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Behçet Disease

Behcets Syndrome

Behcet’s syndrome or Behcet’s disease is a multisystem inflammatory disease characterized by oral aphthous ulcers and a myriad potential systemic manifestations.


Presentation

Behcet’s syndrome is confirmed in the presence of any two of the following criteria:

One of the first symptoms to appear in the case of Behcet’s syndrome is aphthous or herpetiform oral lesions. These lesions have a high recurrence rate and may be found as multiple lesions. Skin lesions are also present in the genital regions. Lesions may be found in scrotum or on the penile shaft. In females lesions develop in the labia, vagina or perineum. These lesions heal with scarring. Tender, erythematosus nodules may develop in the lower extremities and resolve within a few weeks. These nodules are common in females and have a high recurrence rate. In males, acneiform nodules may arise on the trunk and extremities. Ulcerations may occur in axillae, neck, breast and interdigital skin [10].

Oral ulcerations are often followed by ocular manifestations including anterior and posterior uveitis, hypopyon, retinal vasculitis and cystoid macular degeneration. Patients with ocular manifestations may present with blurred vision, photophobia, increased lacrimation and periorbital pain. Ocular symptoms are found to be severe in Iranian and Japanese population. Posterior uveitis may lead to blindness.

As the disease progresses, neurologic manifestations develop. These symptoms often develop 7 to 8 years after the onset of the disease. Most of the neurologic symptoms arise due to the involvement of brain stem. Memory loss is the most common manifestation. Other common neurologic signs include seizures, difficulty in speech, deafness, and clonus.

Pulmonary artery aneurysm may lead to hemoptysis, cough, chest pain and dyspnea [11]. Many small and large blood vessels develop vasculitis and manifestations depend on location of the lesions. Superficial thrombophlebitis with erythema is associated with venous involvement. Deep vein thrombosis is seen in some patients. About 60% of the patients may have arthritis and arthralgia, particularly in knees, ankles, wrists and elbows. Cardiac involvement in the form of coronary vasculitis, pericarditis, myocarditis, endocarditis and diastolic anomalies is also observed in some.

