Materiały źródłowe

• #1 Behcet disease // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/behcet-s-disease

  Behcet disease symptoms can seem like they aren’t related at first. They can include mouth sores, eye irritation and swelling, skin rashes and sores, and genital sores. […] Behcet disease symptoms vary from person to person. Symptoms can come and go or become less serious over time. Symptoms depend on which parts of the body the condition affects. […] Painful mouth sores that look like canker sores are the most common sign of Behcet disease. They begin as raised, round sores in the mouth. They quickly turn into painful ulcers. The sores most often heal in 1 to 3 weeks. But they often come back. […] Some people get sores that look like acne on their bodies. Others get raised and tender growths called nodules on their skin, mainly on the lower legs. […] Open sores can happen on the scrotum or the vulva. The sores are most often painful and can leave scars.

• #1 Behcet disease // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/behcet-s-disease

  Irritation and swelling, called inflammation, in the eye causes redness, pain and blurred vision. Called uveitis, this inflammation most often affects both eyes. In people with Behcet disease, these symptoms can come and go. […] Joint swelling and pain often affect the knees in people with Behcet disease. The ankles, elbows or wrists also might be involved. Symptoms can last 1 to 3 weeks and go away on their own. […] Swelling, called inflammation, in veins and arteries can cause redness and pain. It can cause a blood clot, which leads to swelling in the arms or legs. Inflammation in the large arteries can lead to complications. These include bulges in the artery that can burst, called aneurysms, and narrowed or blocked blood vessels. […] Several symptoms can affect the network of organs that digest food, called the digestive system. Symptoms may include belly pain, diarrhea and bleeding. […] Swelling, called inflammation, in the brain and nervous system can cause headache, fever, confusion, poor balance or stroke.

• #2 Behçet’s Disease: Symptoms, Treatments, Causes & Diagnosis

  https://my.clevelandclinic.org/health/diseases/12980-behcets-disease

  Behets disease can create issues in your mouth and genital area, in the form of recurrent sores. This condition is mostly associated with inflammation of both the blood vessels and the eyes, though it often causes a lot of different issues throughout the body. […] The main symptoms include: Mouth sores and/or genital sores that keep coming back. Skin and joint pain. Inflammation in the eyes. […] Mouth sores, which occur at some time in all patients. Theyre usually recurrent (keep coming back) and painful and affect almost all patients with Behet’s disease. The sores look like the common canker sore, but are more numerous, frequent and painful. Theyre often the first symptom that a person notices, and may occur long before any other symptoms appear. Mouth ulcers are seen on the lips, tongue and inside the cheek.

• #2 Behçet’s Disease: Symptoms, Treatments, Causes & Diagnosis

  https://my.clevelandclinic.org/health/diseases/12980-behcets-disease

  Genital sores look similar to mouth sores and may be painful. They arent as common as mouth sores. They appear on the scrotum (the sack enclosing the testes) in men and on the vulva (the external genital organs) in women. […] Eye inflammation can cause pain, blurry vision, light sensitivity, tears or eye redness. Behet’s disease may eventually lead to vision loss. Severe eye disease that can cause blindness is more common in the Middle East and Japan than in the United States. […] Skin problems are a common symptom. They may look like acne or tender, coin-shaped nodules (erythema nodosum), or ulcers that may be shallow or deep and painful. A red bump or sore may develop if the skin is scratched or pricked. Doctors call this a positive pathergy test. […] Joint pain is common. The ankles, knees, elbows and hips are most often affected. Joint inflammation causes swelling, redness and tenderness, though it usually doesnt cause permanent damage. […] Behets is a chronic (long-term) disease that can disappear and reappear, no matter the treatment. This can make it difficult to tell how well treatment is working. Most patients with Behets will likely deal with symptoms, on and off, for their entire lives. But, most are able to live a full life.

• #3

  https://www.nhs.uk/conditions/behcets-disease/

  The main symptoms of Behet’s disease include: genital and mouth ulcers, red, painful eyes and blurred vision, acne-like spots, headaches, painful, stiff and swollen joints. […] In severe cases, there’s also a risk of serious and potentially life-threatening problems, such as permanent vision loss and strokes. […] Most people with the condition experience episodes where their symptoms are severe (flare-ups or relapses), followed by periods where the symptoms disappear (remission). […] Symptom’s often start in adults in their 20s and 30s, but can also start in childhood. […] Over time, some of the symptoms can settle down and become less troublesome, although they may never resolve completely.

• #4

  https://www.nhs.uk/conditions/behcets-disease/symptoms/

  Behet’s disease can cause a wide range of symptoms, but it’s rare for someone with the condition to have all of them at once. […] Most people experience times when the symptoms improve (remission) and times when they get worse (flare-ups or relapses). […] Almost everyone with Behet’s disease develops mouth ulcers. […] Like mouth ulcers, recurrent genital ulcers are also a common symptom of Behet’s disease. […] Many people with Behet’s disease also develop skin lesions. […] Behet’s disease affects the joints in around 1 in 2 people with the condition, causing arthritis-like symptoms such as pain, stiffness, swelling, warmth and tenderness. […] Inflammation of the eyes is another common symptom of Behet’s disease, occurring in around 7 out of 10 cases. […] In some people with Behet’s disease, the skin is particularly sensitive to injury or irritation.

• #4

  https://www.nhs.uk/conditions/behcets-disease/symptoms/

  Behet’s disease can cause inflammation of the stomach and bowel, which can lead to symptoms such as feeling and being sick, tummy pain, indigestion, loss of appetite, diarrhoea with bleeding. […] The inflammation of the lining of the blood vessels associated with Behet’s disease can sometimes cause blood clots to form. […] A less common type of blood clot associated with Behet’s disease is cerebral venous thrombosis (CVT). […] Inflammation of the blood vessels can cause the walls of your blood vessels to weaken. […] Inflammation of the central nervous system (CNS) causes the most serious symptoms associated with Behet’s disease. […] It’s also common for people with Behet’s disease to experience more general symptoms as a result of the condition, including periods of extreme physical or mental tiredness (fatigue).

