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Behcet's disease : Causes, Types, Symptoms, Diagnosis and Treatment

 What is Behcet's disease?

Behçet's sickness will produce problems in your mouth and sex organ space, within the variety of perennial sores. This condition is usually related to inflammation of each of the blood vessels and therefore the eyes, tho' it usually causes a great deal of various problems throughout the body.

Behçet's sickness, conjointly called Silk Road sickness, could be a chronic (long-term) inflammatory condition, caused by redness (blood vessel inflammation), which is able to have an effect on and injure each of your arteries and veins.

Behçet’s syndrome could be a rare multisystem inflammatory disorder characterized by ulcers touching the mouth and genitalia, numerous skin lesions, and abnormalities touching the eyes. Symptoms embrace tissue layer lesions of the mouth (canker sores) and genitalia (ulcers) that tend to disappear and recur impromptu. Inflammation of the eyes (anterior inflammation, posterior inflammation, or panuveitis) conjointly affects people with Behçet’s syndrome. further systems of the body might also be affected together with the joints, blood vessels, central system, and/or GI tract. The precise explanation for Behçet’s syndrome is unknown.


What is Behcet's disease


Explanation of medical terms and concept Behcet's disease

Behcet's (beh-CHETS) disease, conjointly known as Behcet's syndrome, may be a rare disorder that causes vessel inflammation throughout your body.

The malady will result in various signs and symptoms that may appear unrelated initially. they will embody mouth sores, eye inflammation, skin rashes and lesions, and venereal sores.

Treatment involves medications to scale back the signs and symptoms of Behcet's malady and to forestall serious complications, like sightlessness.

Behçet's malady is seen everywhere on the planet. However it’s most typical in Northern Turkey (up to 420 cases per one hundred,000 people), the Mediterranean basin and geographic region (up to three hundred cases per one hundred,000 people), and therefore the Far East (about fifteen cases per one hundred,000 people). It’s not as common within the US (about seven cases per one hundred,000 people). Anyone will develop this malady at any age, tho' symptoms typically begin to look between the ages of twenty and thirty. Men and girls square measure equally affected. 

Behcet's disease is a rare lifelong ailment that affects the digestive system and other parts of the body It can cause recurrent mouth sores genital sores painful eyes and swelling in various parts of the body People with Behcet's disease may also experience blood vessel inflammation (vasculitis) skin rashes arthritis pain or muscle aches Although there isn't a cure for Behcet's disease treatments can help manage symptoms.

diet and nutrition Behcet's disease is a systemic disease meaning it has an effect on the entire body It isn't one problem but rather it is a collection of problems When you have Behcet's you can have different symptoms in different areas of your body such as mouth sores and eye inflammation These specific organ systems are affected by Behcet's disease because they all share the same blood supply The symptoms caused by these problems vary widely from patient to patient depending on which organs are affected One common symptom among patients with this condition is inflammation in the vascular system (blood vessels) which causes.

Symptoms Behcet's disease

The earliest symptom of Behçet’s syndrome is typically painful canker sores on the secretion membranes that line the mouth (aphthous stomatitis). The sores square measure typically spherical or oval with blood-red (erythematous) borders that will occur anyplace among the mouth. they will be shallow or deep and should seem as one lesion or a cluster of multiple lesions. The sores usually heal among a number of days, up to every week or additional, while not scarring, however often recur. They will precede different symptoms of Behçet’s syndrome by a variety of years. Typically similar sores might seem on the private parts, specifically the pouch and shaft of the member in males and therefore the female genital organ in females. The sores are spherical and painful, however could also be larger and deeper than those moving the mouth. These sores conjointly recur, however not like oral sores, might tend to scar.