Fever
  • High fever flares and digestive involvement starting in early childhood seem to be hallmarks of HA20 clinical features.[ncbi.nlm.nih.gov]
  • We present a 15-year-old-boy with six months of history of fever, dizziness, tinnitus and ataxia. He had a final diagnosis of AIED associated to BD and was successfully treated with the anti-tumor necrosis factor (TNF)-α adalimumab.[ncbi.nlm.nih.gov]
  • The first symptom of the disease was fever. However,the HSV type 1 IgG and HSV type 2 IgM antibody results were positive in our case and,which rendered this case unique. BD and SS concomitantly affect the individual,and BD was the acute type.[ncbi.nlm.nih.gov]
  • Clinical Manifestations Oral, eye or genital sores which endure and do not close Pale colored, flat or concave lesions Lassitude Lack of strength Anorexia Abdominal distention and discomfort Loose stools Long, clear urination Cold limbs Maybe low-grade fever[americandragon.com]
  • Other manifestations included Stevens-Johnson-like eruption, fever of unknown origin, and testicular involvement. All of the patients responded to glucocorticoids; two were also treated with colchicine and one was treated with chlorambucil.[pediatrics.aappublications.org]
Turkish
  • The cases consisted of Western Europeans, Middle Eastern and Turkish individuals. Participants from the Generation R study, a multiethnic birth cohort in Rotterdam, The Netherlands were used as controls.[ncbi.nlm.nih.gov]
  • Researchers conducted a genome-wide association study (GWAS) that enrolled 1,209 Turkish people affected by Behcet's disease and 1,278 unaffected Turkish people - all residents of the country.[disabled-world.com]
  • In this study, we aimed to investigate the therapeutic approaches chosen by Turkish physicians during the initial event and relapses of VBD and the association of different treatment options with the relapses retrospectively.Patients with BD (n   936,[ncbi.nlm.nih.gov]
  • Clinical Utility The HLA class I molecule HLA-B51 is the most strongly associated risk factor and has been shown to be associated with Behçet’s in Turkish and Asian patients, though the association in Caucasian patients is much weaker.[kashilab.com]
  • Wikipedia Symptoms are caused by inflammation in small blood vessels, and consequent oral ulcers, eye problems, or arthritis. rs3024490 (each A allele increases risk about 1.4 times in a Turkish population) [ PMID 20622878 ] rs1800871 (each G allele lowers[snpedia.com]
Fatigue
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: November 24, 2018[patientslikeme.com]
  • […] chill in the fall Oral or genital sores which are not particularly painful Sores may be accompanied by cold pain The sores are pale red The sores tend to grow hollow Dry, rough, painful eyes A somber, white facial complexion Dizziness Head distention Fatigue[americandragon.com]
  • Some less common problems that can be caused by Behcet disease include: Fever and fatigue Blood clots in the veins of the legs (thrombophlebitis) Obstructed blood vessels causing symptoms in other parts of the body Inflammation of the brain or meninges[saintlukeshealthsystem.org]
  • Review of Systems She endorses persistent fatigue, headaches, dry eyes, sinusitis, dry mouth, oral ulcers, reflux, constipation, previous kidney stones, and infrequent menses.[webeye.ophth.uiowa.edu]
  • The primary side effects of IFN- α therapy are flulike symptoms (fever, chills, headache, fatigue, myalgia, etc.) that start a few hours after the initiation of the therapy and continue less than a day.[behcets.info]
Malaise
  • Presentation [ 1 ] Nonspecific symptoms include tiredness, malaise, muscle pains, and transient fevers. Headaches can be common.[patient.info]
  • Although oligozoospermia, amenorrhea, or dysmenorrhea, malaise, hair loss, gastrointestinal complaints (nausea, vomiting, diarrhea), and hematologic side effects are recorded as the main adverse effects of colchicine, Yurdakul et al. [ 38 ] reported no[behcets.info]
Reiter's Syndrome
  • Besides finding these signs, the doctor must rule out other conditions with similar symptoms, such as Crohn's disease and Reiter's syndrome.[healthieryou.com]
Diarrhea
  • HA20 looks like BD if we consider recurrent oral (87%) and genital (67%) ulcers, arthralgia or arthritis (42%), skin involvement (53%) such as erythema nodosum or abdominal symptoms (60%) such as abdominal pain, digestive ulcers or diarrhea.[ncbi.nlm.nih.gov]
  • Some symptoms may include mouth and genital ulcers, eye inflammation and reduced vision, skin rashes and lesions, joint swelling, abdominal pain and diarrhea.[sciencedaily.com]
  • Common side effects of colchicine include nausea, vomiting, and diarrhea. The doctor can decrease the dose to relieve these side effects.[healthieryou.com]
  • Symptoms vary from mild abdominal discomfort to severe inflammation of the large intestine and rectum accompanied by diarrhea or bleeding.[rarediseases.org]