• #5 Behcet’s Disease Signs & Symptoms | Rush

  https://www.rush.edu/conditions/behcets-disease

  Sometimes your Behcet’s symptoms look like symptoms for other diseases, such as Crohn’s disease, or your symptoms may emerge slowly over several years. This makes diagnosis tricky. […] Behcet’s disease is treatable but not curable. Symptoms may disappear (go into remission) and come back (flare). […] With effective treatment, flares typically become less frequent.

• #6 Symptoms of Behcet’s Disease

  https://www.behcets.com/symptoms

  Oral ulceration tends to be the earliest manifestation of Behcet’s Disease and often appears before any other signs or symptoms. The ulcers are painful, shallow or deep, round or oval, and may have a white or yellow base with a red halo. Their size varies from 1-20 mm. The ulcers generally affect the buccal mucosa, tongue, lips, gingiva, tonsils, uvula, palate or the pharynx. Multiple lesions may be present during flares, but some patients may experience a single ulcer, while others may have multiple ulcers. The lesions heal within 10-20 days without scarring. […] Genital ulcers occur slightly less often than oral ulcers, developing in approximately 75% of patients. The lesions tend to be larger and deeper and often heal with scarring. […] Cutaneous manifestations of Behcet’s Disease generally occur in 60-90% of patients.

• #6 Symptoms of Behcet’s Disease

  https://www.behcets.com/symptoms

  Ocular inflammation is one of the hallmark manifestations of Behcet’s Disease. Behcet’s Disease is one of a few autoimmune diseases capable of causing both anterior and posterior uveitis. […] Peripheral arthritis or spondylitis develops in approximately 50% of patients with Behcet’s Disease. […] Gastrointestinal involvement develops in approximately 25% of Behcet’s patients. […] Central nervous system involvement may be more common in American and European Behcet’s patients, affecting as many as 30%. The disease tends to involve the white matter of the brain and the brain stem. The neurologic features are variable and include headache, confusion, strokes, personality changes and memory loss.

• #7 Behcet’s Syndrome – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/behcets-disease

  Recurrent mouth ulcers are the hallmark of Behets. Almost all patients have experienced these, and they can predate other symptoms by months or even years. Ulcers can be small, large or multiple. They are often on the inner lips but can also occur on the tongue, the roof of the mouth and occasionally in the throat. Ulcers in Behets do not typically form on the external lips. The ulcers are usually painful and occasionally leave a scar. Mouth ulcers affect up to 20% of otherwise healthy people, so having mouth ulcers alone is not enough for a diagnosis of Behets. Importantly, many other medical conditions can also be associated with mouth ulcers. Most oral ulcers in Behets resolve within 10-14 days, although replacement with new ulcers may make it feel as if they are sustained. If an ulcer is persistent, your doctor may wish to arrange a biopsy to look into the diagnosis.

• #7 Behcet’s Syndrome – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/behcets-disease

  Genital ulcers occur less frequently than oral ulcers. In Europe approximately 60-70 per cent of people with Behets report suffering from them. They resemble mouth ulcers in appearance, are usually painful and, when deep, may heal with scarring. Genital ulcers in females affect the vulva and vagina and occasionally the cervix. In males, ulcers occur typically on the scrotum but may involve the penis. Because of their location, genital ulcers may become infected. They can be triggered by sexual intercourse and, conversely, are an obvious impediment to sex. Genital ulcers should be checked for possible infection, ideally in a genitourinary medicine clinic. As with oral lesions, non-healing ulcers should be biopsied. […] Skin problems are very common in Behets, occurring in about 80 per cent of patients. Usually people will report redness and spots (pustules) resembling acne. Steroid treatment may also cause acne, which can make it hard to establish if the pustules are related to the Behets or are rather a side effect of treatment.

• #7 Behcet’s Syndrome – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/behcets-disease

  Eye inflammation may be a serious problem in some people with Behets. It is thought that 50-70 per cent of people with Behets develop inflammation of the eye. If this is going to happen, it usually starts within 2-3 years of the diagnosis of Behets. It is the first symptom (or the one that leads to the diagnosis) in 10-15 per cent of cases. Eye inflammation usually comes on in unpredictable, repetitive attacks. Specialist help should be sought quickly if any symptoms develop, as prompt treatment is required to avoid damage to sight. […] Headaches are very commonly reported in people with Behets. Over 80 per cent of people will report this symptom. For the vast majority, headaches are not the result of a serious problem in the brain and typically improve with symptomatic treatment. […] Pain in the muscles and joints are frequently found in Behets. Occasionally joint swelling and tenderness (arthritis) occurs, but this is rarely persistent. Even if arthritis does occur, it rarely leads to damage to the joints and seldom becomes a significant cause of disability.

• #7 Behcet’s Syndrome – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/behcets-disease

  Many patients with Behets experience abdominal pains, and symptoms similar to irritable bowel syndrome (bloating, diarrhoea or constipation). Camera tests such as colonoscopy / OGDs are frequently normal despite symptoms, with only about 1 in 10 symptomatic patients having visible ulceration or inflammation on these tests. Symptoms may respond to irritable bowel syndrome treatments or, when ulcers are present, by treatment with immunosuppressive drugs. […] Most patients with Behets report feeling fatigued and this may impact on quality of life. The reasons for fatigue are often complex, often including inflammation, loss of physical fitness, pain, low mood and/or poor sleep. In addition to treatments to suppress the inflammation, attempts to increase fitness (e.g. swimming or pilates), address sleep disturbances, pain and mood may improve this symptom.

