: Fatigue is the most common manifestation of SLE. It is the result of multiple factors. Relieving fatigue also depends on the identification of potential causes. Photoallergies can also cause fatigue in patients, and regular sunshades and sunscreens as well as protective clothing are very important.

(2) Prevention of infection: Due to the immune dysfunction in SLE and the application of long-term immunosuppressive agents, co-infection is very common. For unexplained fever, you should actively seek medical attention, instead of thinking of recurrence of lupus. Proper use of glucocorticoids and immunosuppressive agents and timely adjustment of dosage and application time can reduce the risk of infection.

(3) Appropriate rest and exercise: Another prominent feature of SLE patients is that sedentary lifestyle, chronic process of disease, mental depression and fibromyalgia can significantly reduce the movement of SLE patients. Aerobic exercise in SLE patients such as hydrotherapy and walking is part of a non-pharmacological regimen for SLE patients. Patients in severely active period should rest in bed, and those who are in remission and mild cases may exercise or engage in non-physical work. Exercise helps prevent muscle atrophy and osteoporosis caused by long-term steroid therapy.

Pediatric systemic lupus erythematosus complications Complications urinary tract infection pyelonephritis cholecystitis folliculitis

Complications of systemic lupus erythematosus

1) Infection: Patients with systemic lupus erythematosus have low cellular immunity and are prone to colds and infections. The infection can induce lupus or aggravate the development of lupus and even worsen. Common bacterial infections include tonsillitis, bronchitis and pneumonia, urinary tract infections, pyelonephritis, cholecystitis, folliculitis, erysipelas, suppurative peritonitis, sepsis, and the like.

2) Chronic renal failure: Lupus nephritis can persist for many years, recurrent and aggravating the condition, which may eventually lead to kidney failure. The patient's urea nitrogen and uric acid increase, such as a short course of disease, can be reduced to normal through treatment.

3) hypoproteinemia: patients with chronic lupus nephritis, due to a large amount of proteinuria, resulting in protein loss, edema, from the back of the foot, calves, thighs up to the genitals, buttocks, waist, abdomen, diffuse edema .

Pediatric systemic lupus erythematosus symptoms common symptoms facial butterfly erythema protein urinary discoid erythema leukoplakia irregular hot joint swelling chest pain pericarditis abdominal pain

General symptoms

Fever, irregular heat type, with general malaise, fatigue, anorexia, weight loss, hair loss, etc.

2. Rash

Symmetrical cheek butterfly rash, across the bridge of the nose, with clear edges, slightly higher than the leather surface, and increased sun exposure; exposed areas of the upper chest and elbow may have erythematous rashes; palmar erythema, refers to The apical erythema of the toe), the erythema of the periorbital area, and the erythema of the distal part of the nail are all caused by vasculitis. There may also be skin bleeding and ulcers, especially the nasal cavity and oral mucosa.

3. Joint symptoms

Joints, muscle pain, joint swelling and deformity.

4. Heart

Can affect the endocardium, myocardium and pericardium, can be expressed as heart failure.

5. Kidney

From focal glomerulonephritis to diffuse proliferative glomerulonephritis, severe cases can die from uremia, and kidney involvement can be the first symptom.

6. Multiple serositis

Can involve the pleura, pericardium, peritoneum, can be affected alone or at the same time, generally do not leave sequelae.

7. Nervous system

Headache, personality change, epilepsy, hemiplegia and aphasia.

8. Other

Liver, spleen, swollen lymph nodes, may have symptoms such as cough, chest pain, and difficulty breathing.

9. Lupus crisis

The lupus crisis is a systemic disease that is caused by a wide range of acute vasculitis, which is often life-threatening. Children are more likely to have a crisis than adults.

(1) Continuous high fever, invalid with antibiotics.

(2) Outbreaks or acute episodes of one of the following manifestations: generalized extreme failure accompanied by severe headache; severe abdominal pain, often similar to acute abdomen; bleeding spots around the fingertips or around the nails; severe oral ulcers.

(3) Progressive decline in renal function with hypertension.

(4) Lupus pneumonia or pulmonary hemorrhage.

(5) The performance of severe neuropsychiatric lupus.

Pediatric systemic lupus erythematosus examination

1. Blood picture: White blood cell count is reduced, often <4×10 ⁹ /L, lymphocytes are reduced, often <1.5×10 ⁹ /L, different degrees of anemia, Coombs test can be positive, platelets are generally normal, can also be reduced.

2. Antinuclear antibodies: Most of them are peripheral type and spot type, and have anti-dsDNA antibody, anti-DNP antibody, anti-Sm antibody, anti-Ro (SSA) antibody, anti-La (SSB) antibody and the like.

3. Immunological examination: C3 decreased; IgG increased significantly, IgA, IgM also increased, gamma globulin increased, showing high gamma globulinemia; positive for circulating immune complexes.

4. Urine routine: There are urine protein, hematuria and tubular urine, liver and kidney function can be abnormal.

5. Lupus belt test: a small piece of skin was taken for biopsy and observed by direct immunofluorescence. It was found that there were granular or linear fluorescent bands on the boundary line between epidermis and dermis, which were caused by IgG, IgA, IgM and complement deposition.

6. Renal biopsy: It is of great value in the diagnosis, treatment and prognosis of lupus nephritis.

In recent years, transcranial Doppler ultrasound (TCD) can be used to diagnose lupus encephalopathy in children. It is considered that TCD is effective, simple, non-invasive and excellent in price. It is helpful for long-term follow-up observation of SLE condition. TCD is an early functional change of cerebrovascular disease in lupus encephalopathy. The detection provides a more sensitive and specific method.

Diagnosis and diagnosis of systemic lupus erythematosus in children

diagnosis

The diagnostic criteria for SLE in children are the same as those for adults. The SLE diagnostic criteria revised by the American College of Rheumatology in 1982 are often used. The 11 diagnostic conditions include:

1. Butterfly erythema on the cheeks.

2. Disc-shaped erythema.

3. Light sensitive.

4. Oral or nasal mucosal ulcers.

5. Non-erosive arthritis.

6. Nephritis (hematuria, proteinuria > 0.5 g / d, cell cast).

7. Encephalopathy (seizures or mental symptoms).

8. Pleurisy or pericarditis.

9. Hematocytopenia (anemia, leukopenia, thrombocytopenia).

10. ANA positive (anti-dsDNA antibody, anti-Sm antibody) or lupus cells positive, or persistent syphilis serum reaction false positive.

11. Fluorescent antinuclear antibodies are positive.

SLE can be diagnosed if 4 or more of the above conditions are met.

Differential diagnosis

The disease should be differentiated from other rheumatic diseases such as juvenile rheumatoid arthritis, dermatomyositis, scleroderma, mixed connective tissue disease, vasculitis, etc. Other diseases that need to be identified are bacterial or viral infections, various types of kidneys. Disease, chronic active hepatitis, blood diseases such as thrombocytopenic purpura, hemolytic anemia, etc.

The material in this site is intended to be of general informational use and is not intended to constitute medical advice, probable diagnosis, or recommended treatments.