Experts generally believe that Behcet's disease (? Et's Disease (BD) is a disease characterized by oral ulcer, vulvar ulcer, ophthalmia and skin damage.
And multiple system vasculitis syndrome. Basic pathological changes: vasculitis of tissues and organs.
Encyclopedia description: Beh? Et disease is a rare immune-mediated systemic vasculitis.
S-membrane ulcer and eye problems. As a systemic disease, it can also involve internal organs, such as T.
Intentional tract of stomach, lung, musculoskeletal, cardiovascular and nervous system. Translation: Behcet's disease.
This is a rare immune-mediated systemic vasculitis, usually accompanied by mucosal ulcers and eye problems. As a systemic disease, it can also involve internal organs,
Such as gastrointestinal tract, lung, musculoskeletal system, cardiovascular system and nervous system.
Dr. Guan proposed: Behcet's disease? Et's Disease (BD), also known as Silk Road Disease, is a recurrent oral ulcer.
For the first time, vulvar ulcer, erythema nodosum and other skin and mucosa lesions are the basic clinical features, and ophthalmia, gastrointestinal ulcer and aortic valve reflux may occur selectively.
1 ~ 2 Variant vasculitis with organ damage, such as thrombosis, venous thrombosis, arterial stenosis, aneurysm, arthritis or cytopenia. Major disability: blindness; owner
The main causes of death: nerve injury, heart failure, aneurysm and intestinal perforation.
2. What is Behcet's disease? What are its hazards?
Behcet's disease can be traced back to 1937, by Turkish ophthalmologist Hulusi Beh? Et (1889–1948) first reported that diseases were named after doctors.
Behcet's syndrome, short for Behcet's disease. Chronic oral ulcer, genital ulcer and uveitis are the "triad" of the disease. With disease
With the development of the disease, skin, gastrointestinal tract, blood vessels, lungs, joints, urinary system and even nervous system will appear pathological changes. The eye is the main disabling organ of Behcet's disease. eye
Inflammation in this part recurs, and the healing process is usually slow, and it often cannot be fully recovered, and then it recurs, leading to serious visual damage or blindness. All parts of the eyeball
Can be involved. Pigment membrane inflammation, retinal vasculitis, optic atrophy, vitreous inflammation and fundus hemorrhage may occur. Anterior uveitis is iridocyclitis and may be accompanied by
Or no hyphema. Then uveitis and retinal vasculitis are the main reasons that affect vision. Repeated attacks of ophthalmia can lead to serious visual impairment or even loss.
Ming. The proportion of gastrointestinal tract involvement in patients with Behcet's disease in China is high. Clinical symptoms can be manifested as abdominal pain, diarrhea, constipation, bloody stool and sudden severe abdominal pain caused by ulcer perforation.
Wait a minute. Serious complications, such as intestinal fistula, perforation, gastrointestinal bleeding, etc. If the treatment is not standardized, it will happen, requiring surgical treatment and poor prognosis. The damage of nervous system is the severity of Behcet's disease.
One of the complications, high recurrence rate and poor treatment effect, is the main cause of death of the disease. Its main manifestations are headache, dizziness, disturbance of consciousness, mental abnormality and meningeal tingling.
Stimulation, epilepsy, weakness of lower limbs, numbness, sensory disturbance, etc. Vascular involvement in Behcet's disease is also an important cause of death. When large blood vessels are damaged, they may show arterial and venous resistance.
Embolism, aneurysm and varicose veins. Venous obstruction is common in superior and inferior vena cava obstruction syndrome. Arterial involvement can form aneurysms, mostly single aneurysms, which are common in aorta.
Arteries, abdominal aorta and thoracic aorta, while arterial occlusion is rare. However, renal artery stenosis can cause renal hypertension, and cardiac vasculitis can affect myocardium, valves and conduction.
Systemic, palpitation, angina pectoris, arrhythmia, etc. Behcet's disease is little known, and other specialists know little about it, so Behcet's patients often can't get it.
Early accurate and effective diagnosis and treatment will lead to aggravation of the disease, involving multiple organs, which will bring difficulties to later treatment.
3. How is Behcet's disease caused and why do you get Behcet's disease?
Behcet's disease is essentially an autoimmune disease caused by immune system disorder of individuals with certain genetic background, which mainly infringes on various sizes.
Arteries, veins or capillaries have different clinical manifestations according to the different involved vessels. Whether a person will get Behcet's disease depends first on whether he is easy.
The genetic background of susceptible genes makes it possible to suffer from this disease only in this background. According to the current clinical research, Behcet's disease does have a tendency of family aggregation.
To. But in clinic, we also see that many patients have no clear family history, and many patients can't find their relatives who are also sick for generations. So there's this.