• #8 Behcet Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470257/

  Behcet disease is characterized by mucocutaneous manifestations, including recurrent oral and genital ulcerations, ocular manifestations, especially chronic relapsing uveitis, and systemic vasculitis involving arteries and veins of all sizes. […] Oral ulcers occur in 97% to 99% of patients with Behcet disease, often representing the initial clinical feature. Lesions are usually painful, recurrent, and multiple and may involve the soft palate, hard palate, buccal mucosa, tongue, gingiva, lips, and tonsils. More than 90% of oral ulcers heal without scarring. […] Genital lesions are seen in more than 80% of patients with Behcet disease. These lesions are also recurrent, although, in contrast to oral lesions, more than 70% of genital lesions heal with scarring. […] More than 50% of patients with Behcet disease have eye involvement, although it is much more common in males and younger patients. Eye involvement is usually not the presenting feature of Behcet disease but usually occurs within the initial few years of diagnosis and is rare to occur late in the disease if not present earlier.

• #8 Behcet Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470257/

  Inflammatory, non-erosive, non-deforming arthritis is seen in 50% of patients with Behcet disease, more common in patients with acneiform lesions. […] The involvement of arterial and venous tracts of all sizes is a hallmark of Behcet disease and can be seen in 25% of patients, more commonly in males. […] Central nervous system involvement is seen in 5% to 10% of patients with Behcet disease, and 80% of it is parenchymal involvement, most commonly of the brainstem, that leads to cerebellar, pyramidal, and sensory signs and symptoms. […] Mucosal ulcerations resembling the orogenital aphthae can be seen in the terminal ileum, cecum, colon, and esophagus. Extensive ulcerations, especially ileocecal lesions, may lead to perforation. […] Behcet disease has no cure and is associated with significant morbidity and mortality. Poor prognosis and higher mortality are related to the male sex and younger age of onset. Major causes of mortality include ruptured pulmonary and peripheral aneurysms and neurologic and gastrointestinal involvement.

• #9 Behçet’s disease physiopathology: a contemporary review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4751097/

  Recurrent oral ulcers represent the earliest disease manifestation in 47-86% of patients. It may take years for the other symptoms to appear afterwards, and oral ulcers are observed in all patients during their clinical course. Lesions have disciform appearance with round and sharp erythematous border, covered with a grayish-white pseudomembrane or a central yellowish fibrinous base and grow rapidly from a flat ulcer to a deep sore. They may occur as single ulcers or in crops and heal with little scarring; prognosis is usually favorable. […] Genital ulcers develop in 57-93% of patients. They are painful and morphologically resemble oral ulcers, but are larger, deeper, have more irregular margins and heal with white or pigmented scars. Male genital lesions most commonly involve the scrotum and usually leave a scar that will help with the diagnosis retrospectively. In females, vulvar, vaginal and cervical lesions are especially common. Rarely, deep vaginal lesions may perforate the bladder resulting in fistulae.

• #9 Behçet’s disease physiopathology: a contemporary review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4751097/

  Ocular disease, involving the retina and the uvea, occurs in 30-70% of BD patients and is associated with high morbidity. It is the primary cause of blindness in approximately 25% of patients despite aggressive corticosteroid treatment. Ocular symptoms occur more frequently amongst males and are associated with disease severity, even though prognosis is improving with the use of aggressive immunosuppressant therapy. […] Neurological involvement in BD (neuro-BD) occurs in 5-10% of patients and is more frequent in males. It usually occurs around 5 years after the onset of the disease and is associated with long-term morbidity and mortality. Neurological disease affects the central nervous system (CNS) more frequently than the peripheral nervous system. […] Behets disease may affect blood vessels of different sizes and types, including arteries and veins, as well as the heart organ. Cardiovascular features have been reported to affect 7-49% of patients, more frequently in males. They occur 3-16 years after the onset of BD.

• #9 Behçet’s disease physiopathology: a contemporary review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4751097/

  Articular involvement occurs in 45-60% of patients and includes arthralgia, monoarthritis or polyarthritis. Non-erosive, non-deforming oligoarthralgia commonly involving the knees, ankles, elbows, and wrists is the most frequent manifestation. […] Gastrointestinal involvement occurs in 3-26% of patients and varies among different populations. It is much more frequent in Japan than in the Middle East and the Mediterranean region. Mucosal inflammation and ulceration occur throughout the gastrointestinal tract and are mostly common in the ileocecal region. […] Irrespective of recent therapeutic advancements, the clinical prognosis of BD patients remains unfavorable. The primary goal is to focus on identifying the pathogenesis of the disease and providing therapies that inhibit the underlying autoimmune inflammatory cascade. Current research points towards targeted cytokines that play a role in the physiopathology of Behets disease.

• #9 Behçet’s disease physiopathology: a contemporary review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4751097/

  Behets disease typically appears between the third and the fourth decades of age, infrequently developing before puberty or after the fifth decade of life. Earlier age onset is correlated with more severe clinical manifestations and mortality. The disease appears to occur more frequently and is associated with more severe ocular, neurological, and cardiovascular clinical manifestations in males. […] As a systemic vasculitis disease, BD may affect almost all vascularized systems and is characterized by episodes of relapses and remissions with sequelae. Although several clinical manifestations are associated with BD, the triple-system complex of oral and genital aphthae, and uveitis first described by Behet in 1937 generally illustrates disease pattern. Children exhibit more frequent perianal aphthosis and arthralgia, less frequent genital ulcers and vascular involvement and a more severe course of uveitis. Over the past few years, modern treatment strategies, involving immunosuppressant therapy, and the use of aggressive approaches have led to improvements in the prognosis of severe forms of BD. Prognosis for the disease is usually reserved, especially when ocular, cardiovascular, neurological, and gastrointestinal manifestations appear.

