Myelopathy due to systemic lupus erythematosus

Introduction

Introduction to systemic lupus erythematosus-induced myelopathy Systemic lupus erythematosus (SLE)-induced myelopathy is a manifestation of SLE in the spinal cord. SLE is a specific autoimmune disease involving multiple factors (genetic, sex hormones, environment, infection, drugs, immune response), which can affect multiple systems, so there are many symptoms of organ damage, and there are many irregularities at the beginning of the disease. Fever, the most common skin damage, butterfly erythema on the cheeks and nose, rash on the neck or trunk; in addition, often combined with joint pain and lymphadenopathy. SLE damage to the central nervous system is the leading cause of death, according to statistics at 20% to 60%, and some reports can be as high as 75% (Johnson, 1968). Recurrent episodes of convulsions are the most common, followed by cranial nerve disorders, mental disorders, cerebrovascular accidents, increased intracranial pressure and meningitis, involuntary movements, and peripheral nerve damage such as Raynaud's phenomenon. basic knowledge The proportion of sickness: 0.0052% Susceptible people: women Mode of infection: non-infectious Complications: hemolytic anemia thrombocytopenia

Cause

Causes of myelopathy caused by systemic lupus erythematosus

Genetic factors (25%):

SLE is a chronic inflammatory disease caused by immune imbalance. Its immunological characteristics are the activation of polyclonal autoreactive T cells and/or B cells, and the production of various autoantibodies. In recent years, more and more Studies have confirmed that genetic factors are associated with the pathogenesis of systemic lupus erythematosus, and apoptosis may have a role in the pathogenesis of SLE.

Immune abnormality (35%):

SLE has a variety of autoantibodies. These antibodies are both the result of immune dysfunction and the cause of pathological changes. Antibodies can mediate brain tissue damage, such as anti-neuronal antibodies associated with diffuse brain damage; studies have shown resistance in cerebrospinal fluid Lymphocyte antibodies can cause demyelination of nerve tissue, and it is speculated that spinal cord damage is related to this.

Vascular lesions (5%):

Experiments have shown that circulating immune complexes can mediate the release of vasoactive substances caused by platelet damage, while lupus anti-coagulant antibodies and anti-cardiolipin antibodies directly damage vascular endothelial cells. Vasculitis is one of the basic diseases of systemic lupus erythematosus. Stenosis or occlusion of the lumen causes extensive microinfarction, while long-term use of glucocorticoids can lead to changes in blood pressure and hemodynamics and early onset arteriosclerosis.

Systemic lupus erythematosus can be considered as an immune complex disease in a certain sense, and vasculitis is caused by this disease. The pathological changes of the disease in the spinal cord are similar to those in the brain, mainly vascular inflammatory lesions, and arteritis changes in the spinal cord and small arteries. Capillaries can be thickened by fibrosis, and there may be small hemorrhage. There is an inflammatory reaction around the blood vessels. It has been reported that the white matter of the spinal cord is subacute degeneration, and its myelin disappears and vacuoles change (vacuolar). Change) and so on.

Prevention

Prevention of myelopathy caused by systemic lupus erythematosus

Early diagnosis, timely and correct treatment; avoid induced factors.

Common predisposing factors are: sun exposure, ultraviolet radiation, cold stimulation can lead to recurrence of the disease; some patients with lupus are obviously related to drugs, such as penicillin, sulfonamides, phenylbutazone, hydralazine (hydrazinazine), Procainamide, chlorpromazine, phenytoin, isoniazid, oral contraceptives, etc., can cause patients with lupus erythematosus in remission to enter active and laboratory changes.

Complication

Systemic lupus erythematosus-induced complications of myelopathy Complications hemolytic anemia thrombocytopenia

There are typical manifestations of SLE, such as facial butterfly erythema, sun allergy, joint pain, heart, kidney and other multi-organ damage.

