What Are the Signs of Lupus in Children?

Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple systems, characterized by extensive vasculitis and connective tissue inflammation, and the presence of antinuclear antibodies, especially anti-dsDNA and anti-Sm antibodies. The clinical manifestations of pediatric SLE are very complicated. In addition to the common manifestations of fever and rash, the clinical manifestations of the disease are different due to the affected organs. They often involve multiple systems including urinary, neurological, cardiovascular, and blood. Multiple organ damage. The process is serious and potentially fatal, and the prognosis of SLE in children is more serious than in adults.

Basic Information

English name
systemic lupus erythematosus; SLE
Visiting department
Pediatrics
Multiple groups
female
Common causes
Genetic factors, immune factors, hormones, environmental factors
Common symptoms
Fever, hair loss, symmetrical erythema of cheek butterfly, joint and muscle pain, joint swelling and deformity, etc.

Etiology of Pediatric Systemic Lupus Erythematosus

Genetic factor
The disease is related to human leukocyte antigens. The Chinese are closely related to HLA-DR2. Relatives of children may have patients with the same disease. The incidence of monozygotic twins is 24% and that of twins is 2%. In recent years, it has been found that HLA -Class II alleles are associated with certain autoantibodies present in patients with SLE. 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 DR4 haplotype), anti-phospholipid antibodies and anti-Sm antibodies were also found to be closely related to certain type alleles, and some complement components, such as C2, C4, C1 genetic defects are also prone to the disease.
2. Immune factors
There are many immunological abnormalities in SLE: B cell hyperfunction, T cell imbalance, abnormal cytokine expression, and abnormal lymphocyte apoptosis. Peripheral blood lymphocytes are reduced, suppressor T cells (CD8 +) cannot reduce B cells to produce immunoglobulin (Ig), T cells produce increased IL-6, stimulate B cell proliferation, while IL-2 decreases, and B cell functions are active The blood Ig shows a polyclonal increase, and there are many autoantibodies in the body, such as antinuclear antibodies (ANA), anti-DNA antibodies, cold-reactive IgM anti-lymphocyte antibodies, IgG anti-neuro antibodies, anticardiolipin antibodies, rheumatoid factors, Autoantibodies can cause acute hemolytic anemia, vasculitis, and coagulopathy (thrombocytopenia, etc.), and the corresponding large amount of immune complexes can cause nephritis, heart and central nervous system diseases, and the complement system in this disease is activated. Blood complement is reduced, and complement components are often present in kidney tissue, especially Clq, suggesting activation of the classical pathway.
3. Hormones
The disease is more common in women, and the incidence of women is 5-9 times that of men. Pregnancy and oral contraceptives can aggravate the condition, suggesting that the disease has an estrogen-mediated immune regulation disorder. The blood testosterone levels in children are often reduced. Children with SLE Serum follicle stimulating hormone, luteinizing hormone and prolactin were higher than normal.
4. Environmental factors
Virus-like inclusion bodies can be found in the patient's endothelial cells, and antiviral antibodies can be detected in the glomerular eluate, which indicates that the disease is related to viral infection. It has also been clinically observed that infection can induce the disease, and the infection is simple Herpes virus can increase serum Sm antibodies in patients, but the role of infection in the pathogenesis of SLE is unclear. Various viral antibodies can be detected in patients' blood, especially antibodies such as anti-rubella virus, EB virus and paramyxovirus. , May suggest polyclonal B-cell activation, rather than a specific immune response. Ultraviolet radiation can induce or aggravate the condition. Certain medication allergies and strong psychological and emotional states can aggravate the condition.