• #10 Behcet’s Disease : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/behcets-disease/

  Painful sores in the mouth called aphthous ulcers. These are very similar in appearance to ulcers that frequently occur in the general population, usually as a result of minor trauma. In Behcets, however, the lesions are more numerous, more frequent, and often larger and more painful. Aphthous ulcers can be found on the lips, tongue, and inside of the cheek. Aphthous ulcers may occur singly or in clusters, but occur in virtually all patients with Behcets. […] Pustular skin lesions that resemble acne, but can occur nearly anywhere on the body. This rash is sometimes called folliculitis. Skin lesions called erythema nodosum: red, tender nodules that usually occur on the legs and ankles but also appear sometimes on the face, neck, or arms. Unlike erythema nodosum associated with other diseases (which heal without scars), the lesions of Behcets disease frequently ulcerate.

• #10 Behcet’s Disease : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/behcets-disease/

  Behcets disease is virtually unparalleled among the vasculitides in its ability to involve blood vessels of nearly all sizes and types, ranging from small arteries to large ones, and involving veins too. Because of the diversity of blood vessels it affects, manifestations of Behcets may occur at many sites throughout the body. However, the disease has a predilection for certain organs and tissues; these are described below. […] Behcets may cause either anterior uveitis (inflammation in the front of the eye) or posterior uveitis (inflammation in the back of the eye), and sometimes causes both at the same time. Anterior uveitis results in pain, blurry vision, light sensitivity, tearing, or redness of the eye. Posterior uveitis may be more dangerous and vision-threatening because it often causes fewer symptoms while damaging a crucial part of the eye the retina.

• #10 Behcet’s Disease : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/behcets-disease/

  Ulcerations may occur anywhere in the gastrointestinal tract from the mouth to the anus. The terminal ileum and cecum are common sites. Involvement of the GI tract by Behcets may be difficult to distinguish from inflammatory bowel disease (such as Crohns disease). […] Clots can occur in veins in any site, most often including veins in the lower extremity (superficial or deep venous thrombosis). Inflammation in arteries can occur as well, such as the pulmonary or abdominal arteries, sometimes causing obstruction of the vessel (thrombosis).

• #10 Behcet’s Disease : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/behcets-disease/

  Aneurysms (outpouchings of blood vessel walls, caused by inflammation) of arteries in the lung, rupture of which may lead to massive lung hemorrhage. […] Arthritis or arthralgias (pain in the joints not accompanied by joint swelling). […] Central nervous system involvement is one of the most dangerous manifestations of Behcets. The disease tends to involve the white matter portion of the brain and brainstem, and may lead to headaches, confusion, strokes, personality changes, and (rarely) dementia. Behcets may also involve the protective layers around the brain (the meninges), leading to meningitis. Because the meningitis of Behcets disease is not associated with any known infection, it is often referred to as aseptic meningitis. […] Male painful genital lesions that form on the scrotum, similar to oral lesions, but deeper. Female painful genital ulcers that develop on the vulva.

• #11 Behçet disease: Background, Treatment and More

  https://dermnetnz.org/topics/behcet-disease

  Behet disease (BD) is a rare, multi-system condition characterised by recurrent painful oral and genital ulcers, a variety of skin lesions, and eye problems. […] Many other organs such as the gut, nervous system, lungs, and arteries may also be affected. […] Oral ulcers anywhere in the mouth, pharynx, and tonsils. […] Genital ulcers painful, recurrent ulcers on the vulva, vagina, perineum and inguinal areas, scrotum, and penis. […] Ophthalmic lesions seen in 70% of patients, and include: Anterior and/or posterior uveitis, Retinal vasculitis, Scleritis, Episcleritis, Thrombosis of the retinal artery and vein, Optic neuritis. […] Musculoskeletal symptoms include: A mono- or oligoarthritis of the medium and large joints, Fatigue, Fibromyalgia. […] Vascular involvement is present in 25% of patients, resulting in: Superficial thrombophlebitis and deep vein thrombosis, Thrombosis of the vena cava, dural sinus, and Budd-Chiari syndrome, Arterial manifestations resulting in stenosis, thrombosis, and aneurysm formation.

• #12 Clinical Presentation of Behçet’s Disease – Decisions in Dentistry

  https://decisionsindentistry.com/article/clinical-presentation-of-behcets-disease/

  Genital ulcers are seen in approximately 75% of patients with BD. Lesions affect the vulva, vagina and cervix in women, and the scrotum and shaft of the penis in men. […] Eye involvement affects nearly 50% of patients with BD. Unless treated, recurrent attacks of panuveitis and retinitis can result in irreversible damage and vision loss. […] Skin lesions of BD appear red and raised, or as bruises or pus-filled bumps that leave faint scars or discolorations; some may develop eruptions that resemble acne. […] The short- and long-term outcomes of patients with BD depend on the patients gender, age at disease onset and disease duration. The two main goals in management are to prevent damage caused by repeated exacerbations of inflammation in major organs, such as the eyes, vascular system, central nervous system and gastrointestinal system, and to prevent skin, mucosa and joint lesions.

• #13 Behcet Disease – EyeWiki

  https://eyewiki.org/Behcet_Disease

  BD is commonly known as a triad of oral aphthous ulcers, genital ulcers, and ocular disease. […] The most common clinical feature, affecting 98-99% of patients, are oral aphthous ulcers. The second most common finding is genital ulcer, which occur in 80-87% of patients. Skin disease is another common finding occurring in up to 69-90%. An asymmetric, non deforming, large-joint polyarthritis can be seen in 44-59% of patients. […] Ocular manifestations carry the most serious implications, affecting roughly 70% of patients. The severity is due to the explosive and recurrent nature of the uveitis, which can lead to permanent, often irreversible, ocular tissue damage. Up to 25% of patients with BD suffer from severe vision loss. […] The clinical finding of nongranulomatous anterior uveitis in BD patients usually presents with a transient hypopyon in 25% of cases. Patients will have signs and symptoms of redness, tearing, pain, photophobia, and blurry vision.