Chronic Diarrhea
  • I have gastrointestinal symptoms, ulcers throughout my GI tract, severe abdominal pains and chronic diarrhea, nausea/vomiting, eye inflammation— specifically Uveitis/Iritis , joint inflammation with severe body pain of varying degrees, lung inflammation[globalgenes.org]
Recurrent Oral Ulceration
  • Behçet disease is a multisystem vasculitis characterised by recurrent oral ulceration in conjunction with other manifestations.[ncbi.nlm.nih.gov]
  • We report the case of a 1-month-old infant who presented with severe recurrent genital ulcerations and at 6 months developed recurrent oral ulcerations. Her orogenital ulcerations continue to recur.[ncbi.nlm.nih.gov]
  • With a history of recurrent oral ulcers, fluorescein angiography showing obliterative retinal vasculitis in the right eye, and steroid responsive optic neuropathy in the left eye, we made the diagnosis of incomplete Behçet disease.[ncbi.nlm.nih.gov]
  • Englin RP, Lehner T, Subak-Sharpe JH: Detection of RNA complementary to herpes simplex virus in mononuclear cells from patients with Behcets syndrome and recurrent oral ulcers. Lancet 2:1356–1361, 1982. CrossRef Google Scholar 9.[link.springer.com]
Aphthous Stomatitis
  • A 19-year-old woman with a history of recurrent aphthous stomatitis and genital ulceration was diagnosed with Behçet disease.[ncbi.nlm.nih.gov]
  • Behcet disease aphthous stomatitis genital ulcer iritis Received December 28, 1987. Accepted February 25, 1988.[pediatrics.aappublications.org]
  • Symptoms The most frequent sign is aphthous stomatitis, (inflammation of the mucosa of the mouth) with the lesions healing in a few days to a month, but recurring. Similar genital lesions recur less frequently.[healthcentral.com]
  • stomatitis, then painful erythematous nodules on lower extremities ( Dermatol Online J 2010;16:18 ) Clinical images Images hosted on other servers: Erythematous papules and pustules resembling acne Erythematous plantar maculae Microscopic (histologic[pathologyoutlines.com]
  • The earliest symptom of Behçet’s syndrome is usually painful canker sores on the mucous membranes that line the mouth (aphthous stomatitis).[rarediseases.org]
Sore Mouth
  • Mouth soresMouth sores (known as oral aphthosis and aphthous stomatitis) affect almost all patients with Beh et's disease. They are often the first symptom that a person notices and may occur long before any other symptoms appear.[healthieryou.com]
Vascular Disease
  • ) disease NOS systemic autoimmune disease systemic collagen (vascular) disease Type 1 Excludes autoimmune disease, single organ or single cell-type -code to relevant condition category Systemic connective tissue disorders M35 ICD-10-CM Diagnosis Code[icd10data.com]
  • Men tend to have a poorer prognosis. [ 7 ] 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.[patient.info]
  • Vascular disease was the main cause of death, (43.9 % of deaths), followed by malignancy (14.6 %), CNS involvement, (12.2 %) and sepsis (12.2 %).[ped-rheum.biomedcentral.com]
  • Retinal Vascular Disease. Berlin: Springer, 2007. Chapter 25.3, p 635-645. Davatchi F, Chams-Davatchi C, Shams H, et al. Adult Behçet's disease in Iran: analysis of 6075 patients. Int J Rheum Dis 2016; 19:95–103.[webeye.ophth.uiowa.edu]
  • Behcet’s disease (BD) is a systemic vascular disease that can affects a variety of organ systems.[eyewiki.aao.org]
Blurred Vision
  • It can present with blurred vision, floaters, eye pain, redness and sensitivity to light. In addition to Behcet's Disease, there are many infectious and non-infectious causes which may result in posterior uveitis.[behcets.com]
  • Inflammation inside of the eye (uveitis, retinitis, and iritis) occurs in more that half of those with Behçet’s disease and can cause blurred vision, pain, and redness.[pvfla.org]
  • vision T: Dark red C: Dry or prickly P: Deep, thin and rapid or Floating, large and rapid Treatment Principle Clear Heat Relieve toxicity Cool the Blood Drain Fire Herb Formulas Points[americandragon.com]
  • Symptoms of eye inflammation include pain , blurred vision , tearing, redness, and pain when looking at bright lights.[medicinenet.com]
  • The retina may become inflamed resulting in blurred vision, abnormal sensitivity to light (photophobia), and/or, inflammation of the thin membranous layer of blood vessels behind the retina (chorioretinitis).[rarediseases.org]
Photophobia
  • The retina may become inflamed resulting in blurred vision, abnormal sensitivity to light (photophobia), and/or, inflammation of the thin membranous layer of blood vessels behind the retina (chorioretinitis).[rarediseases.org]
  • Patients with ocular manifestations may present with blurred vision, photophobia, increased lacrimation and periorbital pain. Ocular symptoms are found to be severe in Iranian and Japanese population. Posterior uveitis may lead to blindness.[symptoma.com]
