Systemic lupus erythematosus scleritis

Introduction to systemic lupus erythematosus Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by unidentified etiology, involving multiple systemic organs, and characterized by anti-cell antibodies, cytoplasmic and cell membrane antigen antibodies. The high immunological activity of this disease is caused by abnormal immune activation and loss of self-tolerance. The genetic defects in immune regulation are the basis of many individuals. The external pathogenic factors include food, sex hormones, and mental factors. , ultraviolet rays, drugs (pteridine, procainamide, penicillin, sulfa drugs, gold preparations, phenytoin, isoniazid, methyldopa, etc.), microorganisms (bacteria, viruses, parasites), etc., genetic Lack of sexual complement and other defects can be susceptible to disease activity. basic knowledge Probability ratio: The proportion of scleritis in SLE patients is about 1%-5% Susceptible people: no specific population Mode of infection: non-infectious Complications: keratitis anterior uveitis glaucoma vasculitis central retinal vein occlusion

Causes of systemic lupus erythematosus scleritis

(1) Causes of the disease

The exact cause is unclear and may be caused by a combination of factors. The immunological activity of this disease is caused by abnormal immune activation and loss of self-tolerance. The genetic defects in immune regulation are the basis of many individuals. The external pathogenic factors include food, sex hormones, mental factors, ultraviolet rays, drugs (benazine, procainamide, penicillin, sulfa drugs, gold preparations, phenytoin, isoniazid, methyldopa, etc.) , microorganisms (bacteria, viruses, parasites), etc., genetic complement deficiency and other defects, can be susceptible to disease activity.

(two) pathogenesis

SLE is a disease mainly mediated by antibodies. The patient's serum has an anti-nuclear antibody (ANA), which is a lupus erythematosus factor, which reacts with its corresponding autoantigen, the DNA-histone complex in the nucleus, resulting in chemotaxis. Factor release, attracting white blood cells, white blood cells are affected by ANA, the nucleus is swollen, the chromatin structure is lost, the nuclear membrane is dissolved, and free bodies are formed. The small bodies contain various inflammatory mediators that cause damage, and the small bodies are engulfed by neutrophils. The inclusion bodies are formed in the cytoplasm, and the nucleus is squeezed to one side. It is purple-red in the blood smear staining. It is called lupus erythematosus cells, and the complex of ANA and DNA can cause capillary damage and skin on various parts. , joint, kidney and eye lesions.

Systemic lupus erythematosus prevention

Actively control systemic lupus erythematosus to prevent the occurrence of ocular complications in the body.

Systemic lupus erythematosus scleritis complications Complications anterior keratitis uveitis glaucoma vasculitis central retinal vein occlusion

SLE scleral inflammation can spread to adjacent tissues, causing keratitis, anterior uveitis, and secondary glaucoma. Some patients also have hard retinal exudation, vasculitis, central retinal vein occlusion, arteriolar stenosis, arteriovenous Cross compression, yellow spots and retinal scars.

Systemic lupus erythematosus scleritis symptoms common symptoms joint swelling pain sclera outer inflammation retinal hemorrhage protein urinary fatigue joint deformity keratitis keratoconjunctivitis dry muscle myasthenia weakness fever

1. The appearance of eye manifestations of the eye is very important for the judgment of SLE. The eye lesions can not only be the first manifestation of SLE, but also the judgment of the severity and prognosis of the disease, and the invasion of other connective tissue diseases. Unlike SLE, which affects almost any part of the eye, any patient with retinal vasculitis or scleritis must be carefully examined for SLE.

(1) Scleritis: It is reported that the incidence of scleritis in SLE patients is 1%. The occurrence of scleritis is a more accurate sign of SLE systemic activity. When the whole body condition worsens, scleritis is intensified and recurrent, and scleritis is still Can be the first manifestation of SLE, SLE scleritis often manifests as diffuse or nodular anterior scleritis, especially in the case of systemic vasculitis, necrotic anterior scleritis can occur, although posterior scleritis is rare, but also SLE One of the clinical manifestations of the eye, any scleritis is part of the systemic damage of SLE, and as SLE systemic disease is controlled, scleritis will also be relieved.