• #13 Behcet Disease – EyeWiki

  https://eyewiki.org/Behcet_Disease

  Severe sight threatening manifestations are related to posterior segment involvement. The most common form of uveitis with BD is an obliterative, necrotizing retinal vasculitis that has the unique feature of affecting both the arteries and the veins in the fundus. […] The most common cause of visual impairment is optic nerve atrophy. […] Behcet’s Disease affects the central nervous system in about 23% of all patients with the disease in the United States. […] Vision loss is the greatest cause of morbidity in BD patients and the prognosis for vision is guarded. The most common causes for significant visual disability worldwide are due to macular edema, occlusive retinal vasculitis, optic atrophy, and glaucoma that develop as manifestations of ocular BD. […] Given the heterogeneous collection of BD-associated eye complications and the unpredictable relapses and exacerbations, a concrete prognosis of visual outcomes is difficult.

• #14 Behçet’s disease – Wikipedia

  https://en.wikipedia.org/wiki/Beh%C3%A7et%27s_disease

  Behet’s disease is a type of inflammatory disorder which affects multiple parts of the body. The most common symptoms include painful sores on the mucous membranes of the mouth and other parts of the body, inflammation of parts of the eye, and arthritis. The sores can last from a few days, up to a week or more. Less commonly there may be inflammation of the brain or spinal cord, blood clots, aneurysms, or blindness. Often, the symptoms come and go. […] Diagnosis is based on at least three episodes of mouth sores in a year, together with at least two of the following: genital sores, eye inflammation, skin sores, a positive skin prick test. […] Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20 percent of cases. Ocular involvement can be in the form of posterior uveitis, anterior uveitis, or retinal vasculitis. Anterior uveitis presents with painful eyes, conjunctival redness, hypopyon, and decreased visual acuity, while posterior uveitis presents with painless decreased visual acuity and visual field floaters.

• #15 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1122381-clinical

  Without treatment, the visual prognosis of uveitis from Behcet disease is very poor, with blindness occurring in more than 75% of patients. In one study, rates of visual impairment (20/50 or worse) and blindness (20/200 or worse) were 12% per year and 9% per year, respectively. […] The severity of Behcet disease may be trending downward; the risk for blindness has steadily fallen from 75% to 25%. Research has focused on the rising use of immunomodulatory and immunosuppressive medications to explain much of this trend; however, researchers in Japan also believe other factors, including lifestyles and hygiene, could play a role.

• #15 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1122381-clinical

  Behcet disease is a rare vasculitic disorder that is characterized by recurrent oral aphthous ulcers, genital ulcers, and uveitis. The systemic manifestations can be variable. Ocular disease has the greatest morbidity, followed by vascular disease, generally from active vasculitis. Cutaneous manifestations can occur in up 75% of patients with Behcet disease and can range from acneiform lesions, to nodules and erythema nodosum. Gastrointestinal (GI) manifestations can be severe; thus, differentiating Behcet disease from active inflammatory bowel disease can often be clinically difficult. […] Prognosis is related to the site and severity of involvement. Aneurysms are an especially feared complication. Thrombotic events and vasculitis may lead to ischemia distal to vascular lesions. Uncontrolled ophthalmologic involvement in the form of anterior and posterior uveitis can lead to vision loss. Neurologic involvement suggests progressive disease and can lead to permanent deficits or even death.

• #15 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1122381-clinical

  The disease usually runs a protracted course, with attacks generally lasting for several weeks and recurring more frequently early in the disease. Mucocutaneous and arthritic involvement typically occur early. […] The mortality rate is low, but death can occur from neurologic involvement, vascular disease, bowel perforation, cardiopulmonary disease, or as a complication of immunosuppressive therapy. A Korean study found that the risk of death is highest during the first year after diagnosis. […] Mortality and morbidity data include the following: The mortality rate in a cohort of 817 patients in France was 5% at a median follow-up of 7.7 years. […] Vascular involvement is one of the major causes of morbidity and mortality in Behcet disease, especially in males from the Middle East and Eastern Mediterranean. An association between cerebral sinus venous thrombosis and pulmonary artery involvement, intracardiac thrombosis and pulmonary artery involvement, and Budd-Chiari syndrome and inferior vena cava syndrome was reported. Lower extremity vein thrombosis frequently accompanies these combinations and often occurs prior to the other vascular involvements.

• #16 Behcet’s Disease Syndrome Causes, Symptoms, Diagnosis, Treatment

  https://www.medicinenet.com/behcets_syndrome/article.htm

  Joint inflammation (arthritis) can lead to swelling, stiffness, warmth, pain, and tenderness of joints with Behet’s syndrome. This occurs in about half of patients with Behet’s syndrome at sometime during their lives. Knees, wrists, ankles, and elbows are the most common joints affected. The skin of patients with Behet’s syndrome can develop areas of inflammation that spontaneously appear as raised, tender, reddish nodules (erythema nodosum), typically on the front of the legs. Some patients with Behet’s syndrome develop a peculiar red or blistery skin reaction in places where they have been pierced by blood-drawing needles (see pathergy test in diagnosis section). Research has found that acne occurs more frequently in patients with Behet’s syndrome that also have arthritis as a manifestation. […] Ulcerations can occur at any location in the stomach, large or small bowel in patients with Behet’s disease.