  • Clinical Manifestations During the mid to later stages of the disease or due to prolonged steroid use, there oral and genital sores that do not heal The sores are dark red and insidiously painful Dry, rough eyes Dark orbits of the eyes Blurred vision Photophobia[americandragon.com]
  • Uveitis can result in blurry vision and an extreme sensitivity to light (photophobia). Rarely, inflammation can also cause eye pain and redness. If untreated, the eye problems associated with Behçet disease can lead to blindness.[ghr.nlm.nih.gov]
  • Other ocular manifestations include hypopyon, retinal vasculitis, retinal haemorrhage, blurred vision and photophobia. Neurological: central nervous system (CNS) involvement occurs late in the disease.[patient.info]
Eye Pain
  • The most common symptoms are Sores in the mouth Sores on the sex organs Other skin sores Swelling of parts of the eye Pain, swelling and stiffness of the joints More serious problems can include meningitis, blood clots, inflammation of the digestive system[medlineplus.gov]
  • This inflammation most commonly affects small blood vessels in the mouth, genitals, skin, and eyes. Painful mouth sores called aphthous ulcers are usually the first sign of Behçet disease .[ghr.nlm.nih.gov]
  • It can present with blurred vision, floaters, eye pain, redness and sensitivity to light. In addition to Behcet's Disease, there are many infectious and non-infectious causes which may result in posterior uveitis.[behcets.com]
Retinal Hemorrhage
  • Frosted branch angiitis is characterized by vascular inflammation, sheathing, retinal edema, and retinal hemorrhages. The disease may be idiopathic in a majority of the cases or may be associated with ocular and systemic pathology.[ncbi.nlm.nih.gov]
  • Other fundoscopic findings include vascular sheathing (23.7%), [7] retinal hemorrhage (9%), [7] macular edema (11.3%), [7] branch retinal vein occlusion (5.8%), [7] and retinal edema (6.6%). [7] However, optic atrophy was the most significant cause of[en.wikipedia.org]
  • Retinal vasculitis manifests as retinal hemorrhages and retinal capillary hypoperfusion on fluorescein angiogram. Optic nerve atrophy is the most common cause of visual impairment.[eyewiki.aao.org]
Red Eye
  • She specifically denies genital ulcers or genital scars, fever, chills, night sweats, alopecia, photosensitivity, Raynaud's phenomena, red eyes, nasal ulcers, cough, dyspnea, hemoptysis, pleuritic chest pain, difficulty swallowing, nausea, vomiting, dysuria[webeye.ophth.uiowa.edu]
Skin Lesion
  • Behcet disease (BD) is a chronic systemic inflammatory disorder characterized by recurrent oral and genital ulcerations, üveitis, and skin lesions. Vascular system involvement is common in BD.[ncbi.nlm.nih.gov]
  • Behçet's disease is a chronic inflammatory disease of unknown aetiology, characterized by recurrent oral and genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis.[ncbi.nlm.nih.gov]
  • Behçet disease is a multisystemic chronic inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions.[ncbi.nlm.nih.gov]
  • lesions, etc, but the coexistence of these 2 diseases are uncommon.[ncbi.nlm.nih.gov]
  • Behçet disease (BD) is a recurrent vasculitis characterized by oral and genital mucous membrane ulcers, uveitis, and skin lesions but only rarely leg ulcers.[ncbi.nlm.nih.gov]
Papule
  • […] aneurysms or severe neurological complications Case reports 34 year old man with aphthous stomatitis, then painful erythematous nodules on lower extremities ( Dermatol Online J 2010;16:18 ) Clinical images Images hosted on other servers: Erythematous papules[pathologyoutlines.com]
  • Pathergy test: minor skin trauma induces an inflammatory papule or pustule after 24-48 hours. This is positive in up to 60% of patients. Imaging studies, including CT and/or MRI scan, may be undertaken in some patients.[patient.info]
  • Systemic manifestations Mucous membranes Recurrent aphthous ulcers, the primary finding in BD, start as painful papules in the oral cavity that rapidly become ulcerated with a pseudomembrane [9,12,13]. They are often numerous and occur frequently.[webeye.ophth.uiowa.edu]
  • […] following 4 " hallmark " symptoms: eye inflammation ( iritis , uveitis , retinal vasculitis, cells in the vitreous) genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men) pathergy reaction (papule[en.wikipedia.org]
  • The test positivity is defined as the development of a papule or pustule at the needle-prick site at 48 h. It is more strongly positive among males.[behcets.info]
Skin Ulcer
  • […] inflammation with venous and arterial vasculitis (possibly obliterative) Positive pathergy test Aphthous ulcers Genital ulcers Erythema nodosum Dermatographia Symptoms of Behcet's Photophobia Vision loss Oral ulcers Genital ulcers Arthritis Headache Skin[webeye.ophth.uiowa.edu]
Arthritis