Symptom

Symptoms of myelopathy caused by systemic lupus erythematosus Common symptoms Facial butterfly erythema thrombocytopenia Spinal cord disease Photoallergy Leukopenia Secondary infection Paraplegia Involuntary movement Peripheral nerve damage Increased intracranial pressure

The clinical manifestations of spinal cord injury resemble acute myelitis, often located in the thoracic segment, most of which are sudden onset, rapid paraplegia, and can also manifest as flaccidity at the beginning of the disease, with a shallow sensory plane of loss, loss of position and vibration, or preservation. Some have a rising development, may have urinary retention, the prognosis of transverse myelitis is poor, often due to secondary infection and death, in addition, patients still need to have typical SLE performance, such as facial erythema, skin Allergic to sun, joint pain, heart, kidney and other organ damage.

Laboratory examination of lupus cells can be found in the blood, anti-cell component antibodies: such as anti-single-stranded DNA antibody (ssDNA), anti-double-stranded DNA antibody (dsDNA), anti-nuclear antibody (ANA) positive, cerebrospinal fluid examination pressure normal, some patients lymph The cells are slightly elevated, the protein content can be increased, generally rarely exceeds 1g/L, 50% of patients have elevated IgG, and sometimes the sugar content is reduced. Some people think that the sugar content is significantly reduced, which is a relatively specific change of transverse myelitis. Anti-neuronal IgG antibodies and anti-lymphocyte antibodies can be found.

SLE currently uses the classification criteria revised by the American College of Rheumatology in 1982.

1. Facial butterfly erythema.

2. Disc-shaped erythema.

3. Sun allergy.

4. Oral or nasopharyngeal ulcers.

5. Non-erosive arthritis.

6. Serositis.

7. Kidney damage.

8. Neuropathy seizures or psychosis.

9. Abnormal blood hemolytic anemia, leukopenia, lymphopenia or thrombocytopenia.

10. Immunological abnormal lupus cells positive, anti-ds-DNA antibody positive, anti-SM antibody positive, or false positive for anti-syphilis serum test for 6 months.

11. Positive for antinuclear antibodies.

Clinically, if other symptoms are excluded, if there are 4 or more of the above 11 criteria, it can be diagnosed as SLE.

Examine

Examination of myelopathy caused by systemic lupus erythematosus

1. Blood immunological examination of anti-single-stranded DNA antibody (ssDNA), anti-double-stranded DNA antibody (dsDNA), anti-nuclear antibody (ANA) positive, anti-Sm antibody and anti-Rib antibody are labeled antibodies, and do not represent disease activity Patients with positive anti-SSA and anti-SSB antibodies often have symptoms such as dry mouth and dry eyes. Patients with positive anti-RNA antibodies often have Raynaud's phenomenon, and 50% to 70% of patients are positive for anti-histone antibodies.

2. Cerebrospinal fluid examination pressure is normal, lymphocytes can be slightly elevated, protein content is rarely more than 1.0g / L, 50% of patients with elevated IgG, sometimes reduced sugar content, anti-neuronal IgG antibodies and anti-lymph Cellular antibodies.

3. ESR, blood routine, urine routine.

4. Serum complement C3, C4, CH50 Complement level is negatively correlated with SLE activity, serum complement is extremely low, disease is in advanced stage, and circulating immune complex: SLE active period is increased.

5. Serum protein electrophoresis active gamma globulin or 2, globulin increased, and returned to normal after treatment.

6. Antiphospholipid antibodies The presence of antiphospholipid antibodies can cause arterial and venous thrombosis; it can also cause thrombocytopenia and hemorrhage. In addition to encephalopathy, antiphospholipid antibodies can also cause stillbirth or miscarriage.

7. Skin lupus test The patient will have immune complex deposition at the junction of the epidermis and dermis of the skin. Taking a skin biopsy, the line-type grass green fluorescence at the junction of the epidermis and the dermis can be seen under a fluorescence microscope. The lupus belt test, lupus belt is helpful for the diagnosis of SLE, but it is not unique to SLE patients, but the strong positive test of lupus belt often indicates that SLE may be.

8. Spinal MRI examination, with differential diagnosis.

Diagnosis

Diagnosis and diagnosis of myelopathy caused by systemic lupus erythematosus

According to the typical clinical manifestations of SLE and the symptoms of spinal cord lesions, the diagnosis of this disease is not difficult.

Attention and acute myelitis, spinal cord tumor, spinal cord compression, etc., through laboratory tests and spinal MRI examination, identification is not difficult.

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