(2) Scleral epidemic: SLE occurs in Sclera epithelium, and 159 cases of scleral epithelium observed by Watson and Hayrhh have no SLE, but scleral inflammation is the first symptom of SLE.

(3) Other eye lesions: In addition to scleritis and scleral inflammation, SLE patients may also have keratitis, conjunctivitis and anterior uveitis in the anterior segment of the eye. Conjunctivitis may affect the sputum, the ball and the conjunctiva of the iliac crest. Subcutaneous fibrosis, which leads to conjunctival retraction, the most common keratopathy is superficial punctate keratitis, corneal stromal infiltration, marginal corneal ulcer and vasospasm, keratoconjunctivitis (Sjögren syndrome) can be accompanied by SLE , but rarely seen, although anterior uveitis rarely occurs, anterior uveitis can be secondary to glaucoma, the condition is not heavy, and effective for appropriate treatment, posterior segment lesions are more serious than the anterior segment lesions, this About 5% of cases have fundus changes, and the fundus changes are not characteristic. They cannot be used as an important basis for diagnosis. However, their appearance or disappearance is often used as a good indicator to assess the severity and prognosis of systemic diseases. The most common ocular posterior segment lesion is Fundus cotton-like exudate, retinal hemorrhage and retinal or optic disc edema, retinal vein tortuosity, cotton-like plaques are located in the posterior pole and its vicinity, 1 to several, size 1/4 ~1PD, this cotton-like plaque is the occlusion of the anterior arterial vasculitis of the retinal capillaries, which causes the capillary bed to be caused by ischemia. The retinal hemorrhage is often located near the optic disc or the macula; most of them are flaming small hemorrhage scattered in the nerve fiber layer. Spot, there are also large or deep hemorrhage, the degree of retinal or optic disc edema is generally not serious, some of the above performance is similar to hypertensive retinopathy, can also be an independent sign of SLE, many scholars emphasize that the whole body of retinopathy and disease There is a relationship between seizures, Rothfield found that retinal hard exudation is associated with central nervous system diseases (epilepsy and organic encephalopathy), and Regan and Foster believe that retinal vasculitis can occur when SLE is not worse, no matter which In the case of SLE patients with retinopathy should be promptly performed systemic examination, if active lesions, immediate treatment, control of systemic diseases and eye abnormalities, SLE central nervous system vasculitis can produce nuclear ophthalmoplegia, nystagmus , II, III, V and VII on cerebral palsy, ipsilateral hemianopia and optic disc edema.

2. Non-ocular manifestations SLE is highly diverse in the occurrence or development of the disease. The clinical manifestations of the patients have obvious individual differences. Most patients have fatigue, anorexia, weight loss, fever and general malaise at the early stage of diagnosis. In the course of the disease, 1 or 2 of the above symptoms recurrence, often suggesting disease activity, fatigue is a difficult symptom to assess, but often indicates that the disease is about to recur, SLE can invade any organ of the body, the most common Clinical manifestations of affected organs.

The characteristics of SLE extraocular tissue and organ involvement are as follows:

(1) Muscle, joint disease: more than 95% of cases can affect the joints, joint swelling and pain as the first symptom, with a transient characteristics, a certain joint pain can last from a few minutes to several days, with the delay of the disease And worse, the pain lasts, arthritis occurs, arthritis is often symmetrical, the affected joints are mostly proximal knuckles, wrist joints, knee joints, although SLE rarely occurs in erosive arthritis, but 10% to 15 % of patients developed joint deformities similar to RA. About 50% of active patients had myalgia, muscle weakness and muscle tenderness, and 5% to 7% of patients had rheumatoid nodules.

(2) Skin lesions: 80% of SLE patients have skin lesions, 20% of patients have first-episode clinical manifestations, the most common lesions in the skin exposed parts, symmetrical rashes, typical butterfly erythema on both cheeks and nose The skin lesion is irregular edematous erythema, the surface is smooth, the edge is clear or blurred, and it is persistent or intermittent. Under ultraviolet irradiation, alcohol intake or mental stress is aggravated, and maculopapular rash and hair loss may occur. A week, erythema and atrophic pale lesions, urticaria, angioedema, bullous lesions, nail deformation and oral ulcers, oral ulcers and hair loss are more common in 25% to 40% of active patients.