• #17 Behçet’s Disease: an Insight from a Cardiologist’s Point of View

  https://opencardiovascularmedicinejournal.com/VOLUME/4/PAGE/63/FULLTEXT/

  Behet’s disease (BD) is an enigmatic inflammatory disorder, with vasculitis (perivasculitis) underlying pathophysiology of its multisystemic affections. Venous pathology and thrombotic complications are hallmarks of BD. However, it has been increasingly recognized that cardiac involvement and arterial complications (aneurysms, pseudoaneurysms, rupture and thrombosis) are important part of the course of BD. Pericarditis, myocardial (diastolic and/or systolic dysfunction), valvular and coronary (thrombosis, aneurysms, rupture) involvement, intracardiac thrombi (predominantly right-sided) are, probably, the most frequent cardiac manifestations. […] Estimates of cardiovascular involvement in BD reach 7-46%, with lethal outcomes in about 20% of severe cases. As it is shown in multiple series of large observations, recurrent thrombophlebitis is the commonest vascular affection in BD. Generally, venous involvement is reported in 29% and arterial in 8-16% of cases. Affections of inferior vena cava and aorta bear the worst prognosis for patients with BD. Blood clots can develop in the deep veins of the lower extremity and migrate to the right heart and pulmonary arteries. Destruction of elastic structures of aorta can result in formation of aneurysms and pseudoaneurysms prone to rupture. Aneurysms of smaller vessels can also develop, sometimes spontaneously. The risk of aneurysms and pseudoaneurysms is especially high in those, undergoing major (coronary artery bypass grafting, angioplasty of aorta and its branches) or even minor vascular interventions (e.g., arterial puncture).

• #18 Neurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome | Practical Neurology

  https://pn.bmj.com/content/23/5/386

  Neurological involvement in Behets syndrome arises predominately through an inflammatory meningoencephalitis characterised by perivenular inflammation due to activation of Th-17 immunological pathways. The brainstem is involved in 50% of cases, the diencephalon and other areas of the brain in 30%, and the spinal cord in 10%. Movement disorders and epilepsy may occur. […] Eighty per cent of cases begin with a relapsing disease course, of whom 70% have only one attack, and 30% have a progressive disease course either from onset or following an initially relapsing course. […] The neurological syndrome is heralded by a subacute worsening (or first development) of the systemic features of mucosal ulceration, fatigue, aching joints and skin lesions. A headache develops, worsens over several days and becomes meningitic. The neurological symptoms arise a week after onset and worsen subacutely. Drowsiness is common and may progress to a profound encephalopathy or stupor.

• #18 Neurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome | Practical Neurology

  https://pn.bmj.com/content/23/5/386

  All large series note that 50% of cases are associated with an inflammatory lesion of the brainstem. […] Thirty per cent have cerebral hemisphere lesions. Patients present with an increasing encephalopathy with focal neurological signs determined by the site and extent of the inflammatory lesion(s), including cognitive dysfunction such as dysmnesia, dysphasia and visual field defects. […] The chronic progressive disease course is associated with poor outcome and greater risk of death. […] Measurements of outcome suggest that 35%45% of patients become disabled over time.

• #19 Behçet Disease – Bone, Joint, and Muscle Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/bone-joint-and-muscle-disorders/vasculitic-disorders/beh%C3%A7et-disease

  Inflammation of blood vessels (vasculitis) throughout the body can cause blood clots to form in the arteries and bulges (aneurysms) to develop in weakened blood vessel walls. Vasculitis can result in strokes if arteries to the brain are affected. It can cause kidney damage if arteries to the kidneys are affected. If arteries in the lungs are affected, bleeding may occur, and people may cough up blood. […] Symptoms may include discomfort and pain in the abdomen, cramping, diarrhea, and sores in the intestines. Symptoms may be similar to those caused by inflammatory bowel disease (Crohn disease and ulcerative colitis). […] Inflammation of the brain or spinal cord is less common but has serious consequences. People may have a headache first. Other symptoms include a fever and stiff neck (symptoms of meningitis), confusion, and loss of coordination. Changes in personality and memory loss may develop years later.

• #20 The facts about Behcet’s Syndrome.

  https://www.gbhealthwatch.com/behcetssyndrome-details.php

  Behets disease is now recognized as a chronic condition that causes canker sores or ulcers in the mouth and on the genitals, and inflammation in parts of the eye. In some people, the disease also results in arthritis (swollen, painful, stiff joints), skin problems, and inflammation of the digestive tract, brain, and spinal cord. […] Behets disease affects each person differently. Some people have only mild symptoms, such as canker sores or ulcers in the mouth or on the genitals. Others have more severe signs, such as meningitis, which is an inflammation of the membranes that cover the brain and spinal cord. Meningitis can cause fever, a stiff neck, and headaches. More severe symptoms usually appear months or years after a person notices the first signs of Behets disease. Symptoms can last for a long time or may come and go in a few weeks. Typically, symptoms appear, disappear, and then reappear. The times when a person is having symptoms are called flares. Different symptoms may occur with each flare; the problems of the disease often do not occur together.

• #21 How is Behçet’s diagnosed?

  https://behcetsuk.org/how-is-behcets-diagnosed/

  Along with 1 out of the 4 ‘hallmark’ symptoms above, along with 2 of the following symptoms: arthritis/arthralgia, nervous system symptoms, stomach and/or bowel inflammation, deep vein thrombosis, superficial thrombophlebitis, cardiovascular problems, inflammatory problems in chest and lungs, problems with hearing and/or balance, extreme exhaustion, changes of personality, psychoses, any other member of the family with a diagnosis of Behçet’s. […] Must have: mouth ulcers (any shape, size or number at least 3 times in any 12 months) along with 2 out of the next 4 ‘hallmark’ symptoms: genital ulcers, skin lesions, eye inflammation, pathergy reaction.

