Behcet Disease: Difference between revisions

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== Definition/Description  ==
== Definition/Description  ==


A syndrome (or sometimes referred to as a disease) that causes inflammation in the blood vessels of the body. The most common symptoms are sores in the mouth, sex organs, and on the skin, as well as swelling in the eye, pain, stiffness, and swelling in the joints. The more serious complications include meningitis, blood clots, inflammation of the GI tract, and blindness.&nbsp;<ref name="1.">National Institute of Arthritis and Musculoskeletal and Skin Diseases. Behcet's disease. http://www.niams.nih.gov (accessed 22 February 2011).</ref><ref name="2.">Medline Plus. Behcet's syndrome. http://www.nlm.nih.gov (accessed 22 February 2011).</ref>&nbsp;
"Behcet's Disease" (BD) is a chronic disease characterized by inflammation in the blood vessels and relapsing and remitting course. <ref name=":0">Alpsoy E, Bozca BC, Bilgic A. Behçet disease: an update for dermatologists. American Journal of Clinical Dermatology. 2021 Jul;22(4):477-502.</ref> It can also be referred as a "Behcet Syndrome" since it involves different body systems such as skin, mucosa, joints, nervous and gastrointestinal systems. <ref>Bettiol A, Prisco D, Emmi G. [https://academic.oup.com/rheumatology/article/59/Supplement_3/iii101/5826909 Behçet: the syndrome]. Rheumatology. 2020 May 1;59(Supplement_3):iii101-7.</ref>
 
The video below is a great introduction to the condition provided by the American Behcet's Disease Association (ABDA), featuring educational information and patients' stories.


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== Prevalence  ==
== Prevalence  ==
[[Image:Silkroad.jpg|frame|Route of Silk Road]]


Occurs in people among the " Silk Road"- in the eastern Mediterranean, Middle East,and East Asia. Most common in males along the Silk Road countries and more common in females in Europe and the U.S. aged 25-30 years.&nbsp;&nbsp;<ref name="3.">Arthritis Foundation. Behcet's disease: how is it diagnosed? http://www.arthritis.org (accessed February 22 2011).</ref> [[Image:Silkroad.jpg|frame|Route of Silk Road]]
The prevalence is variable worldwide <ref name=":1" /> but higher in the countries along the Silk Road. <ref name=":0" /><ref name=":1" /> A recent study calculated the prevalence of BD as 10.3 per 100,000 population globally and 119.8 for Turkey, 31.8 for the Middle East, 4.5 for Asia, 5.3 for Southern Europe, 2.1 for Northern Europe, and 3.8 for North America/Caribbean Islands. <ref>Maldini C, Druce K, Basu N, LaValley MP, Mahr A. [https://academic.oup.com/rheumatology/article/57/1/185/3002782 Exploring the variability in Behçet’s disease prevalence: a meta-analytical approach]. Rheumatology. 2018 Jan 1;57(1):185-95.</ref>
 
*Estimated 15,000 in U.S. with Behcet's.
 
== Characteristics/Clinical Presentation  ==
 
The patient could present with any of the five most common symptoms of Behcet's disease.  
 
*Mouth sores- usually the first and most persistent symptom. &nbsp;They are similar to canker sores but have a longer duration and are usually larger and more painful.  
*Genital sores- &nbsp;less common, but still occur in more than half of the patients with this disease. &nbsp;They are usually mistaken as genital herpes and can be on the external or internal surface of the genitalia. &nbsp;
*Skin lesions- are red, raised bumps or bruises that resemble boils or acne. &nbsp;They usually appear on the legs and upper torso.&nbsp;
*Uvelitis and retinal vasculitis- occuring in half the Behcet's patients, this can cause blurred vision, pain, redness, or blindness if left untreated.&nbsp;
*Arthritis- it results in inflammation in the joints leading to edema, heat, redness, and pain. It occurs in half the Behcet's patients and lasts a few weeks.


In addition to these five common clinical symptoms that patients present with, the circulatory, central nervous systems, and digestive tract can be affected.&nbsp;


*Circulatory system- DVT, aneurysms
*Central nervous system- may experience fever, headache, stiff neck, and difficulty coordinating movements secondary to inflammation in the meninges in the brain and spinal cord.&nbsp;
*Digestive tract- may lead to abdominal pain, diarrhea, and bleeding due to inflammation and ulcers in GI tract. These symptoms are similar to ulcerative colitis and Crohn's disease.&nbsp;<ref name="3." />