Behçet’s syndrome may additionally have an effect on the eyes. Symptoms might embrace inflammation of the rear of the attention (posterior redness) and inflammation of the anterior chamber (anterior uveitis or iridocyclitis). Inflammation of the iris amid pain, tearing (lacrimation), and therefore the accumulation of pus (hypopyon iritis) may additionally occur. The tissue layer might become inflamed leading to blurred vision, abnormal sensitivity to light-weight (photophobia), and/or inflammation of the skinny membranous layer of blood vessels behind the tissue layer (chorioretinitis). Though the lesions that cause inflammation in varied components of the eyes might resolve, perennial recurrences might end in the partial loss of vision (decreased visual acuity) or complete cecity if the illness is uncontrolled. In some cases, eye abnormalities could also be the primary symptom of Behçet’s syndrome. In different cases, they will not develop till many years later.

Individuals with Behçet’s syndrome may additionally exhibit the formation of little, pus-filled growths on the skin (pustules). Some affected people, particularly females, might develop lesions that give those of erythema, a skin disease characterized by the formation of tender, reddish, inflammatory nodules on the front of the legs. These nodules disappear on their own (spontaneously) typically with faint scars or discoloration (pigmentation). Some people with Behçet’s syndrome might develop little eruptions that give disease of the skin (acneiform eruptions) and/or inflammation that erroneously seem to have an effect on the hair follicles on the skin (pseudofolliculitis).

In close to fifty % of cases of Behçet’s syndrome, affected people experience pain (arthralgia) and swelling in varied joints of the body (polyarthritis). this might occur before, during, or when the onset of the opposite symptoms related to Behçet’s syndrome. Pain, which might vary from gentle to severe, usually affects the joints of the knees, wrists, elbows and ankles, and should become chronic. Lasting harm to affected joints is very rare.

Individuals with Behçet’s syndrome may additionally have revenant ulcers within the alimentary tract. Symptoms vary from gentle abdominal discomfort to severe inflammation of the massive internal organ and body part amid diarrhea or hemorrhage.

Approximately 10%-20% of people with Behçet’s syndrome even have involvement of the central systema nervosum. These symptoms typically seem months or years after the initial symptoms of Behçet’s syndrome. Revenant attacks of inflammation involving the brain (parenchymal Neuro-Behçet) or the membranes that surround the brain or funiculus (meningitis or meningoencephalitis) may result in medical specialty harm. Symptoms might embrace headache, the shortcoming to coordinate voluntary movement (cerebellar ataxia), impaired muscle movements of the face and throat (pseudobulbar palsies), stroke, and/or seldom, seizures.

Behçet’s syndrome causes inflammation of the blood vessels (vasculitis). Involvement of little vessels is believed to drive several of the issues that the disorder causes. In some instances, inflammation of the massive veins, notably those within the legs, might occur beside the formation of blood clots (thrombophlebitis). The walls of Associate in Nursing concerned arteries might bulge forming a sac (aneurysm). In terribly rare cases, blood clots from the veins visit the lungs (pulmonary emboli) leading to episodes of pain, coughing, tough or labored respiration (dyspnea), and forcing out blood (hemoptysis).

Unlike most diseases that square measure classified as a rubor, involvement of the kidneys or peripheral nerves is incredibly rare.

It is particularly necessary to spot Behçet’s illness once there's ocular, central systema nervosum or massive vas involvement as manifestations square measure typically the foremost serious.

Behcet's disease symptoms vary from person to person, can come and go or become less severe over time. Signs and symptoms depend on which parts of your body are affected.

Areas commonly affected by Behcet's disease include:

  • Mouth. Painful mouth sores that look the same as canker sores square measure the foremost common sign of Behcet's illness. They start as raised, spherical lesions within the mouth that quickly become painful ulcers. The sores sometimes heal in one to a few weeks, though they do recur. 

  • Skin. Some people develop acne like sores on their bodies. Others develop red, raised and tender nodules on their skin, especially on the lower legs.

  • Genitals. Red, open sores can occur on the scrotum or the vulva. The sores are usually painful and can leave scars.

  • Eyes. Inflammation in the eye (uveitis) causes redness, pain and blurred vision, typically in both eyes. In people with Behcet's disease, the condition can come and go.

  • Joints. Joint swelling and pain often affect the knees in people with Behcet's disease. The ankles, elbows or wrists also might be involved. Signs and symptoms can last one to three weeks and go away on their own.