• #22 What is Behçets?

  https://behcetsuk.org/what-is-behcets/

  Most of the symptoms are painful but not life-threatening. However, when the disease affects major parts of the body, such as the eyes or brain, it can cause serious consequences including blindness or strokes – but this is fortunately rare. […] Behçet’s symptoms typically come and go in a series of attacks (flare-ups) throughout life. The disease can often, but not always, become less severe with increasing age, where flare-ups become less aggressive and happen less frequently. […] Most people with Behçet’s have a normal lifespan and, with appropriate medical help, can hope to lead close-to-normal daily lives.

• #23 Behçet’s Disease (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/behcets-disease-pro

  Although the disease usually develops in young adulthood, it is reported that about 15-20% of all Behet’s patients develop the condition in childhood. Paediatric Behet’s disease differs from adult. Gastrointestinal system involvement, neurological findings, arthralgia and positive family history are more common in children, while genital lesions and vascular lesions are more common in adult patients. […] There is a very variable course of recurrences and remissions lasting for years. […] The majority of new manifestations occur within the first five years after onset of the disease. […] Prognosis will depend on which systems are involved. Men tend to have a poorer prognosis. Mortality is usually low but death may occur as a result of neurological involvement, vascular disease, bowel perforation, cardiopulmonary disease, or as a complication of immunosuppressive therapy.

• #24 Behçet Disease – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/beh%C3%A7et-disease

  Central nervous system involvement is less common but is serious. Onset may be sudden or gradual. The first manifestations may be parenchymal involvement with pyramidal signs, small-vessel disease with a pattern resembling that of multiple sclerosis, nonparenchymal involvement with aseptic meningitis or meningoencephalitis, or dural sinus thrombosis. […] Behet disease typically has a waxing and waning course characterized by exacerbations and remissions. Prognosis tends to be worse in young males. Risk also appears to be higher if patients have an HLA-B51 allele. Mucocutaneous and ocular lesions and arthralgias are often worse early in the disease. Central nervous system and large-vessel manifestations, if they develop, typically occur later. Occasionally, the disease results in death, usually due to neurologic, vascular (eg, aneurysms), or gastrointestinal manifestations. Risk of death is highest for young males and patients with arterial disease or a high number of flare-ups. Many patients eventually go into remission.

• #25 What is Behcets Disease

  https://behcetdiseasesociety.org/menu/25/what-is-behcets-disease

  Behcet`s disease is a long term problem; there will be times when patient can be free of symptoms (and these times are called remissions), and this does not mean that the disease is cured, usually new symptoms develop later, (central nervous system involvement and major vessel disease could appear late during the course of Behcet`s disease after 5-10 years). The frequency of oral and genital ulcers and joint symptoms might decrease with time. About 25% of patients with eye disease might develop severely impaired vision and this does not always mean an eventual loss of useful vision. The right treatment may prevent new symptoms from appearing and control the existing ones. The mortality between patients with Behcet`s disease is about 9%, mainly because of large vessel disease and neurological involvement. Male patients who develop the disease at younger ages (before 25 years) might be at greater risk to have a more severe disease.

• #26 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329099-overview

  The disease usually runs a protracted course, with attacks generally lasting for several weeks and recurring more frequently early in the disease. Mucocutaneous and arthritic involvement typically occur early. […] The mortality rate is low, but death can occur from neurologic involvement, vascular disease, bowel perforation, cardiopulmonary disease, or as a complication of immunosuppressive therapy. A Korean study found that the risk of death is highest during the first year after diagnosis. […] Vascular involvement is one of the major causes of morbidity and mortality in Behcet disease, especially in males from the Middle East and Eastern Mediterranean. An association between cerebral sinus venous thrombosis and pulmonary artery involvement, intracardiac thrombosis and pulmonary artery involvement, and Budd-Chiari syndrome and inferior vena cava syndrome was reported. Lower extremity vein thrombosis frequently accompanies these combinations and often occurs prior to the other vascular involvements.

• #26 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329099-overview

  Without treatment, the visual prognosis of uveitis from Behcet disease is very poor, with blindness occurring in more than 75% of patients. In one study, rates of visual impairment (20/50 or worse) and blindness (20/200 or worse) were 12% per year and 9% per year, respectively. […] The severity of Behcet disease may be trending downward; the risk for blindness has steadily fallen from 75% to 25%. Research has focused on the rising use of immunomodulatory and immunosuppressive medications to explain much of this trend; however, researchers in Japan also believe other factors, including lifestyles and hygiene, could play a role.

• #27 Behcet’s Disease Information — Dr Jeffrey Chaitow

  https://www.jeffchaitowpractice.com.au/behcets-disease

  Skin involvement: There are different skin lesions. […] Eye involvement: This is one of the most serious symptoms of the disease. […] Joint involvement: Joints are involved in about 30-50% of children with BD. […] Nervous system involvement: Rarely, children with BD can develop problems with their nervous system. […] Blood vessel involvement: Blood vessel involvement is seen in about 12-30% of juvenile BD patients and can signal a poor outcome. […] Gastrointestinal involvement: This is especially common in patients from the Far East. […] There are also some differences between girls and boys. Boys usually experience a more severe disease course, with more eye and blood vessel involvement, than girls. […] The disease can go into remission, but may have flare-ups. It can be controlled, but not cured. […] The overall activity generally decreases with time. […] Children with milder disease may recover, but the majority of paediatric patients have long periods of remission followed by flare-ups of the disease.