  • The spondyloarthropathies comprise ankylosing spondylitis (AS), reactive arthritis, psoriatic arthritis (PsA) and arthritis associated with inflammatory bowel disease.[ncbi.nlm.nih.gov]
  • Golimumab is a novel fully human anti-TNF-α monoclonal antibody that has been approved for the treatment of rheumatoid arthritis, psoriatic arthritis, and ankylosing spondylitis, with very promising results.[ncbi.nlm.nih.gov]
  • Arthritis: Literally means joint inflammation. It is a general term for more than 100 conditions known as rheumatic diseases. Arthritis causes joint swelling, pain, and stiffness.[healthieryou.com]
  • ( A39.84 ) mumps arthritis ( B26.85 ) rubella arthritis ( B06.82 ) syphilis arthritis (late) ( A52.77 ) rheumatic fever ( I00 ) tabetic arthropathy [Charcôt's] ( A52.16 ) Type 2 Excludes: M45 ICD-10-CM Diagnosis Code M45 Ankylosing spondylitis 2016 2017[icd10data.com]
  • Arthritis Rheum 62:2796–2805 PubMed CrossRef Google Scholar 26.[link.springer.com]
Arthralgia
  • Behçet disease (intestinal BD) and inflammatory bowel disease (IBD) share a lot of characteristics, including genetic background, clinical manifestations, and therapeutic strategies, especially the extraintestinal manifestations, such as oral ulcers, arthralgia[ncbi.nlm.nih.gov]
  • HA20 looks like BD if we consider recurrent oral (87%) and genital (67%) ulcers, arthralgia or arthritis (42%), skin involvement (53%) such as erythema nodosum or abdominal symptoms (60%) such as abdominal pain, digestive ulcers or diarrhea.[ncbi.nlm.nih.gov]
  • Symptoms · Skin irritation · Hereditary · Arthritis/Arthralgia · Bowels – GI manifestations include abdominal pain, nausea, and diarrhea with or without blood, and they often involve the ileocecal valve.[powerofpain.org]
  • […] haemorrhage, and focal atelectasis gastrointestinal manifestations of Behçet disease : affects 5-60% of cases CNS manifestations of Behçet disease : affects 10-50% of cases musculoskeletal manifestations of Behçet disease : 50% develop articular disorders/arthralgia[radiopaedia.org]
  • Arthralgia and/or arthritis are frequent (45%) and can occur as an initial symptom.[orpha.net]
Joint Swelling
  • swelling and pain … has struggled with what was diagnosed as Behcet's disease , a form of vasculitis, which causes lesions, mouth blisters and joint pain. — Brad Townsend , The Dallas Morning News , 14 May 2012[merriam-webster.com]
  • Some symptoms may include mouth and genital ulcers, eye inflammation and reduced vision, skin rashes and lesions, joint swelling, abdominal pain and diarrhea.[sciencedaily.com]
  • Genital sores Eye disease: Redness Blurry vision Light sensitivity Watery eyes Joint swelling and pain—usually does not cause permanent joint damage Skin problems such as sores that: May appear as red, raised sores or bumps, or may be flat Usually appear[cancercarewny.com]
  • swelling Brain and spinal cord: Fever Headache Stiff neck Confusion Personality changes Memory loss Trouble speaking Digestive system : Stomach pain Diarrhea Bleeding from sores in the stomach and intestines Sometimes Behcet's also affects other organs[webmd.com]
  • Joint swelling and pain often affect the knees in people with Behcet's disease. The ankles, elbows or wrists also may be involved. Signs and symptoms may last one to three weeks and go away on their own. Vascular system.[mayoclinic.org]
Dysuria
  • She specifically denies genital ulcers or genital scars, fever, chills, night sweats, alopecia, photosensitivity, Raynaud's phenomena, red eyes, nasal ulcers, cough, dyspnea, hemoptysis, pleuritic chest pain, difficulty swallowing, nausea, vomiting, dysuria[webeye.ophth.uiowa.edu]
Confusion
  • This can result in confusion and coma . Typically these features occur later in the disease course, years after the diagnosis.[medicinenet.com]
  • Genitals: Sores on the scrotum in men Sores on the vulva in women Skin : Bumps that look like acne Red lumps on the legs and ankles Joints: Pain in the knees, ankles, wrists, and elbows Joint swelling Brain and spinal cord: Fever Headache Stiff neck Confusion[webmd.com]
  • . · Nervous System Symptoms · Neurological involvements range from aseptic meningitis to vascular thrombosis such as dural sinus thrombosis and organic brain syndrome manifesting with confusion, seizures, and memory loss · Neurological system – CNS involvement[powerofpain.org]
  • The neurologic features are variable and include headache, confusion, strokes, personality changes and memory loss. Central nervous system symptoms in Behcet's Disease may be due to aseptic meningitis or lesions resulting in focal brain dysfunction.[behcets.com]
  • I have folliculitis on my thighs and back that never goes away, central nervous system/neurological (brain) problems with inflammation, partial seizures, headaches, neck stiffness of varying degrees most of every single day, confusion, coordination issues[globalgenes.org]