Genetic background does not mean that a person will definitely get sick. Whether you get sick or not depends on the environmental factors of the acquired patient. At present, it is considered to affect the immune system and people.
Factors that stabilize the body itself may become the cause of Behcet's disease, such as respiratory tract infections such as colds and tonsillitis, and digestive tract infections such as diarrhea and vomiting.
Urinary tract infection caused by cleaning behavior is the most harmful to the immune system, so most patients often have the cause of infection before the onset. Others, such as life, are not.
Regularity, day and night upside down, overeating, depression and anxiety, excessive mental stimulation, etc. May affect people's own stability. There is even research on trace elements.
Lack will also affect the stability of the autoimmune system. When these blows are frequent enough or strong enough, the defective genes in the body will change from quantitative to qualitative. At this time,
People have changed from a healthy person to a sick person. Sudden onset, seemingly sudden, is actually a gradual process. Therefore, the onset of Behcet's disease is
The result of many factors.
4. Will children and the elderly get Behcet's disease?
Home > Department of Special Needs Diagnosis and Treatment, East China Hospital Affiliated to Fudan University, Pudong Clinic of East China Hospital Affiliated to Fudan University, Shanghai International Medical Center > Guan > Guan Health Number > 20 Frequently Asked Questions of Behcet's Disease Patients-Guan's Expert Team * * *
According to epidemiological data, Behcet's disease patients at home and abroad are 20-40 years old, and children and elderly Behcet's disease patients are relatively rare. children
Children and the elderly with recurrent oral ulcers, vulvar ulcers, skin lesions and/or ophthalmia need careful screening. Infectious diseases such as herpes must be excluded.
Virus infection, fungal infection, tuberculosis infection, syphilis, excluding autoimmune diseases, such as inflammatory bowel disease, sarcoidosis, spondyloarthropathy, systemic lupus erythematosus, Sjogren's syndrome, and common autoimmune diseases in dermatology, such as lichen planus, pemphigus and pemphigoid. In particular, infectious diseases such as herpes virus infection, fungal infection, tuberculosis infection and syphilis must be excluded. Because the treatment principles of infectious diseases and Behcet's disease are essentially different, once an infectious disease is misdiagnosed as Behcet's disease,
Treatment may lead to the rapid deterioration and progress of the primary disease, causing serious organ damage to patients and even life-threatening. After excluding other causes with similar clinical manifestations, children and elderly patients who meet the diagnostic criteria of Behcet's disease still need long-term observation and follow-up to avoid misdiagnosis and missed diagnosis.
In addition, some studies have shown that the risk of serious organ damage in early-onset Behcet's disease in men is relatively high. Therefore, we should pay more attention to male patients with early Behcet's disease.
Once severe organ involvement is found, immunosuppressants and/or biological agents should be used as soon as possible to control and avoid organ damage.
5. Will Behcet's disease be passed on to children?
Behcet's disease is reported all over the world, but it has obvious geographical distribution. Judging from the current data, most cases are concentrated in Japan, South Korea, China and the Middle East.
And the Mediterranean region. The incidence of Behcet's disease is not only different between countries, but also within a country. Take China as an example, Behcet's disease is more common in North China.
The estimated incidence rate is not less than 14/65438+ 10,000, and it is mainly sporadic in the south. The special regional distribution of the disease suggests a certain genetic background. Behcet's disease currently
Rare diseases are defined as rare diseases, and rare diseases refer to those diseases with extremely low incidence. According to the definition of the World Health Organization (WHO), rare diseases refer to diseases in which the number of patients accounts for the total population.
0.65 ‰ ~/kloc-0 ‰ disease. According to the current estimated incidence14/100000, Behcet's disease patients account for about 0. 14‰ of the total population. To calculate its danger
Degree, we must conduct a family survey, a large-scale survey of the whole province and even the whole country. So what we can see at present is the case report of Behcet's disease family inheritance. Zuzao restaurant
The report on the inheritance of Behcet's disease is: 1976, four generations of five people in a family have recurrent oral, eye and genital ulcers; The two suffer from a rare emotional disorder.
Both brothers with mental disorders and their mothers suffer from Behcet's disease, which shows that Behcet's disease has the same family aggregation as other autoimmune diseases. Foreign scholars in 1999
In the retrospective study in, it was found that 12.3% of the relatives of children with Behcet's disease suffered from Behcet's disease, which was significantly higher than that of adults. Behcet's disease in children was discovered in 2003.
Family data are consistent with autosomal negative inheritance, but adult Behcet's disease family is not. The above information also suggests that Behcet's disease is indeed hereditary. Current research
The genetic factor HLA-B5 1 is related to Behcet's disease, but it is still uncertain whether HLA-B5 1 itself or its closely linked genes lead to Behcet's disease susceptibility.