• #28

  https://www.singhealth.com.sg/patient-care/conditions-treatments/behcets-disease

  Behcets disease, also called Behcets syndrome, is a rare disease that mainly affects young adults. It causes inflammation in many parts of the body including the eyes, mouth, skin, genitals, joints, blood vessels and nervous system. […] The first symptoms often appear between the age of 20 and 30. The disease tends to be more severe in men and when the onset is at an earlier age. […] Signs and symptoms of Behcet’s disease may vary from person to person and also depending on which parts of the body are affected. […] Painful mouth ulcers are the most common sign of Behcets disease. Such ulcers can occur on the inner surface of the lips, gums, cheeks or tongue. They usually heal within 7 to 10 days but often recur. […] Skin rashes are also common in Behcets disease. Some patients develop acne-like rashes on their body, while others may develop red, raised and tender nodules on their shins. Skin rashes may clear up on their own within 10 to 14 days, but they usually recur. […] Patients with Behcets disease may develop painful ulcers on their genitals. These ulcers most commonly occur on the penis or the vulva, as round, red and ulcerated lesions. They are not a form of sexually transmitted disease. […] Behcets Disease may cause inflammation of the eyes, known as uveitis, which presents with redness, pain and blurred vision. If not treated promptly, it can lead to blindness. […] Behcets Disease can cause arthritis. The affected joints become swollen, red and painful due to inflammation. Joints that are commonly affected are the knees, ankles, elbows and wrists. […] Inflammation of the veins and arteries may occur, resulting in redness, pain and swelling in the arms or legs. Occasionally such inflammation can lead to blood clots in the blood vessel (thrombosis). […] A variety of signs and symptoms may affect the digestive system, including abdominal pain, diarrhoea or bleeding from the intestines. […] Inflammation in the brain and nervous system may occur, causing headaches, fever, disorientation, poor balance and stroke.

• #29 Neuro-Behçet’s disease – Wikipedia

  https://en.wikipedia.org/wiki/Neuro-Beh%C3%A7et%27s_disease

  Some of the less common symptoms include stroke (1.5%), epilepsy (2.25%), brain tumors, movement disorders, acute meningeal syndrome, and optic neuropathy. […] The main clinical characteristic is cerebral venous thrombosis (CVT). If one experiences CVT, a clot in one of the blood vessels of the brain blocks the blood flow and may result in stroke. This happens in the dural venous sinuses. Stroke-like symptoms such as confusion, weakness, and dizziness may be monitored. Headache tends to worsen over a period of several days. Some of the less common symptoms include intracranial hypertension and intracranial aneurysms. […] In one study of 387 Behet’s disease (BD) patients that has been done for 20 years, 13% of men with BD developed to NBD and 5.6% of women developed to NBD. Combining all statistical reports, approximately 9.4% (43 of 459) BD patients advanced to NBD. In addition, men were 2.8 times more likely to experience NBD than women. This fact indicates possible gender-based pathology.

• #30 Learn About Behcet’s Disease Symptoms & Manifestations, Causes, and Management | Behcet’s Connection – Celgene Corporation

  https://www.behcetsconnection.com/about-behcets

  Behets Disease is a rare, chronic, multisystem inflammatory disease. The most common manifestation is mucocutaneous lesions accompanied by lesions in other organ systems. Behets disease affects multiple organ systems and presents to multiple different manifestations. The manifestations of Behets Disease are recurring and remitting and may not occur simultaneously. They may present as seemingly isolated conditions and are often treated by different medical specialists, depending on the system affected. For example, oral ulcers, the most common onset manifestation, are often treated by a dentist or primary care physician. Similarly, a dermatologist will typically see and treat skin manifestations, while a gynecologist or urologist may treat genital ulcers. The manifestations of Behet’s Disease vary widely in type and severity, with the potential to impact multiple organ systems. However, nearly every person with Behets has frequent, recurrent oral ulcers, and typically, multiple ulcers at once. These ulcers are painful and can be refractory to treatment. Often the first manifestation of Behets, oral ulcers contribute substantially to diminished quality of life (QoL) as a result of their debilitating impact on daily functions such as chewing, swallowing, and speaking. Recurrent oral ulcers in Behets tend to crop and scar. In addition to oral ulcers, many people experience other painful manifestations such as arthritis, genital ulcers, and skin lesions. The pain caused by these manifestations can also negatively impact QoL, with visible manifestations also affecting body image.

• #31 Qualifying for SSDI for Behcet’s Disease | Boston Disability Lawyer | Keefe Disability Law

  https://www.keefelaw.com/library/qualifying-for-ssdi-for-behcet-s-disease-boston-disability-lawyer.cfm

  If the symptoms experienced by someone with Behcets disease are severe enough, they may be unable to work. Scientific evidence supports the notion that Behcets disease can be sufficiently debilitating to prevent a person from working. Disability among Behcets patients is high, causing many of them to give up or change their work. The top-cited reason for stopping work was oral ulcerations affecting speech, followed by fatigue. […] With no known cure, treating Behcets disease is about managing symptoms. Medication can help reduce pain and inflammation in milder cases. The SSA requires medical evidence to support your SSDI claim. A doctor inserts a sterile needle and examines the area in a couple of days. Small red bumps are a sign of an overactive immune system, which could indicate Behcets syndrome.

• #32 Medication for Behcet’s Disease | NYU Langone Health

  https://nyulangone.org/conditions/behcets-disease/treatments/medication-for-behcet-s-disease

  Medication can be essential to treating the symptoms of Behets disease, which may come and go unpredictably, with periods of active symptoms, or flares, and periods of dormancy when symptoms disappear, known as remissions. […] Most people with Behets disease have painful, recurrent mouth ulcers. […] Medication can help put Behets disease into remission, a period of time when the signs and symptoms of the condition disappear. But symptoms can return, or flare up, at any time, and its impossible to predict when that might happen. […] To maintain remission, you may take immunosuppressants with or without corticosteroids for several years. […] Behets disease is a disease that improves with increasing age, and many people achieve medication-free remission with time.

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