Systemic lupus erythematosus (SLE) is an atopic autoimmune disease involving multiple factors including genetics, sex hormones, environment, and infection, with clinical manifestations of systemic involvement of various systems and organs, as well as the presence of large amounts of autoantibodies in the serum.
Currently, there are several hypotheses for the pathogenesis of lupus, but there is increasing evidence that environmental stimuli and infections induce exposure to autoantigens in a genetic background of peripheral immunodeficiency, along with increased secretion of interferon by plasmacytoid dendritic cells, resulting in increased presentation of antigens by immune cells and decreased negative feedback regulation by T lymphocytes, leading to slowed formation and clearance of immune complexes, leading to pathogenesis.
The main pathological changes of SLE are inflammatory reaction and vascular abnormalities, which can appear in any organ of the body. The basic pathological changes include: (1) fibrin-like degeneration of connective tissue: it is caused by the deposition of eosinophilic material composed of immune complexes and fibrin in connective tissue. (2) Necrotizing vasculitis: inflammation and necrosis of the vessel walls of medium and small vessels due to the deposition of immune complexes or direct antibody attack, with secondary thrombosis narrowing the lumen, leading to local tissue ischemia and dysfunction.
SLE is an autoimmune disease that affects all systems of the body, and its clinical manifestations are complex and diverse, varying widely from patient to patient. Common clinical manifestations include.
Systemic symptoms: fever, fatigue, malaise, weight loss, etc.
Skin and mucous membrane manifestations: butterfly erythema (characteristic), discoid erythema, polymorphic erythema, subacute skin damage, etc.; oral ulcers, nasal ulcers, etc.; photosensitivity, hair loss, etc.
Joint muscles: often symmetrical multi-joint pain and swelling, which may be accompanied by morning stiffness; myalgia and pressure pain in the proximal muscles of the extremities.
Urinary system: renal involvement, manifesting as lupus nephritis, with proteinuria, hematuria, tubular urine, renal hypertension, renal insufficiency, etc.
Central system: neuropsychiatric lupus, with only migraine, personality changes, memory loss or mild cognitive impairment in mild cases: severe cases may manifest as cerebrovascular accidents, coma, and persistent status epilepticus.
Hematologic system: anemia, leukopenia, and thrombocytopenia are often present.
Respiratory system: manifests as pleurisy, lung parenchymal infiltration, interstitial lung lesions, etc.
Cardiovascular system: manifests as pericarditis, myocarditis, endocarditis, and coronary vascular involvement.
Digestive system: gastrointestinal vasculitis, which manifests as abdominal pain, bloating, diarrhea, intestinal ischemia, intestinal obstruction, and autoimmune hepatitis.
The hallmark antibodies in lupus are: ① antinuclear antibodies, with a diagnostic sensitivity of 95% (a screening test for SLE) and a specificity of 65%. (ii) Anti-double-stranded DNA antibody, with a sensitivity of 70% and specificity of 95%. Its titer is related to disease activity and prognosis, as well as to lupus glomerulonephritis. (iii) Anti-Sm antibody, with a sensitivity of only 25% but a specificity of 99%.
Lupus crisis: It refers to acute life-threatening severe SLE, mainly including acute glomerulonephritis, neuropsychiatric lupus, severe thrombocytopenic purpura, diffuse hemorrhagic alveolitis and acute severe interstitial lung lesions, and severe mesenteric vasculitis.
Prevention and treatment of SLE.
Dietary attention: Patients with SLE should be given a balanced, healthy and nutritious diet. The current recommended dietary structure for patients is 50% to 55% carbohydrate, 15% protein and no more than 30% fat. Some foods such as fish oil have certain anti-inflammatory properties, and eating several meals of fish food per week has similar effects to taking aspirin. The buds and pods of some plants contain L-cadaverine, which enhances the immune inflammatory response in patients with autoimmune diseases and should be avoided. Vitamin D not only prevents osteoporosis, but also has a good regulatory effect on the differentiation of immune cells.
Take proper rest and exercise to avoid strain and muscle atrophy. Avoid light and sunlight to prevent photosensitivity.
Treatment: It cannot be cured at present, but proper treatment can lead to complete remission. Special emphasis is placed on early diagnosis and early treatment to avoid or delay irreversible tissue and organ pathological damage. ①Lighter forms of SLE, despite lupus activity, without significant visceral damage. Drug therapy includes NSAIDs and antimalarials, the latter of which is effective in reducing lupus activity and hormonal side effects. Small doses of hormones can be added depending on the condition. ②SLE in moderate to heavy cases is mainly divided into two stages: induction of remission and consolidation therapy. The purpose of induction of remission is to rapidly control the disease, stop or reverse the organ damage, and strive for complete remission of the disease. Glucocorticoids have powerful anti-inflammatory and immunosuppressive effects, and are the basic drugs for the treatment of SLE. Clinical use emphasizes individualization, and the dose standard for heavy-duty is prednisone 1 mg/kg*d, divided into 2~3 oral doses. Cyclophosphamide is is one of the effective drugs for the treatment of severe SLE. Macrolimus ethyl ester has shown a good efficacy/risk ratio as both induction and maintenance therapy for lupus nephritis. Methotrexate is mainly used in SLE with predominantly arthritis, myositis, plasmacytitis and skin damage and is well tolerated on long-term dosing. Azathioprine is mainly used for maintenance therapy and is more effective in pluritis, hematologic and rash. Cyclosporine is effective for lupus nephritis and hematologic involvement.
Prognosis: Compared to the past, there has been a significant improvement. With regular treatment, the 1-year survival rate is 96%, the 5-year survival rate is 85%, and the 10-year survival rate has exceeded 75%. The main causes of death in patients in the acute phase are severe damage to multiple organs and infection in SLE, especially in those with severe neuropsychiatric lupus and acute lupus nephritis. Chronic renal insufficiency, adverse reactions to drugs (especially long-term use of large amounts of hormones) and coronary atherosclerotic heart disease are the main causes of death in the distant stages of SLE.