To sum up, whether a person will get Behcet's disease depends first on whether he has the genetic background of susceptible genes, and whether he will get sick will be affected by the environmental factors of acquired people.
6. Is Behcet's disease a sexually transmitted disease? Is it contagious?
Behcet's disease is not a sexually transmitted disease, so it is not contagious. Behcet's disease is an autoimmune disease, that is, the fundamental cause is the dysfunction of the patient's own immune system.
Inflammation of the body is clinically manifested as mucosal lesions, and some patients have damage to the eyes and/or internal organs. However, due to some sexually transmitted diseases such as syphilis and acquired immunity,
Patients with acquired immune deficiency syndrome (AIDS) may have skin and mucosal lesions and/or eye and eye diseases similar to Behcet's disease.
(or) Symptoms and signs of visceral organ damage. For example, the typical skin lesion of patients with primary syphilis is chancre, and small red papules or induration appear at the invasion site of Treponema pallidum.
Later, it showed erosion, forming superficial ulcer, hard and painless, round or oval, clear boundary, neat edge, dike uplift and darkness around.
Red infiltration, characteristic cartilaginous hardness, flat base, no pus, cellulose-like film attached to the surface, not easy to remove. If it is squeezed slightly, a small amount of slurry may infiltrate.
The export contains a large number of Treponema pallidum, which is an important source of infection. Hard chancre is mostly single, and there are 2-3 cases. Female chancre is more common in labia majora and clitoris.
Urethral orifice, pubic mound, especially cervix. Hard chancre has the following characteristics: 1, and the damage is often single; 2. Cartilage hardness; 3, no pain; 4. The damaged surface is clean.
Secondary syphilis skin and mucous membrane damage, lymph node enlargement; The heart, nerves, stomach, eyes and ears of tertiary syphilis are all involved, and the gums are swollen and damaged. Clinically, never be careful.
Identification easily confuses the two. Clinically, we often encounter some patients (especially middle-aged and elderly patients) with oral ulcers, vulvar ulcers and some skin lesions.
It looks like Behcet's disease, but further examination confirms or suspects syphilis and AIDS. Therefore, for "high-risk" patients with contact history, routine detection and syphilis serum test are needed.
Test and HIV antibody screening test (ELISA) ruled out diagnosis.
What are the symptoms of Behcet's disease?
The clinical manifestations of Behcet's disease are various, and the symptoms can appear one after another. The first symptom of most patients is recurrent oral ulcer. The so-called recurrence usually refers to the course of disease exceeding 1 year, every
More than 3 episodes a year. Oral ulcers are usually deep, large and flaky, and the mouth, lips, buccal mucosa and throat can all be involved. Some patients go out first.
At present, vulvar ulcer is mainly manifested as scrotal ulcer in men, while labia, vagina and cervix are involved in women. Ulcers usually heal for a long time, often more than 10 days, but
The frequency of attack is lower than that of oral ulcer. If the patient sees a doctor at the acute stage and is diagnosed as vulvar ulcer or has old scars, it will be helpful for diagnosis. Obtain research recognition
Therefore, patients with vulvar ulcer as the first symptom have a higher risk of damage to important organs. Behcet's disease is the main cause of uveitis in China. If you suddenly have vision,
Decline, eye pain, photophobia symptoms, need to see an ophthalmologist as soon as possible to determine whether there is uveitis. Some patients can take digestive tract symptoms as the main manifestations, such as after eating.
Sternal pain, dysphagia, recurrent right lower abdominal pain, watery diarrhea. Less Behcet's disease patients are mainly manifested as repeated chest tightness, shortness of breath, sudden headache, numbness of limbs and dyskinesia.
Performance. Once it appears, cardiovascular and central nervous system involvement can be confirmed by subsequent auxiliary examination. Patients with Behcet's disease often have a poor prognosis, and it will be life-threatening if they are not treated in time.
Life. Nonspecific symptoms, such as fever and fatigue, can appear in the active stage of the disease. Unexplained fever may also be a precursor to Behcet's disease.
8. How is Behcet's disease classified clinically?
The classification of Behcet's disease needs a series of indicators to evaluate, including the morphology and characteristics of clinical symptoms, oral ulcer, erythema nodosum, ophthalmia and so on. , combined with the corresponding internal organs.
The results of laboratory examination, ultrasound, magnetic resonance imaging, CT, endoscopy and even diagnosis and treatment observation all depend on the specific situation of each patient. Behcet's disease can be divided into:
There are 8 types of Behcet's disease, including cutaneous mucinous Behcet's disease, ocular Behcet's disease, nervous Behcet's disease, intestinal Behcet's disease, cardiac Behcet's disease, vascular Behcet's disease, blood Behcet's disease and articular Behcet's disease.
