I. Diagnostic criteria.
According to the classification criteria revised by the American Rheumatism Association (ARA) in 1997, it consists of 11 items:
(1) Zygomatic-cheek erythema;
(2) discoid lupus;
(3) photosensitivity;
(4) Oral ulcers;
(5) Non-erosive arthritis;
(6) pleurisy or pericarditis;
(7) Proteinuria (>0.5 g/d) or urinary cell tubularity;
(8) Seizures or psychosis, except for drugs or known metabolic disorders;
(9) Hemolytic anemia or leukopenia, or lymphocytopenia, or thrombocytopenia;
(10) positive anti-dsDNA antibodies, or anti-Sm antibodies, or positive antiphospholipid antibodies (including one of anti-cardiolipin antibodies, or lupus anticoagulant, or false positive syphilis serologic test lasting at least 6 months); (11) anti-nuclear antibodies. Abnormal titers of antinuclear antibodies at any time and in the case of drug-induced “drug lupus”. If four or more of the 11 items of the classification criteria are met, SLE can be diagnosed after excluding infections, tumors and other connective tissue diseases.
II. Treatment principles
1.General treatment
Applicable to all SLE patients. It includes psychological and spiritual support, avoidance of sunlight or ultraviolet radiation, prevention and treatment of infection or other comorbidities, and selection of appropriate exercise according to the condition.
2.Medication
(1) Non-steroidal anti-inflammatory drugs (NSAIDS): for patients with low fever, joint symptoms, skin rash and pericarditis and pleurisy, and caution for those with hematologic lesions.
(2) Antimalarials: Chloroquine or hydroxychloroquine, effective for rash, hypothermia, arthritis, mild pleurisy and pericarditis, mild anemia and reduced blood leukocyte count and combined dry syndrome, use with caution in those with ophthalmia. Long-term application is useful for reducing hormone dose and maintaining remission. The main adverse effects are cardiac conduction disorders and retinal pigmentation, and electrocardiogram and ophthalmologic examination should be performed regularly.
(3) Glucocorticoids: Different doses and dosage forms are used according to the condition. Adverse effects of hormones include Cushing’s syndrome, diabetes mellitus, hypertension, various infections complicated by low resistance, stress ulcers, aseptic osteonecrosis, osteoporosis and growth retardation or stagnation in children.
(4) Immunosuppressants.
①Cyclophosphamide (CTX): effective in nephritis, pulmonary hemorrhage, central nervous system vasculitis and autoimmune hemolytic anemia. Adverse effects include gastrointestinal discomfort, bone marrow suppression, liver damage, hemorrhagic cystitis, alopecia, amenorrhea and reduced fertility.
② Azathioprine: orally useful for autoimmune hepatitis, nephritis, skin lesions and arthritis. Adverse effects include gastrointestinal discomfort, bone marrow suppression, liver damage and allergic reactions.
(③Methotrexate (MTX): taken quietly or orally, it is effective for arthritis, pluritis and fever, and the dosage needs to be reduced for renal impairment, with occasional adverse reactions of enhanced photosensitivity.
④Cyclosporine A (CSA): oral, currently used mainly for SLE patients who have failed to respond to other drug treatments.
⑤ Vincristine: static dosing, effective for thrombocytopenia.
(5) Other treatments: high-dose immunoglobulin shock, plasma exchange, for patients with severe disease, uncontrolled or intolerable by conventional treatment, or with contraindications.
(6) Treatment of lupus nephritis.
①Glucocorticoids.
②Immunosuppressants.
(3) Plasma replacement and immunosorbent therapy.
④High-dose immunoglobulin shock therapy is indicated for active lupus nephritis (LN) with immunocompromised co-infection.
⑤Others such as anticoagulants, systemic lymph node irradiation and Chinese medicine, and dialysis therapy is feasible for renal insufficiency.