1. Definition of SLE disease severity and lupus crisis
Mild SLE: SLE is clearly diagnosed and important target organs (including kidney, hematological system, respiratory system, cardiovascular system, digestive system and central nervous system) are not involved.
Moderate and heavy SLE: refers to the involvement of important organs and affects their functions.
Renal involvement: glomerulonephritis, acute glomerulonephritis, nephrotic syndrome.
Hematologic involvement: hemolytic anemia, granulocytopenia, thrombocytopenia, thrombotic thrombocytopenic purpura; Ma Wukai, Department of Rheumatology and Immunology, Second Affiliated Hospital of Guiyang Traditional Chinese Medicine
Neurological involvement: convulsions, impaired consciousness, coma, stroke, transverse myelitis, mononeuritis or polyneuritis, psychiatric symptoms, demyelinating syndrome
digestive system involvement: intestinal obstruction, mesenteric vasculitis, acute pancreatitis
respiratory system involvement: alveolar hemorrhage, pulmonary hypertension, pneumonia, interstitial fibrosis of the lungs
cardiovascular system involvement: pericardial tamponade, myocarditis, etc.
Others: cutaneous vasculitis, severe skin damage, myositis, etc.
Definition of lupus crisis: life-threatening acute and severe SLE is called lupus crisis, and the main clinical manifestations include
Acute glomerulonephritis.
Severe central nervous system damage.
Severe hemolytic anemia.
Severe thrombocytopenic purpura.
Severe granulocyte deficiency.
Severe cardiac damage.
Severe lupus pneumonia or alveolar hemorrhage.
Severe lupus hepatitis.
Severe vasculitis, etc.
2.Treatment of mild SLE
For the treatment of mild SLE, hormone is not the first choice of treatment drug.
Firstly, non-steroidal anti-inflammatory drugs and anti-malarial drugs are applied, and hormones can be considered after the treatment is ineffective.
Short-term local application of hormones can be used for the treatment of skin mucosal lesions, but the use of strong hormonal topical drugs for the face should be avoided as much as possible, and even if used, it should not exceed 1 week.
Hormones (prednisone ≤10mg/d, or methylprednisolone ≤8mg/d) can help control the disease, and usually have fewer side effects.
3.Treatment of moderately active SLE
The treatment of moderately active SLE is generally divided into 2 phases, namely induction of remission and maintenance therapy. Hormone combined with immunosuppressive therapy is recommended.
Induction remission therapy: The dosage of hormone is usually 0.5-1mg?kg-1?d-1 of prednisone (0.4-0.8mg?kg-1?d-1 of methylprednisolone), which can be taken in the morning, or in divided doses if acute symptoms such as persistent hyperthermia need to be controlled. Concomitant immunosuppressive drugs are usually required.
Maintenance therapy: After 4-8 weeks of induction remission treatment, the hormone is slowly reduced by 10% of the original dose every 1-2 weeks to prednisone 0.5 mg kg-1 d-1 (methylprednisolone 0.4 mg kg-1 d-1), and then the reduction rate is slowed down according to the condition.
If the condition permits, maintain the treatment dose: prednisone <10mg/d (methylprednisolone <8mg/d).
In the process of drug reduction, if the disease is unstable, the original dose can be maintained temporarily or increased as appropriate or combined with immunosuppressive therapy.
4.Treatment of heavy SLE
The treatment of severe SLE especially emphasizes individualized program and requires the combination of other immunosuppressive drugs.
Treatment of severe SLE is also divided into 2 phases, namely induction of remission and maintenance therapy.
Induction of remission: The hormone dosage is usually the standard dose of prednisone 1 mg?kg-1?d-1 (methylprednisolone 0.8 mg?kg-1?d-1), given at dawn. Type III, IV, V+III/V+IV lupus nephritis can be considered with intravenous methylprednisolone 500-1000 mg for 3-d shock treatment.
Maintenance therapy: 2 weeks after the disease is stabilized or within 8 weeks of treatment, the hormone is slowly reduced at a rate of 10% of the original dose every 1-2 weeks to 0.5 mg?kg-1?d-1 of prednisone (0.4 mg?kg-1?d-1 of methylprednisolone), and the reduction rate is slowed down according to the disease.
In the process of drug reduction, if the disease is unstable, the original dose may be maintained temporarily, or the dose may be increased or combined with immunosuppressive therapy as appropriate.
Immunosuppressants such as cyclophosphamide, azathioprine, methotrexate, mycophenolate, cyclosporine, tacrolimus, etc. can be used. Cyclophosphamide is one of the first-line drugs in the treatment of severe SLE, especially in patients with severe lupus nephritis and vasculitis.
The most classical regimens for the induction of remission in lupus nephritis are the American College of Rheumatology (ACR) regimen and the European League Against Rheumatism (EULAR) regimen.
The ACR regimen: intravenous cyclophosphamide (500-1000 mg/m2 once a month for 6 doses, then repeated every 3 months for 2 years) combined with methylprednisolone shock therapy (500-1000 mg/d for 3 d), followed by sequential prednisone therapy (0.5-1.0 mg?kg-1?d-1, tapered). This regimen evolved from the National Institutes of Health (NIH) regimen.
EULAR regimen: intravenous cyclophosphamide (500 mg every 2 weeks for 6 doses) combined with methylprednisolone shock therapy (0.5-0.75 mg/d for 3 d), followed by prednisone 0.5 mg?kg-1?d-1, tapered after 4 weeks, and reduced to prednisone ≤10 mg/d maintenance over 4-6 months.