Clinical manifestations of systemic lupus erythematosus in children

General symptoms
Fever, irregular heat pattern, general discomfort, fatigue, poor appetite, weight loss, hair loss, etc.
Rash
Symmetrical cheek-shaped erythema, across the bridge of the nose, with clear edges, slightly higher than the skin, and increased sun exposure; erythema-like maculopapular rash on exposed parts such as the upper chest and elbows; Erythema lateralis, erythema perineum, and erythema distally under the nail are all caused by vasculitis. There may also be skin bleeding and ulcers. Pay special attention to the presence of ulcers in the nasal cavity and oral mucosa.
3. Joint symptoms
Joint and muscle pain, joint swelling and deformity.
4. Heart
It can affect the endocardium, myocardium, and pericardium, and can manifest as heart failure.
5. Kidney
From focal glomerulonephritis to diffuse proliferative glomerulonephritis, severe cases can die from uremia. Kidney involvement can also be the first symptom.
6. Multiple serositis
It can affect the pleura, pericardium, and peritoneum. It can be affected alone or at the same time, and generally does not leave sequelae.
7. Nervous system
Headaches, personality changes, epilepsy, hemiplegia, and aphasia.
8. Other
Swollen liver, spleen, and lymph nodes may have symptoms such as cough, chest pain, and dyspnea.
9. Lupus crisis
Lupus crisis is a systemic disease that occurs rapidly due to extensive acute vasculitis and is often life-threatening. Children are more prone to crisis than adults, manifested by:
(1) Sustained high fever, ineffective with antibiotics.
(2) One of the following manifestations of an outbreak or acute attack: systemic extreme failure with severe headache; severe abdominal pain, often similar to acute abdomen; bleeding spots under or around the fingertips; severe oral ulcers
(3) Progressive decline in renal function with hypertension.
(4) Lupus pneumonia or pulmonary hemorrhage occurs.
(5) manifestations of severe neuropsychiatric lupus.

Pediatric systemic lupus erythematosus examination

Blood image
Leukocyte count decreased, often <4 × 10 9 / L, lymphocyte decreased, often <1.5 × 10 9 / L, varying degrees of anemia, anti-human globulin test (Coombs test) can be positive, platelets are generally normal, and can be reduced.
2. Antinuclear antibodies
Most are peripheral and spotted, with anti-dsDNA antibodies, anti-DNP antibodies, anti-Sm antibodies, anti-Ro (SSA) antibodies, anti-La (SSB) antibodies, etc.
3. Immunological examination
C3 decreased; IgG increased significantly, IgA and IgM also increased, gamma globulin increased, showing hypergammaglobulinemia; circulating immune complex assay was positive.
4. Urine routine
There are urinary protein, hematuria, and cast urine, and liver and kidney function can be abnormal.
5. Lupus band test
A small piece of skin was obtained by biopsy and observed by direct immunofluorescence. It was found that there were particles or linear fluorescent bands at the boundary 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.
It has been reported in recent years that transcranial Doppler ultrasound (TCD) can be used to diagnose lupus encephalopathy in children. It is believed that TCD is effective, simple, non-invasive and cost-effective, which is helpful for long-term follow-up observation of SLE. TCD provides a sensitive and specific method for the detection of early functional changes of cerebrovascular disease in lupus encephalopathy.

Diagnosis of systemic lupus erythematosus in children

The diagnostic criteria for SLE in children are the same as those for adults. The diagnostic criteria for SLE revised by the American Society of Rheumatology in 1982 are often used. Its 11 diagnostic conditions include:
1. Butterfly erythema on the cheek;
Discoid erythema
3. Light sensitive;
4. Oral or nasal ulcers;
5. Non-invasive arthritis;
6. Nephritis (hematuria, proteinuria> 0.5g / d, cell cast);
7. Encephalopathy (seizures or mental symptoms);
8. pleurisy or pericarditis;
9. Hemocytopenia (anemia, leukopenia, thrombocytopenia);
10. ANA positive (anti-dsDNA antibody, anti-Sm antibody) or lupus cells positive, or false positive of continuous syphilis serum reaction;
11. Positive fluorescent antinuclear antibodies.
SLE can be confirmed if 4 or more of the above conditions are met.

Differential diagnosis of systemic lupus erythematosus in children

Rheumatoid disease
Such as rheumatoid arthritis manifested as symmetrical joint swelling and pain, may have progressive deformity, rarely kidney damage, RF factor high titer positive, but anti-ds-DNA antibodies and anti-Sm antibodies are mostly negative.
2. Polymyositis and Dermatomyositis
Myalgia and muscle weakness are obvious, muscle enzyme spectrum is significantly increased, renal damage is less, and anti-ds-DNA antibodies and anti-Sm antibodies are mostly negative.
3. Nodular polyarteritis
The skin changed to a subcutaneous nodule, an increased white blood cell count, and ANA was negative.
4. Mixed connective tissue disease
Generally, there is a swelling-like swelling of fingers, more severe Raynaud phenomenon, severe muscle inflammation, high titer of anti-RNP antibody, and negative anti-Sm antibody.
Other diseases that need to be identified include: vasculitis, bacterial or viral infections, various types of kidney disease, chronic active hepatitis, blood diseases such as thrombocytopenic purpura, and hemolytic anemia.