(3) Vascular lesions: SLE is the most common vasculitic connective tissue disease after RA, including skin, mucosal vascular damage, gangrene, deep vein thrombosis, Rayo phenomenon in 20% of patients, and other clinical Simultaneous performance, with the improvement of the condition to reduce or disappear, peripheral and central nervous system damage and small arteritis can lead to organ infarction, such as cerebral embolism and hemorrhage, myocardial infarction, diffuse proliferative lupus nephritis, intestinal perforation, etc. The patient developed systemic arteritis similar to nodular polyarteritis.

(4) Heart disease: The most common cardiac manifestation of SLE is pericarditis, but no serious consequences, myocardial involvement, coronary death caused by myocardial infarction, even in 20-year-old women, atypical warts Biological endocarditis, also known as Libman-Sacks endocarditis, was first described by Libman and Sacks in 1924 as a neoplasm observed on the tricuspid valve, but in recent years it was found to be more common in the mitral valve, and some patients were in Libman. -Sacks endocarditis based on secondary infection, can cause subacute bacterial meningitis, ECG examination can find ST-T changes, but rarely pulmonary hypertension and heart failure.

(5) Kidney disease: Strictly speaking, almost all SLE involve the kidney. In most cases, the degree of kidney damage is mild and there is no clinical manifestation, while in other patients, although there is kidney damage, there is no renal dysfunction, only 40 % to 50% of patients with renal damage also have renal dysfunction (proteinuria or hematuria, nephrotic syndrome or renal failure), clinical pathological classification of lupus nephritis can be divided into:

1 local proliferative lupus nephritis (mild).

2 diffuse proliferative lupus nephritis (severe).

3 membranous lupus nephritis (moderate.

4 mesangial lupus nephritis (slight), coexistence of lupus nephritis and hypertension can lead to fatal consequences, untreated hypertension exacerbates renal damage, the extent and extent of renal damage is associated with the severity of SLE lesions Late renal disease is the leading cause of death in SLE patients.

(6) Neurological diseases: Central nervous system damage is the second cause of death in SLE patients. The most common central nervous system symptoms are psychiatric symptoms (organic brain syndrome), and epilepsy is the second common type of neurological damage. Performance, other rare hemipares, aphasia, chorea, periodic headaches and cerebellar symptoms, can occur with other lesions in the worse of SLE, and often have cranial neuropathy (optical nerve, trochlear nerve and abduction) during SLE activity and vasculitis More common neuropathy, less common trigeminal and facial neuropathy) and peripheral neuritis.

(7) Pulmonary lesions: 50% to 70% of patients with SLE have pleural and pulmonary lesions, manifested as exudative or dry pleurisy, acute pneumonia and diffuse interstitial pneumonia, exudate of exudative pleurisy per The protein content in 100ml exceeds 3g, the glucose content exceeds 55mg, occasionally lupus cells, chest pain is rare, SLE acute pneumonia is often caused by infection, accompanied by breathing difficulties, less common cough or hemoptysis.

(8) Other lesions: SLE patients may have gastrointestinal symptoms, loss of appetite, nausea, vomiting and abdominal pain. Diffuse abdominal pain with or without rebound pain may indicate peritonitis and mesenteric arteritis. Acute pancreatitis may come from In patients with acute SLE or glucocorticoid therapy, acute SLE usually has less severe hepatic enlargement, elevated liver transaminases, and soon becomes normal after treatment. 50% of SLE patients have lymph node lesions, 20% have light, medium The spleen is accompanied by an increase in polyclonal immunological activity, which is sufficient to explain the disorder of lymphatic hyperplasia. There is almost always a blood system abnormality in active SLE, mostly due to positive cell anemia caused by red blood cell destruction, and significant hemolysis can occur in 10% or less patients. The patient has mild thrombocytopenia and a small number of patients may have parotid swelling.

