Behcet's disease scleritis

Introduction to Behcet's disease Behcet's disease (BD) is a chronic recurrent systemic vasculitis with unknown etiology and is a systemic immune disease. The characteristic manifestations are oral ulcers, genital ulcers and inflammation of the eyes. The connection between ophthalmia, oral ulcers and genital ulcers was discovered as early as the "father of medicine" Hippocrates. It was not until 1937 that the Turkish dermatology professor Hulusi Behcet first recognized and described the triad of the disease. . basic knowledge The proportion of sickness: 0.00005% Susceptible people: no specific people Mode of infection: non-infectious Complications: conjunctivitis scleritis

Cause of Behcet's Scleritis

(1) Causes of the disease

The etiology is still unclear. American scholars have found the genome of HSV-1 in lymphocytes of some critically ill patients, and infected the patient's lymphocytes with herpes simplex virus infection, resulting in more -interferon than normal people and other diseases. According to the phenomenon that the antibody produced by HSV-1 is much higher than that of normal people, it is suggested that this disease may be related to the infection of HSV-1. Japanese scholars apply different staphylococci, especially the antigen psoriasis test of hemolytic streptococcus. It was found that patients with this disease had a higher and stronger delayed skin immune response than the control group, and even caused recurrence of the disease, and the lymphocytes of the patient were co-cultured with streptococcus to induce lymphocytes that stimulate neutrophil function. Factor, so that the disease may be related to streptococcus, some scholars based on about 1/3 of patients have a history of tuberculosis in the past, or currently diagnosed with different tuberculosis, some patients have a strong OT skin test response, anti-tuberculosis PPD antibody The positive rate and titer were higher than those of the control group, and some patients achieved satisfactory results after anti-tuberculosis treatment. It is believed that the occurrence of this disease is closely related to tuberculosis, 3 The arguments are in-depth study, yet to make a positive conclusion, does not rule out the disease is a heterogeneous disease, caused by a variety of causes.

(two) pathogenesis

The pathogenesis of this disease has not been elucidated, but different immune abnormalities can be observed, such as enhanced neutrophil chemotaxis, decreased CD4 helper T cell ratio and normal CD4 suppressor cell number, presence of human mucosal antibody, patient lymph Cells in the test tube have a cytotoxic effect on human oral epithelial cells, half of the patients have increased CIC, deposition, leading to vasculitis, many patients can detect condensed globulin, polyclonal immunoglobulin abnormalities, etc., whether these changes are related to the pathogen And, for example, anti-mucosal cell antibodies are primary or secondary, and further research is needed.

Behcet's disease scleritis prevention

This disease is often a concomitant disease of some systemic diseases. Once it occurs, it should actively find the relevant primary disease and treat the primary disease to effectively control the disease.

Behcet's disease with scleritis Complications conjunctivitis scleritis

A small number of patients have ocular anterior segment involvement, including conjunctivitis, keratitis, scleritis, and scleral inflammation.

Behcet's disease scleritis symptoms Common symptoms Oral mucosa ulcer Internal bleeding Venous thrombus Pustule Oral ulcers Scleral outer inflammation

1. Ocular manifestations of ocular lesions may be the only manifestations of Behcet's disease, and may also be associated with other systemic lesions, especially central nervous system lesions. Eye lesions usually begin to invade one eye, eventually involving both eyes, but one of them occurs More severe, one-third of patients have aseptic anterior chamber empyema, which Behcet describes as a typical ocular manifestation, usually in the late stages of the disease, and disappears quickly, aseptic anterior chamber empyema and infectivity The difference between anterior chamber empyema is that the former does not coagulate and can move with changes in body position. The anterior uveal, retinal and optic neuropathic lesions are more common than anterior chamber empyema. Anterior uveitis can cause cataract, after iris Adhesive and secondary glaucoma, retinal vasculitis generally involves small branches of arteries and veins, vascular white sheath, and intraretinal hemorrhage, edema and exudation, central retinal vein or branch obstruction, arteriolar thinning and varicose vein expansion, sometimes can occur Serous retinal detachment, optic vasculitis can cause optic disc congestion or edema and permanent optic atrophy, retinal and optic vasculitis can lead to loss .

(1) scleritis: Behcet's disease scleritis is rare, the same scleritis with Behcet's disease is also rare, the incidence of Behcet's disease in patients with scleritis is 0.68%, scleritis is the only symptom of Behcet's disease, causing The patient's attention and medical treatment, after further examination and diagnosis of Behcet's disease, can also occur with retinal vasculitis and optic discitis. For young scleritis patients with recurrent retinal vasculitis in both eyes, it is important to consider the possibility of Behcet's disease. Detailed bone mucosal and cutaneous examinations are helpful in the diagnosis of this disease, and scleritis activity can be synchronized with the activity of systemic diseases.