Workup

Imaging techniques like MRI, CT scan, and radiography are useful in identifying tissue swelling, effusions and areas of ischemia. Brain MRI is necessary when there is an involvement of CNS. MRI helps in locating focal lesions of cerebral vasculopathy. Aneurysms can be detected using angiograms. Echocardiography is an imaging method useful in diagnosing ventricular thrombi.

There are no specific laboratory tests for the diagnosis of Behcet’s syndrome. Patients often have normal levels of serum components. Some of the complements may show a change when ocular or skin manifestations arise. Few patients may show increased ESR and C-reactive protein levels. Anticardiolipin antibodies are reported in some patients.

Gastrointestinal lesions are diagnosed using endoscopy. Eye examination is essential when ocular manifestations are obvious. Retinal vessels are evaluated using fluorescein angiography. Positive pathergy test is indicative of Behcet’s disese as skin prick may trigger nodule or pustule formation within 2 days. Perivascular infiltration and vasculitis can be identified using sample biopsy.

Colitis
  • Behçet disease with gastrointestinal involvement in ulcerative colitis (UC) patient has been reported in just 1 previous case report, but, which can not be diagnosed as definite intestinal BD based on Korean novel diagnositic criteria due to lacking the[ncbi.nlm.nih.gov]
  • Symptoms vary from mild gastrointestinal discomfort to ulcerative colitis or regional enteritis and malabsorption problems. Arthritis occurs in about two-thirds of patients, most commonly affecting the knees and ankles.[healthcentral.com]
  • Ulcerative colitis: Inflammation of the colon. Symptoms include stomach pain and diarrhea.[healthieryou.com]
  • Because these symptoms are very similar to symptoms of other diseases of the digestive tract, such as ulcerative colitis and Crohn's Disease, careful evaluation is essential to rule out these other diseases.[eyewiki.aao.org]
  • Rectal dialysate and fecal concentrations of neutrophil gelatinase-associated lipocalin, interleukin-8, and tumor necrosis factor-alpha in ulcerative colitis.[journals.plos.org]
Colonic Ulcer
  • A 61-year-old man developed sudden hematochezia due to bleeding from multiple small colonic ulcers; AL-amyloid deposition was found on immunohistochemical examination of biopsy specimen of colonic ulcer.[ncbi.nlm.nih.gov]
HLA-B5
  • Presence of HLA-B51/HLA-B5 genetic structure is associated with increased risk of this syndrome. But these genes are not shown to have any effect on the severity of the disease. No known preventive measures exist for Behcet’s syndrome.[symptoma.com]
  • Patients positive for HLA-B51 or HLA-B5 are more likely to be male and also have higher prevalences of genital ulcers, ocular manifestations and skin manifestations, and a decreased prevalence of gastrointestinal involvement. [ 5 ] Management [ 6 ] The[patient.info]
  • […] into hyperactivity by environmental stimuli at a lower threshold than in individuals who do not have Behçet disease. [ 14 , 15, 16 , 17] Genetics Behçet disease is a sporadic disease, but a familial aggregation is well known. [ 18 ] Carriers of HLA-B51/HLA-B5[emedicine.medscape.com]
  • Carriers of HLA-B51/HLA-B5 are at increased risk of developing BD compared with non-carriers.[ncbi.nlm.nih.gov]
HLA-Bw51
  • "Close association of HLA-Bw51 with Behçet's disease". Arch. Ophthalmol . 100 (9): 1455–8. doi : 10.1001/archopht.1982.01030040433013 . PMID 6956266 . Durrani K, Papaliodis GN (2008). "The genetics of Adamantiades–Behcet's disease".[en.wikipedia.org]
  • Close association of HLA-Bw51 with Behçet's disease. Arch Ophthalmol. 1982; 100: 1455–8. pmid:6956266 View Article PubMed/NCBI Google Scholar 3. Mizuki N, Meguro A, Ota M, Ohno S, Shiota T, Kawagoe T, et al.[journals.plos.org]
  • Close association of HLA-BW51 with Behçet's disease. Arch Opthalmol 1982 ; 100 : 1455 -1458 Mizuki N, Inoko H, Ohno S. Molecular genetics (HLA) of Behçet's disease. Yonsei Med J 1997 ; 38 : 423 -427 McMenemey WH, Lawrence BJ.[ajnr.org]

Treatment

Treatment modality depends on the organ involved and severity of the symptoms. Topical steroids and sucralfate solution are recommended in mild cases of oral and genital ulcerations. Colchicine is used to prevent relapse of mucocutaneous lesions [12]. Severe lesions are treated with systemic corticosteroids, azathioprine, dapsone and interferon-alpha. Mild ocular manifestations are treated with azathioprine. In severe cases of ocular symptoms, cyclosporine A or infliximab is used in combination with corticosteroids. Interferon-alpha is also recommended in severe cases of ocular manifestations. Systemic corticosteroids and azathioprine are suggested for gastrointestinal ulcerations. For cutaneous manifestations, colchicine and dapsone are used. Thrombi in vessels are treated with systemic anticoagulants with corticosteroids or azathioprine. Aneurysms in pulmonary vessels need cyclophosphamide with corticosteroids.

