Behcet's disease
Introduction
Introduction to Behcet's disease Behcet is a multi-system chronic damaging syndrome. It is characterized by genital ulcers and recurrent uveitis in addition to recurrent oral ulcers. basic knowledge The proportion of illness: 0.0035% Susceptible people: no specific people Mode of infection: non-infectious Complications: retinal vein occlusion retinal vasculitis
Cause
Cause of Behcet's disease
The cause of Behcet's disease is not yet clear. Some people think that it is caused by viral infection or allergic reaction to infectious factors, but it has not been fully proved. Recently, there have been many studies in immunology, and it has been found that some patients have elevated serum immunoglobulins and immune complexes, and have various antibodies, such as anti-oral mucosal antibodies and anti-arterial wall antibodies. Therefore, some people think that this disease may be some factors, including an autoimmune disease induced by the virus.
Prevention
Behcet disease prevention
Controlling the development of fundus lesions is essential for the treatment of this disease and for preventing blindness. In the treatment of corticosteroids and antimetabolites, care should be taken to avoid serious side effects such as myelosuppression and corticosteroid discontinuation.
Complication
Behcet disease complications Complications retinal vein occlusion retinal vasculitis
The main cause of blindness is the complications of the fundus, such as complicated retinal vein occlusion. It has been thought that this disease must be accompanied by anterior chamber empyema, which is rarely seen in clinical practice. It is more common in the fundus, and often accompanied by retina. Vascular inflammation can cause macular edema and even cause retinal vascular occlusion, which can seriously affect vision.
Symptom
Symptoms of Behcet's disease Common symptoms Congestion block wounds acne-like rash
Mainly based on clinical signs:
1, the main signs: repeated oral, genital ulcers, skin disorders and various types of uveitis.
2, secondary signs: arthritis, gastrointestinal diseases, vasculitis and neurological diseases.
Clinical manifestations:
1, general malaise, fatigue, low fever, loss of appetite, genital, oral ulcers recurrent, skin nodular erythema changes.
2, eye performance: about 80% of patients with both eyes at the same time or successive uveitis, the course of disease for 10 to 20 years, the type is:
1 light iridocyclitis.
2 acute iridocyclitis, choroiditis.
3 acute total uveitis, which is characterized by recurrent granulomatous inflammation, fine KP, anterior chamber empyema, complicated cataract, secondary glaucoma, fundus manifestations of macular sac, edema, degeneration, central artery or venous obstruction Retinal edema, exudation, hemorrhage, optic nerve nipple congestion, atrophy, and finally can lead to permanent loss of vision.
Examine
Behcet disease check
[Laboratory Inspection]
1. Skin irritation test Forearm flexor injection of normal saline 0.1ml, 48h diameter red induration or small pustules, small papules were positive, suggesting that neutrophil chemotaxis is enhanced, the positive rate is about 40%.
2. C-reactive protein (CRP) determination of CRP increased before and after the onset of ocular inflammation, especially before the onset of the most obvious; increased CRP and increased neutrophil count, 1 week of positive eye attack The rate is 86.1%, so it is considered that the determination of CRP has a certain value for predicting the onset of ocular inflammation.
3. Erythrocyte sedimentation rate and white blood cell classification When the disease occurs, the erythrocyte sedimentation rate is significantly accelerated. The neutrophil ratio was also significantly increased.
4. Pathological examination The basic pathological changes of all affected organs were vasculitis. Most are exudative, a few are proliferative, or both. Acute exudative lesions manifested as luminal congestion, thrombosis, fibrin-like degeneration of the wall and surrounding tissues, and neutrophil infiltration and red blood cell spillover. Neutrophil nuclei often break into nuclear dust. There is obvious edema, cellulose exudation, and abscess formation. There are no exceptions to the proliferative pathology.
5. Serum fibrinolysis system and immunogenetics (small arm of chromosome 6) can be examined if necessary or necessary.
[Other inspections]
1. X-ray examination of digestive tract barium meal examination, patients with a course of less than 7 years, often combined with stomach, duodenal bulb and small intestine ulcer. Ulcer perforation can occur in individual cases. The small intestine may have different degrees of functional changes. It can be seen that the intestinal lumen is widened, the small intestine mucosa is thickened, and there is a segmentation phenomenon. Sometimes the jejunal intestinal wall is straight and sausage-like.
Patients with advanced disease over 10 years may have a wide polypoid filling defect in the stomach, small intestine and colon, which is obvious in the small intestine and colon, and may have proliferative unequal knots in the ileocecal and ascending colon. Nodular filling defects, mucosal folds disorder, pathological diagnosis of ulcerative gyrus, colitis.
Lung performance is less common. If the pulmonary vascular infarction or hemorrhage, often in the lung field or hilar area, there is a similar density of pneumonia, lung metastases, round or oval shadow, heart shadow can be increased. Gas cerebral angiography can have mild ventricular enlargement. Myelin adhesions can be found in myelography.
Angiography can detect segmental stenosis changes in different parts of the renal artery, mesenteric artery, and cerebral arteries.
2. Patients with ECG may have ST segment and T wave changes, most of which have sinus tachycardia.
3. When the EEG is involved in the nervous system, most of them show diffuse slow waves, but there are also many waveform changes without characteristic waveforms. Diffuse slow waves may occur in patients with symptoms of brainstem, and mild to moderate slow waves may occur in patients without brainstem symptoms.
4. Acupuncture reaction This is the only specific test with strong specificity in this disease. Its practice is to use a sterile intradermal needle to penetrate the skin in the middle of the flexion of the forearm, and then withdraw. After 48 hours, observe the skin reaction at the needle penetration. If there is red papule or red papule with white herpes, it is regarded as Positive result. At the same time, when a multi-site acupuncture test was performed, some positive results appeared, but some were negative. Acupuncture positive reactions often occur in patients undergoing venipuncture or intramuscular injections. The positive rate of venipuncture was higher than that of intradermal puncture.
There was no significant relationship between the positive reaction of acupuncture and the location of the disease, but it was positively correlated with the activity of the disease. The positive result of the acupuncture test appeared in more than 60% of BD patients in China, while the positive rate in the Mediterranean coastal countries reached 80%. The positive rate of this test in European and American countries is low. This test has fewer false positives and its specificity is 90%.
5. Other related examinations According to the patient's clinical manifestations, related examinations of the affected system, including imaging such as magnetic resonance, angiography, ultrasound cardiovascular examination, endoscopy, cerebrospinal fluid, etc.
Diagnosis
Diagnosis and diagnosis of Behcet disease
It should be differentiated from uveitis with retinal vasculitis; it is associated with anterior chamber purulent anterior uveitis, which is mostly monocular, with anterior or posterior uveitis, and does not combine Most of the diseases such as oral or sickness ulcers are monocular, with anterior or posterior uveitis, and no systemic manifestations such as oral or genital ulcers.
