SLE is an autoimmune disease of unknown cause, characterized by multisystem or organ lesions and the presence of multiple autoantibodies in the serum, with a peak age of 15-45 years and a 9-13 times higher incidence in women than in men.
SLE skin and mucous membrane manifestations are diverse and can be broadly divided into two categories: specific and non-specific.
(1) Atopic: 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%).
Diagnostic criteria for SLE: Refer to the 1982 ACR revised diagnostic criteria for SLE.
Criteria
1 Zygomatic erythema Flat or elevated fixed erythema over the cheek area, often without involvement of the nasolabial folds
2 Discoid erythema Raised erythema covered with keratinous scales and follicular plugs; old lesions may have atrophic skin scarring
3 Photosensitivity skin irritation caused by sun exposure
4 Oral ulcers Painless ulcers of the oral cavity or nasopharynx
5 Arthritis non-erosive arthritis involving 2 or more peripheral joints, characterized by swelling, pain or oozing of the joints
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: can be red blood cells, hemoglobin, granular tubularity or mixed tubularity
8 neurological abnormalities ① convulsions: non-drug or metabolic disorders, such as uremia, ketoacidosis or electrolyte disorders caused by; ② psychosis: non-drug or metabolic disorders, such as uremia, ketoacidosis or electrolyte disorders caused by
9 Hematologic 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 (except drug effects)
10 Immunologic 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 Immunofluorescent anti-nuclear antibody titer abnormalities or other experimental titers equivalent to this method, excluding drug-related lupus
SLE drug treatment
( 1 ) Non-steroidal anti-inflammatory drugs (NSAIDs): For patients with low 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, but 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, small dose (≤ 0.5mg/kg/d) is suitable for active SLE patients with damage to important organs; medium dose (0.5-0.75mg/kg/d) is suitable for those with high fever or mild damage to one important organ; large dose (1mg/kg/d) is suitable for those with malignant high fever or severe damage to one or more important 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, and repeat if necessary. ( 4 ) Immunosuppressant CTX: effective for nephritis, pulmonary hemorrhage, CNS vasculitis and autoimmune hemolytic anemia, dose 800-1000mg, iv, 1 time / month (or 100-200mg, iv or oral, 1 time / day), change to 1 time / 2-3 months after the condition is stable, the cumulative dose should not exceed 200mg/kg. (ii) Azathioprine, 50-100mg/d, orally, for autoimmune hepatitis, nephritis, skin lesions and arthritis. ③ MTX: 7.5mg-15mg, iv or oral, once/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 treatment: 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 Glucocorticoids: Different doses are used according to the patient’s nephritis symptoms, pathology and disease activity. A small dose (0.5 mg/kg/d) is suitable for LN with mild clinical symptoms, asymptomatic proteinuria or hematuria. A large dose (1 mg/kg/d) is suitable for active LN (nephrotic syndrome, pathology of diffuse hyperplasia, membrane hyperplasia or membranous nephritis). neonatal 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, on the prevention of renal fibrosis, renal function preservation and survival are significantly improved . CSA, 2.5-5mg/kg / d, divided into 2 oral doses, used in active LN, nephrotic syndrome, etc. . ③ Plasma exchange and immunosorbent therapy: for acute LN, rapid deterioration of blood Cr, Ccr, and those who are ineffective in conventional treatment or have serious toxic side effects or contraindication to hormones and immunosuppressants. ④ High-dose immunoglobulin shock therapy: for active LN, immunocompromised combined with infection. ⑤ Other: such as anticoagulants, total lymph node irradiation and traditional Chinese medicine, dialysis treatment is feasible in renal insufficiency.