Those presenting with intestinal stenosis, severe bleeding, perforations and fistula, may need surgery. Resection is suggested for ischemic damage with vasculitis. Surgery is also recommended in coronary thrombosis, endocardial fibrosis or ventricular aneurysms. Some ocular manifestations like glaucoma, cataract and retinal detachment may require surgical intervention. Clots and aneurysms in the CNS are also improved with surgery.

Prognosis

In general, prognosis depend on the clinical manifestations of the disease. Treatment, rest and exercise are the best steps to improve the symptoms of the condition. Effective treatment helps to prevent exacerbations. Patients may enter a period of remission in most of the cases, while serious complications may arise in some. Those who develop retinal vasculitis, vascular aneurysms, and neurological manifestations may not have a good prognosis. Retinal vasculitis may lead to blindness while aneurysms in the blood vessels may rupture.

Etiology

Etiology of the disease is not defined. Viral etiology, though accepted earlier, was disproved over the years. New theories propose various other factors as the causative feature including bacterial infection, exposure to chemicals and, most recent, autoimmune reaction. Clinical manifestations are caused by immune responses that lead to vasculitis of small and medium sized blood vessels. The reaction is also thought to initiate inflammation of epithelium mediated by hyperactive T lymphocytes and plasma cells. Increased function of neutrophils noted in patients is also implicated in the development of clinical manifestations.

A genetic component, particularly HLA-B51, has an association with this syndrome. Interleukin 10 (IL-10) and IL-23 are also reported to have strong influences in the development of Behcet’s disease [2]. Patients with active form of Becet’s syndrome have elevated levels of IL-6, while IL-10 remains lower than normal [3].

Epidemiology

This syndrome exists worldwide, but with distinct differences in the regions. Worldwide prevalence studies show the estimate to be 1:10,000. Behcet’s disease is a more prevalent condition in the Far East, Middle East and Mediterranean countries like Iran, Turkey and Israel. In Turkey the estimate is in the range of 80-370 in a population of 100,000. Western countries show a prevalence ranging from 5 to 6.4 per 100,000 population [4]. Incidence was found to be higher among males in the Mediterranean region, and this is particularly true for men in the age group of 30-40 years. In Eastern countries the male to female ratio was found to be 1:1, while in Japan and Korea females are more commonly affected. Although there is a variation in the sex ratios from different regions, the course of the disease is more severe among males. Average onset age (25-35 years) was similar in men and women, some cases have been reported in childhood as well. It is rare for the disease to occur before school age. In Turkey the mean age for the onset of Behcet’s disease is 11.7 years.

Sex distribution
Age distribution

Pathophysiology

A number of theories have been put forward to explain pathogenesis of this disease. The latest and most accepted one is the autoimmune pathogenesis of Behcet’s syndrome. Infectious agents like Streptococcus, Staphylococcus species, and herpes simplex virus (HSV) may trigger an immune reaction. Heat shock proteins (HSP) are also suspected as triggers of cross-reactive immune reaction that lead to Behcet’s disease. In high-risk populations, including Turkey and Japan, HSPs bring out an enhanced activation of lymphocytes resulting in a proliferative response, characteristic of the disease [5]. Different ethnic populations show increased T- and B-cell responses to these proteins and hence HSPs may be pathophysiologically related to the development of Behcet’s syndrome.

T-cells and neutrophils undergo inflammatory tissue infiltration resulting in vasculitic and vasculopathic lesions that are common manifestations in the disease [6]. The specific roles of the neutrophils in the disease is not fully characterized. Chemotaxis, active oxygen production, phagocytosis, and infiltration are all implicated in the course of this syndrome [7]. Many recent evidences suggest a critical role for nitric oxide, a free oxygen radical released by endothelial cells upon stimulation by cytokines, in the development and progression of Behcet’s disease. Increased production of nitrogen oxide is associated with increased susceptibility to the syndrome [8].

Behcet’s disease may be seen in a family, which suggests genetic pathophysiology of the disease. Presence of HLA-B51/HLA-B5 genetic structure is associated with increased risk of this syndrome [9]. But these genes are not shown to have any effect on the severity of the disease.

Prevention

No known preventive measures exist for Behcet’s syndrome.