Systemic lupus erythematosus scleritis examination

No specific laboratory test means that lupus cells are phagocytic cells with large uniform inclusion bodies formed by neutrophils phagocytosis of nucleoprotein (DNA-histone) interacting with ANA, 70% of patients with SLE~ 80% can detect lupus cells, ANA is more sensitive and more specific than lupus cell examination, 98% of active phase and 97% of remission SLE patients are ANA positive, and ANA titer and SLE severity and activity have Correlation, but ANA does not have absolute specificity for SLE. Other autoimmune diseases, such as acute viral infections, chronic infectious diseases, ANA positive, ANA negative can not negate the diagnosis of SLE, SLE can appear a variety of ANA, such as DNA histone antibody, double stranded DNA (dsDNA) antibody, single stranded DNA (ssDNA) antibody, anti-RNA antibody, anti-histone antibody, nuclear ribonucleoprotein (nRNP) Antibody, anti-SM antibody and anti-cardiolipin antibody, dsDNA antibody is 70% positive in SLE, measured by radioimmunoassay (Farr method), normal binding rate is below 20%, antibody titer decreases with disease remission, Sm antigen An acidic nuclear protein soluble in physiological saline in the nucleus. The anti-Sm antibody is positive in about 30% of SLE patients. Therefore, the antibody is almost only positive in SLE, so it is called labeled antibody of SLE, and anti-nRNP antibody can be found in In patients with scleroderma, connective tissue disease, and polymyositis, anticardiolipin antibodies are associated with false positives in some SLE syphilis tests, and patients with anticardiolipin antibodies are more likely to have thrombosis and recurrent spontaneous abortions. In addition, some Anti-clotting factors VIII, IX, XI and XII antibodies, anti-platelet antibodies and anti-immunoglobulins (such as RF) can also be detected in patients with SLE. These antibodies are non-specific and can be detected in other diseases.

Decreased complement levels (C3, C4 and total complement levels decreased) and anti-DNA antibody positives have a higher specificity for the diagnosis of SLE, low complement levels, serum condensed globulin and CIC are associated with aggravation of SLE.

Lupus band test (LBT): The direct fluorescence method is used to deposit immunoglobulin and complement in the epidermis and dermal junctions of patients. It is a granular, spherical or linear arrangement of bright yellow-green fluorescence. Band, in the normal skin exposed parts of SLE, the positive rate is 50% to 70%, and the lesion area can be as high as 90% or more. IgG deposition is more diagnostic than IgM deposition.

Active nephritis can have proteinuria, cell tubular urine and microscopic hematuria. SLE patients should be regularly checked for serum creatinine and urine routine, positive pigmented anemia, about 50% of patients with white blood cells <4000/mm3, neutrophils and lymphocytes Absolute counts of cells are reduced, 1/3 of patients have thrombocytopenia, 5% to 10% of patients with hemolytic anemia, reticulocyte increase, low hematocrit or positive Coombs test can predict that SLE disease will progress, and other systemic lesions will appear.

During the SLE active cerebrospinal fluid examination, the protein was increased, the neutrophils and lymphocytes were slightly elevated, and the complement C4 was decreased.

1. X-ray examination: diffuse alveolar infiltration at the base of the lung appears when there is active lesion in the lung.

2. Ultrasound examination: the pericardial thickening is shown when the lesion involves the pericardium.

Diagnosis and differentiation of systemic lupus erythematosus scleritis

Any patient with joint pain and multiple system involvement should be suspected of having SLE. The current international application is the diagnostic criteria proposed by the American College of Rheumatology in 1982 (Table 2). Those who meet 4 or more items may Diagnosing SLE, but as far as the individual is concerned, those who meet the criteria are not necessarily SLE. Conversely, those who do not meet the criteria may not be able to rule out SLE. Some early, light, and atypical SLEs are even more so. Based on the above criteria, the standard recommended by the Chinese Rheumatology Association is that 4 of the 13 items can be diagnosed, with a specificity of 93.6% and a sensitivity of 97.3%. The 13 criteria are:

1 butterfly erythema or disc erythema.

2 light allergy.

3 oral ulcers.

4 non-arthritis or joint pain.

5 serositis (pleuritis or pericarditis).

6 nephritis (proteinuria, tubular urine or hematuria).

7 nervous system damage (convulsions and mental symptoms).

8 abnormal blood (WBC <4000 / mm3) or platelets < 80,000 / mm3 (or hemolytic anemia).

9 lupus cells or dsDNA antibodies are positive.

10 anti-Sm antibody positive.

11ANA is positive.

12 lupus belt test positive.

13C3 complement is lower than normal.

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