  • Blood vessels. Inflammation in veins and arteries will cause redness, pain, and swelling within the arms or legs once a blood results. Inflammation within the giant arteries will cause complications, like aneurysms and narrowing or blockage of the vessel. 

  • Digestive system. A variety of signs and symptoms can affect the digestive system, including abdominal pain, diarrhea and bleeding.

  • Brain. Inflammation in the brain and nervous system can cause headache, fever, disorientation, poor balance or stroke.

When to see a doctor

Make a briefing along with your doctor if you notice uncommon signs and symptoms that may indicate Behcet's unwellness. If you have been diagnosed with the condition, see your doctor if you notice new signs and symptoms. 

Causes Behcet's disease

The exact reason for Behçet’s syndrome isn't far-famed. Studies recommend that some folks could have a genetic predisposition to the condition. A sequencetic predisposition implies that someone could carry a gene for a sickness however it should not be expressed unless one thing within the setting triggers the sickness. Researchers have incontestable that bound people with Behçet’s syndrome, particularly those of geographical region and Asian descent, have associate magnified frequency of bound “human blood corpuscle antigens” (HLAs) within the blood. people with Behçet’s syndrome ar a lot of probably to possess HLA-B51 than the final population. The potential role of HLA-B51 in predisposing people to Behçet’s syndrome and its overall association with the disorder is unknown. alternative genetic markers and their role within the development of Behçet’s sickness ar being studied. microorganism or microorganism infections have additionally been urged as a potential cause for the disorder. Another theory is that sickness is an auto-inflammatory disorder during which the body loses the power to befittingly regulate and manage inflammation.

Autoimmune disorders are caused once the body’s natural defenses against “foreign” or invasive organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons. whereas investigation is current, no autoantibodies so far are known to recommend that Behçet’s syndrome is associated disease.

Behcet's sickness may well be associated with autoimmune disease, which implies the body's system erroneously attacks a number of its own healthy cells. It's probably that genetic and environmental factors play a role.

The signs and symptoms of Behcet's sickness are thought to result in inflammation of the blood vessels (vasculitis). The condition will involve arteries and veins of all sizes, damaging them throughout the body.

Several genes are found to be related to the sickness. Some researchers believe {a virus|an epidemic|a plague|an outbreak|an endemic|a scourge|a pandemic|a deadly sickness|a virulent disease|A pestilence} or microorganism will trigger Behcet's disease in people that have bound genes that create them at risk of Behcet's.

Risk factors Behcet's disease

Factors that might increase your risk of Behcet's include:

  • Age. Behcet's disease commonly affects men and women in their 20s and 30s, though children and older adults also can develop the condition.

  • Where you live. People from countries in the Middle East and East Asia, including Turkey, Iran, Japan and China, are more likely to develop Behcet's.

  • Sex. While Behcet's disease occurs in both men and women, the disease is usually more severe in men.

  • Genes. Having certain genes is associated with a higher risk of developing Behcet's.

Complications 

Complications of Behcet's sickness rely on your signs and symptoms. For example, untreated inflammation will result in reduced vision or vision defects. individuals with eye signs and symptoms of Behcet's sickness ought to visit a watch specialist (ophthalmologist) often as a result of treatment will facilitate forestall this complication.

Diagnosis Behcet's disease

The designation of Behçet’s syndrome is formed to support the clinical judgment of a medical practitioner. Criteria are accepted, primarily based upon the identification of repeated oral ulcerations (aphthous stomatitis) that occur at the side of a minimum of 2 of the following: eye lesions, skin lesions, repeated venereal ulcerations, and a positive pathergy check. (During a pathergy check, a medical practitioner pricks a private with a sterile needle. A positive outcome happens if a ruddy spot (nodule or pustule) forms forty eight hours once the prick.) However, these criteria are fashioned so patients can be enclosed in clinical studies (“classification criteria”) and aren't extremely “diagnostic” criteria.

No tests will verify whether or not you've got Behcet's illness, therefore your doctor can trust totally on your signs and symptoms. As a result nearly everybody with the condition develops mouth sores, mouth sores that have recurred a minimum of 3 times in twelve months square measure typically necessary for a designation of Behcet's illness.