Summary

Behcet’s syndrome or Behcet’s disease is a multisystemic inflammatory disease first described by Turkish dermatologist Hulusi Behçet in 1937. It was initially described as a ‘triple symptoms complex’ with characteristic symptoms – aphthous ulceration, genital ulceration and inflammatory eye disease [1]. The condition may cause heterogeneous clinical manifestations including ocular, mucocutaneous, neurologic, cardiovascular, pulmonary, renal and gastrointestinal symptoms. Symptoms often cluster together, but the manifestations may vary from region to region.

The precise etiology of the disease is not yet known. Highest incidence of Behcet’s syndrome is reported from the Middle East, Mediterranean and the Far East. It is a rare syndrome and young adult men are more commonly affected by the condition. Family aggregation of the syndrome, affecting three brothers in a family, is also reported. A number of etiologic factors are thought to be associated with the development of Behcet’s syndrome, the latest and most accepted one being autoimmune reaction. Treatment is based on clinical manifestations of the condition.

Patient Information

Behcet’s syndrome or Behcet’s disease is an autoimmune disease characterized by triad of symptoms – oral ulcers, sores in the genital area and inflammation in parts of the eye. Many tissues and blood vessels may become inflamed, resulting in the different symptoms of the disease. Symptoms may vary from person to person depending on the location of inflammation and the organ involved. Some of the areas that are commonly affected by the disease include mouth, skin, genitals, eyes, blood vessels, digestive system, brain, and joints.

The actual cause of the disease is not fully characterized. A combination of environmental and genetic factors are thought to play an important role in the development of this condition. This syndrome is more common among men and women in the age group of 30 to 40 years. People from Far East, Mediterranean, and Middle East have an increased risk of developing this syndrome. Certain genes are also known to be associated with the disease. Behcet’s syndrome is seen in both men and women, but is more severe in men.
Confirmatory diagnosis depends on the presence of any of the two symptoms given below:

  • Mouth sores
  • Recurrent genital sores
  • Eye problems 
  • Skin sores
  • Positive pathergy test – In this test, a sterile needle is inserted into the skin and if small pustules are formed within 2 days, it is considered to be a positive test for increased immune activity. 

Complete cure for the disease is not available. Treatment often depends on the severity of the symptoms and the organ involved. Topical creams are used for sores in the skin and genital area. Eye drops containing corticosteroids are useful in improving the symptoms of the eye. When topical medications are not effective, oral medications are recommended. Medications are used to suppress the immune system and also to control inflammation. In some cases medications are suggested to regulate the response of the immune system. Prompt treatment, adequate rest, good diet and exercise go a long way in controlling the symptoms of Behcet’s syndrome.

References

Article

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  2. Ozen S, Eroglu FK. Pediatric-onset Behçet disease. Curr Opin Rheumatol. 2013;25(5):636-42.
  3. Talaat RM, Ashour ME, Bassyouni IH, Raouf AA. Polymorphisms of interleukin 6 and interleukin 10 in Egyptian people with Behcet's disease. Immunobiology. 2014; 219(8):573-582.
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  5. Lehner,T. The role of heat shock protein, microbial and autoimmune agents in the aetiology of Behcet's disease. Int Rev Immunol, 1997;14:(1):21–32.
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  7. Mege JL, Dilsen N, Sanguedolce V et al., Overproduction of monocyte derived tumor necrosis factor α, interleukin (IL) 6, IL-8 and increased neutrophil superoxide generation in Behcet's disease. A comparative study with familial Mediterranean fever and healthy subjects. J Rheumatol. 1993;20(9):1544–1549.
  8. Örem A, Vanizor B, Çimşit G, Kiran E, Deǧer O, and Malkoç M. Decreased nitric oxide production in patients with Behcet's disease. Dermatology. 1999;198(1):33–36.
  9. de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A. HLA-B51/B5 and the risk of Behçet's disease: a systematic review and meta-analysis of case-control genetic association studies.Arthritis Rheum. 2009;61(10):1287-96. 
  10. Alpsoy E, Zouboulis CC, Ehrlich GE. Mucocutaneous lesions of Behcet's disease. Yonsei Med J. 2007;48(4):573-85.
  11. Hamzaoui K, Berraies A, Kaabachi W, Ammar J, Hamzaoui A. Pulmonary manifestations in Behcet disease: impaired natural killer cells activity. Multidiscip Respir Med. 2013;8(1):29.
  12. Calis M, Ates F, Yazici C, Kose K, Kirnap M, Demir M, et al. Adenosine deaminase enzyme levels, their relation with disease activity, and the effect of colchicine on adenosine deaminase levels in patients with Behçet's disease. Rheumatol Int. 2005;25(6):452-6.

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Last updated: 2018-06-22 10:09