Complications of systemic lupus erythematosus in children

Infection
Patients with systemic lupus erythematosus have low cellular immunity and are prone to colds and infections. Infection can cause or worsen lupus. Common bacterial infections include tonsillitis, bronchitis and pneumonia, urinary tract infections, pyelonephritis, cholecystitis, folliculitis, erysipelas, suppurative peritonitis, and sepsis.
2. Chronic renal failure
Lupus nephritis lesions can persist for many years, recurring and aggravating the condition, may eventually lead to renal failure. Patients with increased urea nitrogen and uric acid, such as short duration, can be reduced to normal through treatment.
3. Hypoproteinemia
In patients with chronic lupus nephritis, due to a large amount of proteinuria, protein loss will occur, and edema will develop from the back of the foot, ankle, calf, and thigh to the genitals, and diffuse edema in the hips, waist, and abdomen.

Pediatric systemic lupus erythematosus treatment

General treatment
Rest in bed, strengthen nutrition, low-salt diet, avoid sun exposure and vaccination, and use various drugs with caution, so as not to induce disease activities and prevent infection.
Glucocorticoid
Prednisone, total amount 60mg, taken in divided doses; disease control, laboratory tests were basically normal and changed to low-dose maintenance therapy, which must last for several years. In severe cases, methylprednisolone impact therapy is available. Pay attention to blood pressure and add vasodilators if necessary.
3. Immunosuppressive
Commonly used drugs are cyclophosphamide, azathioprine and methotrexate. Because these drugs do not control SLE activity as quickly as hormones, they are not recommended as the sole or first choice for treating SLE. Cyclophosphamide is effective for all types of SLE, especially severe kidney damage such as diffuse proliferative nephritis, central nervous system and lung damage. Early combined use with hormones is the key to reducing mortality and improving quality of life. Some people believe that cyclophosphamide intravenous shock therapy is an effective method to reduce renal fibrosis and stabilize renal failure. Most scholars believe that while intravenous cyclophosphamide treatment should be emphasized the use of a large amount of input balance fluid, that is, hydration therapy, in order to be more secure. During the treatment of SLE, pay attention to the following points:
(1) Acute renal failure: When the creatinine clearance is less than 20ml / min, cyclophosphamide shock can be performed after the methylprednisolone shock is relieved.
(2) Cyclophosphamide (CTX) has had a severe infection in the last 2 weeks, or the white blood cell count was <4 × 10 9 / L, or was allergic to cyclophosphamide, or used other cells such as immunosuppressants within 2 weeks. Nephrotic syndrome manifestations, when serum albumin <2g / L, cyclophosphamide should be used with caution. Since the onset of SLE in children is between 11 and 15 years of age, pubertal development should be considered during treatment. At present, in lupus nephritis, azathioprine can be maintained after the application of CTX shock to treat the disappearance of urinary protein.
(3) Methotrexate (MTX) and azathioprine can be used in combination with hormones, respectively, which have a good effect on controlling the activity of SLE and reducing the amount of hormones. treatment.
(4) Cyclosporine (CsA) In recent years, cyclosporine or mycophenolate mofetil has been used to treat lupus nephritis.
4. Symptomatic treatment
Joint symptoms should be treated with non-steroidal anti-inflammatory drugs and skin symptoms combined with hydroxychloroquine.
5. Other
In severe cases, intravenous immunoglobulin (IVIG) and plasma exchange can be used. CD34 + cell transplantation has also been reported in the treatment of severe SLE.

Prognosis of systemic lupus erythematosus in children

The prognosis of SLE in children is related to the degree of disease activity, the type and progression of kidney damage, the manifestation of clinical vasculitis, and the involvement of multiple systems. Diffuse proliferative lupus nephritis (type IV) and persistent central nervous system disease have the worst prognosis.

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