Behcet's disease of skin and mucous membrane is the basic phenotype, and nerve Behcet's disease, vascular Behcet's disease and joint Behcet's disease can overlap with other phenotypes in a few cases.
9. How to check Behcet's disease and what items to check?
202111/30pm1:22pm20 Frequently asked questions of Behcet's disease patients-related expert team * * * Same answer-micro-medicine (registered network)
Behcet's disease is a kind of systemic vasculitis with recurrent oral ulcer as the first episode and skin mucosal lesions such as vulvar ulcer and erythema nodosum as the basic clinical features.
Selective ophthalmia, gastrointestinal ulcer, aortic regurgitation, arthritis, lower extremity venous thrombosis, arterial stenosis, aneurysm and other 1 ~ 2 organ damage, on this basis.
Basically, nervous system injury is a key signal. Because Behcet's disease can involve the unique heterogeneity of large, medium and small blood vessels in the whole body. Behcet's disease involves arteries to static blood vessels.
Pulse from small blood vessels to large blood vessels, all over the body, causing multiple system damage. Therefore, the examination of Behcet's disease patients focuses on the word comprehensive. The exam is mainly divided into physical fitness.
Examination, physical and chemical examination, imaging examination, pathological examination and special examination. Detailed and comprehensive medical history collection and physical examination play an important role in rheumatic diseases, including
It depends on the experience accumulated by clinicians all the year round. Physical and chemical examination often includes various inflammatory indicators, humoral immunity, cellular immune gene detection and other examinations for exclusive tumors.
Metabolic and other connective tissue diseases; It is worth noting that negative HLAB5 1 cannot be used as the basis for excluding Behcet's disease. As an intuitive and non-invasive examination, image examination.
Examination is widely used in the diagnosis and differential diagnosis of Behcet's disease, including the evaluation of general cranial magnetic resonance vibration of nervous system, CTA of systemic vascular system, and evaluation.
Doppler of heart structure, gastrointestinal endoscopy for evaluating digestive tract ulcer, etc. However, considering the medical expenses, not all patients need to complete all the above impact tests.
Clinicians usually screen patients according to their clinical manifestations and the prevalence of systemic involvement in Behcet's disease, and focus on the systems that are easily involved. Pathological examination is currently Behcet.
This disease has no recognized pathological features. Acupuncture reaction is the main aspect of special examination, but it has been gradually eliminated because of its large error and relatively uncomfortable medical experience.
10. Behcet's disease has no abdominal pain or diarrhea. Do you still need a colonoscopy?
Behcet's disease has no obvious individualized tendency of organ damage, and the proportion of eye damage and heart damage in Behcet's disease population is similar. But unfortunately, the intestinal injury of patients in China is equivalent.
The proportion of damage is significantly higher than that in the Middle East, Europe and America. Therefore, patients with Behcet's disease in Asia are more likely to suffer from intestinal Behcet's disease. It is worth noting that early Behcet's disease is not common in daily life.
Symptoms, and even some patients often find persistent intestinal fistula after gastrointestinal surgery for other reasons. So the detection rate of Behcet's disease in China is lower than the actual situation. also
It is precisely because of the early asymptomatic characteristics of intestinal obstruction that many patients often delay going to the hospital until intestinal perforation and peritonitis. At this time, the treatment of patients is difficult and the mortality rate is high.
High, the clinical cost is also significantly higher than the previous period. Therefore, early detection and active treatment of intestinal obstruction is of great significance. As an advanced examination technique, colonoscopy can be fully utilized.
Explore the whole colon to the ileocecal part, which is the high incidence of Behcet's disease. Therefore, even if Behcet's disease patients have no abdominal pain or diarrhea, we should actively give colonoscopy.
Examination, with a view to early detection and treatment of intestinal congestion, to prevent further aggravation of the disease.
1 1. What medicine does Behcet's disease take? Common drug side effects?
At present, there is no "specific drug" that can radically cure Behcet's disease, and many drugs are effective, so we should choose drugs according to the classification, activity and individual situation of patients.
The side effects of local medication are relatively small, including glucocorticoid ointment, glucocorticoid combined with gentamicin diluted with mannitol to make mouthwash to treat oral ulcer.
In terms of treatment, antibiotic ointment is used for anti-infection treatment of genital ulcer, corticosteroid eye ointment or eye drops are used for uveitis, and mydriatic agents are used to prevent post-inflammatory adhesion if necessary.
Even in severe ophthalmia, adrenocortical hormone can be injected under the bulbar conjunctiva.