Systemic lupus erythematosus
Systemic lupus erythematosus is a polygenic heterogeneous autoimmune disease with multifactorial involvement. The basic features include (1) the presence of multiple autoantibodies in the serum. (ii) Multiple systems may be involved. It is most commonly seen in women of childbearing age. The prevalence rate in China is about 7/100,000 people, which is higher than that in western populations.
I. Etiology
1. Genetic factors There are family tendencies and racial differences in the development of SLE.
2. Other factors such as infections, drugs, sex hormones, etc.
II. Pathogenesis
Based on genetic factors, the abnormal immune response is promoted under the influence of environmental factors or (and) sex hormones. A variety of autoantibodies such as anti-nuclear antibody, anti-double-stranded DNA antibody and anti-Sm antibody can be detected in SLE patients. Immune complex disease is the main mechanism causing tissue damage. For example, anti-DSDNA antibodies bind to circulating antigens to form immune complexes deposited in the glomerulus or form in situ complexes in the glomerular basement membrane, thus causing inflammation and the development of lupus nephritis.
Pathology
1. The basic pathological changes include fibrin-like degeneration, mucinous edema of the connective tissue matrix, and necrotizing vasculitis.
2. Characteristic pathological changes of damaged organs Glomerulonephritis Class V: membranous lesion type Class VI: glomerulosclerotic type
IV. Clinical manifestations
1, systemic symptoms: fever, general malaise, malaise, weight loss, etc.
2. Skin and mucous membranes: Pteroidal erythema is often diagnostic specificity. There may be photosensitivity phenomenon, hair loss, reticular cyanosis, oral ulcers, etc.
3.Joints and muscles: there may be joint swelling and pain, the most susceptible is the proximal interphalangeal joint of the hand, knee, foot and wrist joints can be involved. Asymmetrical.
4, kidney: there may be proteinuria, hematuria, tubular urine, leukocyturia, etc. The manifestations are acute nephritis, acute progressive nephritis, occult glomerulonephritis, chronic nephritis, and nephrotic syndrome.
5, cardiovascular: may have pericarditis, myocarditis, endocardial and valvular lesions, etc.
6.Lung: lupus pneumonia, pleurisy and pleural effusion are more common.
7. Nervous system: Brain damage may present with mental disorders and epileptiform seizures. Spinal cord damage may manifest as paraplegia, urinary and fecal incontinence or sensorimotor disorders. There may also be cranial nerve and peripheral nerve damage.
8.Hematological system: anemia, leukopenia, thrombocytopenia, etc.
9. Digestive system: loss of appetite, abdominal pain, vomiting, etc.
V. Laboratory tests
1. Patients often have anemia, leukocytopenia and thrombocytopenia, or show a decrease in whole blood cells. Blood sedimentation is often increased. Urinalysis is abnormal, such as proteinuria and hematuria. Urea nitrogen and creatinine in blood are elevated in severe renal impairment. In active cases, there is a significant decrease in serum complement C4, C3 and CH50, and circulating immune complexes in the blood may be elevated.
2.Immunological examination ANA is almost 100% positive when the disease is active. Anti-double-stranded DNA antibodies have high specificity for diagnosis, but the positive rate is low and is closely related to disease activity and kidney damage. Antibody potency decreases with disease remission, and anti-Sm antibodies are also called specific antibodies for this disease because of their high specificity, but the positive rate is low and can be used as a basis for retrospective diagnosis.
VI. Diagnosis and differential diagnosis
Internationally, the classification criteria proposed by the American College of Rheumatology in 1982 are more commonly used, with a specificity of 96.4% and a sensitivity of 93.1%.
Cheek erythema, discoid erythema, sun allergy, oral ulcer, arthritis, pluritis, renal lesions, neurological abnormalities, hematological abnormalities, immunological abnormalities
Diagnosis can be made by satisfying 4 of the above 11 items
Differential diagnosis Should be differentiated from rheumatoid arthritis, primary glomerulonephritis, and other diseases. Drug-related lupus.
VII. Treatment
Treatment principles: 1. early diagnosis and treatment. 2. treatment plan must be individualized and the toxic side effects (risk/benefit ratio) of drugs must be detected. 3. assess the degree of organ damage and disease activity. 4. regular comprehensive examination and maintenance treatment during the remission period.