In addition, a diagnosis of Behcet's disease requires at least two additional signs, such as:

  • Recurring genital sores

  • Eye inflammation

  • Skin sores

Tests you might need include:

  • Blood tests or other laboratory tests might rule out other conditions.

  • Pathergy test, in which your doctor inserts a sterile needle into your skin and examines the world one to 2 days later. If the check is positive, attiny low red bump forms underneath your skin wherever the needle was inserted. This means your system is overreacting to a minor injury. 

Treatment Behcet's disease

The treatment of Behçet’s syndrome is directed toward the particular symptoms that are apparent in every individual. Specific therapies for Behçet’s syndrome are unit symptomatic and accessory. Severity of the condition in addition because the patient’s age and sex could all have an effect on treatment choices. Spontaneous remission over time is common for people with Behçet’s syndrome.

For repeated ulcers, the appliance of corticosteroid-containing preparations to the affected areas is also useful in aborting developing attacks. a solution containing an area anesthetic like topical anesthetic, lidocaine, or antihistamine could quickly relieve pain. inflammatory disease related to Behçet’s syndrome is also treated with colchicine and NSAID medication (NSAIDs). continued medical care with the drug colchicine is also effective in preventing continual attacks of oral and reproductive organ ulcers or inflammatory disease.

Ocular inflammation is treated with eye drops containing corticosteroids to alleviate pain. Imuran has been wont to manage the progression of disease in folks with Behçet’s syndrome. Sulfasalazine, Imuran, and corticosteroids are also administered to treat inflammatory internal organ malady and channel lesions related to Behçet’s syndrome. Central system and tube-shaped structure abnormalities are also treated with corticosteroids in addition, usually in conjunction with immunological disorder agents. In patients with curdling of major blood vessels, general anticoagulants and immunosuppressants ought to be thought-about.

Inflammation of the joints, skin, and/or mucous membranes or alternative organs is also reduced with oral steroid hormone medication. However, corticosteroids don't stop continual episodes of symptoms and will not scale back harm once used alone. Therefore, immunological disorder agents like Imuran, antimetabolite, cyclosporine, or alkylating agents are also used for improved management of inflammation and organ protection. expertise is evolving with the employment of interferon-alpha and with agents that inhibit neoplasm death issue (TNF) within the treatment of Behçet’s malady.

There's no cure for Behcet's malady. If you've got a light kind, your doctor would possibly recommend medications to regulate the pain and inflammation of flares. you would possibly not like medication between flares.

For more-severe signs and symptoms, your doctor would possibly impose medications to regulate the Behcet's malady throughout your body, additionally to medications for flares.

Treatments for individual signs and symptoms of Behcet's disease

Medications to manage signs and symptoms you've got throughout flares may embrace the following:

  • Skin creams, gels and ointments. Topical corticosteroid medicines are applied directly to skin and genital sores to reduce inflammation and pain.

  • Mouth rinses. Using special mouthwashes that contain corticosteroids and other agents might reduce the pain of mouth sores.

  • Eyedrops. Eye Drops containing corticosteroids or other anti-inflammatory medicines can relieve pain and redness in your eyes if inflammation is mild.

Systemic treatments for Behcet's disease

If topical medications do not facilitate, your doctor may suggest a drug known as colchicine (Colcrys, Mitigare) for continual oral and sex organ sores. Joint swelling additionally may improve with colchicine.

Severe cases of Behcet's unwellness need treatments to manage injury from the unwellness between flares. If you have got moderate to severe Behcet's unwellness, your doctor may prescribe:

  • Corticosteroids to control inflammation. Corticosteroids, like Deltasone, are used to cut back the inflammation caused by Behcet's illness. Doctors typically inflict them with another medication to suppress the activity of your system.
    Side effects of corticosteroids embody weight gain, persistent symptom, high force per unit area and bone cutting (osteoporosis). 

