Systemic lupus erythematosus basics

  SLE is an autoimmune disease of unknown cause, characterized by multisystem or organ lesions and the presence of multiple autoantibodies in the serum.  SLE skin and mucous membrane manifestations are diverse and can be broadly divided into two categories: (1) specific: pteroid erythema (35%), subacute cutaneous lupus erythematosus (10%), discoid erythema (20-30%) and neonatal lupus.  Non-specific: photosensitivity (16-58%), alopecia (30%), oral ulcers (20%), cutaneous vasculitis (10-50%), Raynaud’s phenomenon (30-40%), urticarial rash (10%), rarely lupus lipofuscinosis or deep lupus (2%), and maculopapular lupus erythematosus (0.2%).  SLE diagnostic criteria: refer to the 1982 ACR revised diagnostic criteria for SLE: Criteria 1, zygomatic erythema Flat or elevated erythema of fixed skin across the zygomatic area, often not involving the nasolabial folds 2, discoid erythema Raised erythema covered with keratinous scales and follicular emboli, old lesions may have atrophic skin scarring 3, photosensitivity Sun exposure-induced skin sensitization 4, oral ulcers Painless ulcers in the oral cavity or nasopharynx 5, arthritis non-erosive arthritis, involving 2 or more peripheral joints, characterized by joint swelling, pain or exudate 6, pluritis ① pleurisy: chest pain, pleural sassafras sounds or pleural exudate; or ② pericarditis: abnormal electrocardiogram, pericardial sassafras sounds or pericardial exudate 7, renal lesions ① proteinuria >0.5g/dl or >+++; or ② cellular tubularity: may be erythrocytes, hemoglobin, granular tubularity or mixed tubularity 8. neurological abnormalities ① convulsions: non-drug or metabolic disorders, such as uremia, ketoacidosis or electrolyte disorders; ② psychosis: non-drug or metabolic disorders, such as uremia, ketoacidosis or electrolyte disorders; 9. hematological abnormalities ① hemolytic anemia with reticulocytosis; or ② leukopenia <4x10 9 /L, at least 2 times; or ③ Lymphopenia <1.5x10 9 /L, at least 2 times; ④ Thrombocytopenia <100x10 9 /L (excluding drug effects) 10, immunological abnormalities ① positive lupus cells; or ② positive anti-double-stranded DNA; or ③ positive anti-Sm antibody; or positive syphilis serologic test 11, anti-nuclear antibody Immunofluorescence anti-nuclear antibody titer abnormalities or other experimental titers equivalent to this method, excluded (1) Non-steroidal anti-inflammatory drugs (NSAIDs): For patients with low-grade fever, joint symptoms, rash and pericardial and pleurisy, use with caution in patients with hematologic lesions.  (2) Antimalarials: Chloroquine 0.125/day, 0.25/day after one week, or hydroxychloroquine 200mg/day, 200mg after one week, 2 times/day, effective for rash, hypothermia, arthritis, mild pleuritis and pericarditis, mild anemia and WBC reduction, and combined with SS, use with caution in patients with ophthalmia. The main side effects are cardiac conduction disorders and retinal pigmentation, and regular electrocardiogram and ophthalmologic examination should be performed.  (3) Glucocorticosteroids: choose the same dose and dosage form according to the condition. Take prednisone as an example, a small dose (≤ 0.5mg/kg/d) is suitable for active SLE patients with damage to vital organs; a medium dose (0.5-0.75mg/kg/d) is suitable for those with high fever or mild damage to one vital organ; a large dose (1mg/kg/d) is suitable for those with malignant high fever or severe damage to one or more vital organs. For patients with fever, take the dose in divided doses; for patients without fever, take the dose in the morning and gradually reduce the dose to 5-10mg/d for maintenance after the condition is stabilized. For patients with severe disease, mega-dose shock therapy can be used, usually methylprednisolone 800-1000mg/d intravenously for 3-5 days, then change to regular amount of hormone, or repeat if necessary.  (4) Immunosuppressants ① CTX: effective for nephritis, pulmonary hemorrhage, CNS vasculitis and autoimmune hemolytic anemia at a dose of 800-1000mg, iv, once/month (or 100-200mg, iv or orally, once/day), changed to once/2-3 months after stabilization, with cumulative dose not exceeding 200mg/kg.  ② Azathioprine, 50-100mg/d orally, is useful for autoimmune hepatitis, nephritis, skin lesions and arthritis.  ③ MTX: 7.5mg-15mg, iv or oral, 1 time/week, effective for arthritis, pluritis and fever, reduced dosage for renal impairment, occasional side effects of enhanced photosensitivity.  ④ Cyclosporine A (CSA), 3-3.5mg/kg/d in 1-2 oral doses, currently used mainly for SLE patients who have failed to respond to other medications.  ⑤ Vincristine: 1-3mg, iv, 1 time/week, 3-4 times in a row, effective for thrombocytopenia.  (5) Other treatments: high-dose immunoglobulin shock, plasma exchange, for patients with severe disease, not controlled or tolerated by conventional treatment, or with contraindications.  Treatment of lupus nephritis ① Glucocorticoids: Different doses are used according to the patient's nephritis symptoms, pathology and degree of disease activity. (1mg/kg/d) is indicated for acute nephritis syndrome with progressive deterioration of renal function in the near future and pathology of crescentic or diffuse proliferative nephritis.  Immunosuppressant: CTX 800-1000mg plus saline 40ml, iv, 1 time / month, 6 times in a row, followed by 1 time / 2 weeks, the cumulative dose does not exceed 150-200mg/kg, has a significant improvement in the prevention of renal fibrosis, preservation of renal function and survival rate. syndrome, etc.  ③ Plasma exchange and immunosorbent therapy: for acute LN, rapid deterioration of blood Cr and Ccr, ineffective conventional treatment or serious toxic side effects or contraindication to hormones and immunosuppressants.  ④ High-dose immunoglobulin shock therapy: for active LN, immunocompromised combined with infection.  ⑤ Others: such as anticoagulants, total lymph node irradiation and traditional Chinese medicine, and dialysis for renal insufficiency.