Definition: Systemic lupus erythematosus (SLE) is an autoimmune-mediated diffuse connective tissue disease with immune inflammation as the prominent manifestation.
Clinical manifestations: SLE is more common in women of childbearing age, more in women than in men.
1. Systemic manifestations: fever, fatigue.
Skin and mucous membrane: butterfly erythema, photosensitivity, alopecia, palmar and perineal erythema, discoid erythema, erythema nodosum, lipofuscinosis, reticulocutaneous cyanosis, Raynaud’s phenomenon, etc. Oral ulcers or mucosal erosions are common in SLE.
3. Joints and muscles: symmetrical multi-joint pain and swelling, usually without causing bone destruction. Myalgia and muscle weakness may occur, and a few may have an increased muscle enzyme profile.
4. Renal damage: manifested as proteinuria, hematuria, tubular urine, and even renal failure.
5. Neurological damage: central nervous system manifestations include aseptic meningitis, cerebrovascular disease, demyelination syndrome, headache, motor disorders, myelopathy, seizures, acute psychosis, anxiety, cognitive disorders, mood disorders, psychotic disorders; peripheral nervous system manifestations include Green-Barre syndrome, plant nervous system dysfunction, mononeuropathy, myasthenia gravis, cranial neuropathy, neurological Plexiform lesions, polyneuropathy, and other lesions. Transverse myelitis is rare.
6. Hematologic manifestations: anemia and/or leukopenia and/or thrombocytopenia are common.
7. pulmonary manifestations: pleurisy, pleural effusion, pulmonary parenchymal infiltrates, interstitial lung disease, pulmonary hypertension, pulmonary infarction, pulmonary atrophy syndrome, hemoptysis in a few critically ill patients, diffuse hemorrhagic alveolitis with high mortality.
8. Cardiac manifestations: pericarditis often appears, manifested as pericardial effusion, but pericardial tamponade is rare. SLE may present with warty endocarditis, coronary artery involvement, angina pectoris and electrocardiogram ST-T changes, and even acute myocardial infarction.
9. Gastrointestinal manifestations: nausea, vomiting, abdominal pain, diarrhea or constipation, of which diarrhea is more common, may be accompanied by protein-losing enteritis and cause hypoproteinemia. SLE can also be complicated by acute pancreatitis. Increased liver enzymes are common, with only a few cases of severe liver damage and jaundice.
10. Other: Ocular involvement includes conjunctivitis, uveitis, fundus changes, optic neuropathy, etc. SLE is often accompanied by secondary dry syndrome with involvement of exocrine glands, manifesting as dry mouth and dry eyes.
11. Immunological abnormalities: positive anti-nuclear antibodies, including positive anti-double-stranded DNA antibodies and positive anti-Sm antibodies have high diagnostic specificity. Other diagnostic values such as positive antiphospholipid antibodies, hypocomplementemia, positive direct anti-human globulin test, positive skin lupus band test, and kidney immunofluorescence showing multiple immunoglobulin and complement components deposition are also available.
How to diagnose: The diagnosis is established with evidence of multisystem involvement, combined with detection of specific autoantibodies, renal puncture biopsy, and exclusion of other diseases that may cause multisystem involvement.
How to treat.
1. General treatment
(1) Patient education: correct understanding of the disease, elimination of fear, understanding of the significance of regular medication, learning to recognize the signs of disease activity, cooperation with treatment, compliance with medical advice, and regular follow-up. Understand the necessity of long-term follow-up. Avoid excessive exposure to ultraviolet light, use UV protection (sunscreen, etc.) and avoid overexertion.
(2) Symptomatic treatment and removal of various factors affecting the prognosis of the disease, such as attention to the control of hypertension and prevention of various infections.
2. Drug treatment.
(1) glucocorticoids; (2) cyclophosphamide; (3) azathioprine; (4) methotrexate; (5) cyclosporine; (6) mycophenolate; (7) hydroxychloroquine; (8) monoclonal antibodies (such as anti-CD20 antibody, anti-CD22 antibody, anti-CTLA-4 antibody, anti-BlyS antibody, etc.).
3. Other treatments: plasma exchange, immunosorbent, etc.
Prognosis: Irregular follow-up, non-compliance with medical advice and non-standardized treatment are important causes of death. In recent years, the prognosis of SLE has been significantly improved compared with the past due to the strengthening of patient education and the improvement of treatment level. 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 stage are severe multi-organ damage and infection in SLE, especially those with severe neuropsychiatric lupus and acute lupus nephritis; chronic renal insufficiency, adverse effects of drugs (especially long-term use of high-dose hormones) and coronary atherosclerotic heart disease are the main causes of death in the distant stage of SLE.