  • Medications that suppress your immune system. The inflammation related to Behcet's malady will be reduced by medicine that stops your system from offensive healthy tissues. These medicines might embrace Imuran (Azasan, Imuran), cyclosporine (Gengraf, Neoral, Sandimmune) and cyclophosphamide. These medicines will increase your risk of infection. different attainable aspect effects embrace liver and excretory organ issues, low blood counts, and high pressure level. 

  • Medications that alter your immune system's response. Interferon alfa-2b (Intron A) regulates the activity of your system to regulate inflammation. It's going to be used alone or with alternative medication to assist in the management of skin sores, joint pain and eye inflammation in individuals with Behcet's malady. Facet effects embrace flu-like signs and symptoms, like muscle pain and fatigue.
    Medications that block a substance known as neoplasm death issue (TNF) are effective in treating a number of the signs and symptoms of Behcet's, particularly for people that have more-severe or resistant symptoms. Examples embrace Remicade (Remicade) and adalimumab (Humira). Facet effects may embrace headache, efflorescence associated with an increased risk of infections. 

Coping and support

The unpredictability of Behcet's illness will create it significantly frustrating. Taking excellent care of yourself may assist you cope. In general, try to:

  • Rest during flares. When signs and symptoms seem, take time for yourself. Be versatile and alter your schedule once potential so you'll be able to rest after you ought to. try to minimize stress. 

  • Be active when you have the energy. Moderate exercise, such as walking or swimming, can make you feel better between Behcet's disease flares. Exercise strengthens your body, helps keep your joints flexible and can improve your mood.

  • Connect with others. Because Behcet's could be a rare disorder, it should be troublesome to seek out others with the malady. raise your doctor regarding support teams in your space. If it is not potential to attach with somebody shut, the Yankee Behcet's malady Association offers message boards and chat rooms wherever you'll be able to connect with people World Health Organization have Behcet's. 

Preparing for your appointment

You're seemingly starting out by seeing your medical aid doctor. He or she could refer you to a doctor WHO treats inflammatory disease and different rheumatic diseases (rheumatologist).

Depending on your signs and symptoms, you would possibly conjointly got to see Associate in Nursing oculist for eye issues, a medical specialist or medical specialist for venereal sores, a medical specialist for skin issues, a doctor for biological process difficulties, or a brain doctor for symptoms that involve the brain or central system.

Here's some data to assist you make preparations for your appointment.

What you can do

Make a list of:

  • Your symptoms, including when they began and how severe they are

  • Key personal information, including major stresses and recent life changes

  • All medications, vitamins and supplements you take, including doses

  • Questions to ask your doctor

Ask a family member or friend to come along to help you remember the information you receive.

For Behcet's, questions to ask your doctor include:

  • What do you think is causing my symptoms?

  • What tests do I need? Do they require preparation?

  • Is my condition temporary or long lasting?

  • What are my treatment options, and which do you recommend?

  • I have another medical condition. How can I best manage these conditions together?

  • Do you have brochures or other printed material that I can take? What websites do you recommend?

What to expect from your doctor

Your doctor is likely to ask you questions, such as:

  • Do you have your symptoms all the time, or do they come and go?

  • What, if anything, seems to improve your symptoms?

  • What, if anything, seems to worsen your symptoms?

  • Does anyone in your family have a similar illness?

General summary

Behcet's disease is an unusual illness in many ways It has not been linked to any other diseases or conditions; it affects people of all ages races and both sexes equally often Researchers are still learning more about this complex disorder and how it progresses over time The only way to diagnose Behcet's disease with certainty is through a review of the patient's history and physical examination along with laboratory testing.? Although there is currently no cure for Behcet's disease, treatment can lessen its symptoms and prevent complications during flare-ups.

Research shows that a person can develop Behcet's at any age. People under 25 are more likely to experience symptoms but not exclusively The trigger is unknown although autoimmune disorders have been linked with the disease Biologic agents found in mold and bacteria may also cause it.

What foods should you avoid with Behcet's disease?

Coping with Behcet's disease you may have to avoid some foods In general avoid any food that makes your mouth or skin hurt Some common problem foods include spicy acidic and salty foods and those that are rich in sugar Watch out for MSG (monosodium glutamate) and sulfites as well.

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