(2) scleral outer inflammation: scleral outer inflammation is also rare in patients with Behcet's disease, often occurs after retinal vasculitis and optic disc edema, etc., like other eye abnormalities, may occur during the active period of Behcet's disease Scleral outer inflammation.

2. Non-ocular manifestations In addition to oral ulcers, genital ulcers and ocular inflammation, there are also manifestations of invasion of the skin, joints, large blood vessels, gastrointestinal tract and central nervous system.

Oral mucosal ulcers are 2 to 10 mm in diameter, round and oval, with a yellow base in the center and red halo around them. The ulcer often appears in more than one place, which occurs in the buccal mucosa, lips, gums, tongue and pharynx, ulcers. 3 to 30 days of healing, ulcers are easy to relapse, usually without scars, female external genitalia, vagina, and even the cervix and male scrotum, mucosal ulcers of the penis are similar to oral ulcers, but the recurrence rate is low, the ulcer is deep, usually left Scars, genital ulcers are less common than oral ulcers, and non-mucosal genital ulcers are characterized by nodular lesions with central ulcers.

Typical skin lesions of Behcet's disease include nodular erythema, pustules, acne-like papules and skin allergies, acupuncture by pathergy test, and significant red after 24 to 48 hours of intradermal injection of air or saline. Swelling, rash, apical small pustules or folliculitis are positive, these are non-specific manifestations of cutaneous vasculitis, positive for acupuncture test, 90% positive in Japanese and Turkish patients, positive rate in Chinese patients At 62.2%, the test was highly specific and rarely tested positive in other diseases and normal subjects.

60% of Behcet's patients may have rheumatoid factor (RF)-negative non-walking recurrent arthritis, without deformity, which occurs in the knee, ankle, wrist, especially the knee, the most involved, cardiovascular involvement It is an arteriovenous occlusion and an aneurysm. The large, medium and small blood vessels of almost different parts can be involved. The venous lesion is twice as much as the arterial lesion. It is characterized by deep vein occlusion (common in the superior and inferior vena cava, subclavian vein, femoral vein). , iliac veins and hepatic veins, and superficial thrombophlebitis, arterial lesions often occur under the subclavian, stenosis or obstruction of the kidney, neck and femoral artery, cardiovascular disease abnormalities in Behcet's disease, poor prognosis, gastrointestinal tract can occur Ulcers, especially in the lower ileum and right colon, some patients with intestinal ulcers further develop to perforation, partial colectomy and distal ileal resection are required, 10% of patients with Behcet's central nervous system involvement can cause sensation, exercise and Neuropsychiatric abnormalities, meningoencephalitis can cause headache, fever, neck stiffness, cerebrospinal fluid cells and local neurological dysfunction.

Examination of Behcet's disease scleritis

Patients with Behcet's disease can detect many immune abnormalities, but lack specificity, can appear elevated circulating immune complex (CIC), decreased complement levels, leukocytosis, elevated condensed globulin and -interferon, neutrophils Increased chemotactic activity, as well as elevated serum IgA, IgG, and IgM, serum CD4 and CD8 ratios changed from normal 2:1 to 1:1, acute episodes of ESR increased, C-reactive protein ( Increased CRP), increased central nervous system, increased cerebrospinal fluid protein and lymphocytes.

1. Brain CT examination showed more than half of the abnormalities.

2. X-ray examination of gastrointestinal, lung, bone and joint X-ray examination and arteriovenous angiography, also help to diagnose.

3. Fundus fluorescein angiography helps determine the extent of retinal and optic disc disease.

Diagnosis and diagnosis of Behcet's disease

The disease has no specific serological and pathological diagnosis methods, so the diagnosis is mainly based on clinical, and the current internationally used diagnostic criteria are developed by the Japanese Behcet's Research Society and the International Behcet's Research Group (ISG) established in 1990. The former divides patients into complete and incomplete types, and recurrent anterior uveitis, recurrent oral ulcers, pleomorphic skin lesions and genital ulcers are called incomplete types; three main types appear. The sign or two main signs and some other lesions are called complete types. The ISG standard is:

Recurrent oral ulcers (recurred at least 3 times in 1 year), plus typical eye damage.

Two of the following four items can be diagnosed:

1 recurrent genital ulcer or genital scar.

2 typical eye damage (anterior uveitis, vitreous cells or retinal vasculitis).

3 skin damage (nodular erythema, pseudo folliculitis or pustular papules or acne-like nodules).

4 Acupuncture test positive reaction, when young patients with anterior uveitis, scleritis, scleritis, retinal vasculitis or vascular occlusion, should consider the possibility of Behcet's disease, suspected Behcet's disease, should For the examination of the whole body, especially the mucosa and skin, and the understanding of the medical history, for individual cases, the following points may be important for diagnosis: subcutaneous thrombophlebitis, deep vein thrombosis, epididymitis, arterial occlusion and aneurysm, central Nervous system involvement, arthritis, gastrointestinal ulcers and family history.

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