== Associated Co-morbidities  ==


Include: ulcerations in mouth, genitals, and GI tract, skin lesions, lung aneurysms, arthralgias, headaches, confusion, strokes, personality changes, dementia, and aseptic meningitis, anterior/posterior uveitis&nbsp;<ref name="4.">The John Hopkins Vasculitis Center. Hellmann D. Behcet's disease. www.hopkinsvasculitis.org (accessed 22 February 2011).</ref> [[Image:Img0019.jpg|frame|Mouth Ulcers]]
== Pathological Process ==
The etiology is unknown <ref name=":0" /> <ref name=":2">Rodríguez-Carrio J, Nucera V, Masala IF, Atzeni F. Behçet disease: from pathogenesis to novel therapeutic options. Pharmacological Research. 2021 May 1;167:105593.</ref> and extensive debate existed on whether to classify it as autoimmune or autoinflammatory but recently classified as the major histocompatibility complex class I (MHC-I) opathy <ref name=":2" />. Genetic factors are thought to play a crucial role since strong association with HLA-B51 antigen is proved <ref>Ohno S, Ohguchi M, Hirose S, Matsuda H, Wakisaka A, Aizawa M. Close association of HLA-Bw51 with Behçet's disease. Archives of ophthalmology. 1982 Sep 1;100(9):1455-8.</ref><ref>Ohno S, Aoki K, Sugiura S, Nakayama E, Itakura K, Aizawa M. HL-A5 and Behcet's disease. The Lancet. 1973 Dec 15;302(7842):1383-4.</ref><ref name=":0" /><ref name=":1" />  as well as higher prevalence in specific ethnic populations, and high sibling recurrence rate. <ref name=":1" />
== Clinical Presentation  ==
[[File:Oral ulcer in Behcet Syndrome.jpg|thumb|Oral ulcer in Behcet Syndrome]]
Vasculitis in BD can involve any kind and size of vessels, and this explains why the disease can have an effect on multiple systems in the body. <ref name=":3">Akdeniz N, Elmas ÖF, Karadağ AS. Behçet syndrome: A great imitator. Clinics in Dermatology. 2019 May 1;37(3):227-39.</ref> So, the patient could present with any of the symptom(s) below:
* '''Mucocutaneous involvement''': Oral aphthosis, genital aphthosis, papulopustular lesions, pathergy phenomenon, erythema-nodosu-like lesions, superficial thrombophlebitis, pyodermagangrenosum-like lesions, erythemamultiforme-like lesions, nailfold capillary abnormalities. <ref name=":3" />
* '''Ocular involvement''': Uveitis, vitritis, retinal infiltrates, sheathing of retinal veins, occlusive vasculitis, neovascularization, secondary cataracts, glaucoma, frosted branch angiitis and macular oedema, conjunctivitis, and conjunctival aphthosis, and scleritis being less common. <ref name=":3" /> Ocular involvement is the most common cause of morbidity in people with BD. <ref>Davatchi F, Chams-Davatchi C, Shams H, Shahram F, Nadji A, Akhlaghi M, Faezi T, Ghodsi Z, Sadeghi Abdollahi B, Ashofteh F, Mohtasham N. Behcet’s disease: epidemiology, clinical manifestations, and diagnosis. Expert review of clinical immunology. 2017 Jan 2;13(1):57-65.</ref>
* '''Musculoskeletal involvement''': [[Arthritis]], arthralgia, enthesopathy, [[Avascular Necrosis|avascular necrosis]], myalgia, and [[Inflammatory Myopathies|myositis]]. <ref name=":3" />
* '''Vascular involvement''': Pulmonary, carotid, aortic, iliac, femoral, and popliteal arterial involvement; pulmonary aneurysm; superficial and [[Deep Vein Thrombosis|deep vein thrombosis]]; Budd-Chiari syndrome; superior and inferior [[Vena Cava|vena cava]] occlusion; and dural sinus [[thrombosis]]. <ref name=":3" />
* '''Cardiac involvement''': Pericarditis, myocarditis, endocarditis with valvular regurgitation, coronary arteritis, [[Coronary Artery|coronary artery]] aneurysms, diastolic dysfunction, atrial septal aneurysm, conduction system disturbances, atrial and ventricular arrhythmias, mitral valve prolapse, intracardiac thrombosis, valvular insufficiency, and [[Myocardial Infarction|myocardial infarction]]. <ref name=":3" />[[File:Neurologic involvement in Behcet's Disease.jpeg|thumb|Sigmoid sinus thrombosis presenting with posterior alexia in a patient with Behcet's disease and polycythaemia: a case report.]]
* '''Gastrointestinal (GI) involvement''': The most common symptoms are abdominal pain, nausea, vomiting, diarrhoea, and GI bleeding. The GI manifestations of BD are known to cause significant morbidity and mortality. <ref>Nguyen A, Upadhyay S, Javaid MA, Qureshi AM, Haseeb S, Javed N, Cormier C, Farooq A, Sheikh AB. [https://karger.com/iid/article/6/4/175/824174/Behcet-s-Disease-An-In-Depth-Review-about Behcet’s disease: an in-depth review about pathogenesis, gastrointestinal manifestations, and management.] Inflammatory Intestinal Diseases. 2021 Nov 4;6(4):175-85.</ref> <ref>Skef W, Hamilton MJ, Arayssi T. Gastrointestinal Behçet's disease: a review. World Journal of Gastroenterology: WJG. 2015 Apr 4;21(13):3801.


== Medications  ==
</ref>
* '''Neurologic involvement''': The manifestations include focal or multifocal parenchymal involvement, isolated cerebral venous sinus thrombosis, intracranial hypertension, isolated behavioural syndromes, peripheral nervous system involvement, and secondary neurologic involvement. <ref name=":3" /> The combination of neurologic symptoms and/or signs in patients diagnosed with BD is called Neuro-Behcet's disease (NBD). <ref>Borhani-Haghighi A, Kardeh B, Banerjee S, Yadollahikhales G, Safari A, Sahraian MA, Shapiro L. Neuro-Behcet's disease: an update on diagnosis, differential diagnoses, and treatment. Multiple sclerosis and related disorders. 2020 Apr 1;39:101906.</ref> NBD is a serious risk for mortality and usually occurs in males. <ref>Benamour S, Naji T, Alaoui FZ, El Kabli H, El Aidouni S. Manifestations neurologiques de la maladie de Behçet. Revue Neurologique. 2006 Nov 1;162(11):1084-90.</ref><ref name=":1" />
* '''Pulmonary involvement:''' Pulmonary artery aneurism, pulmonary infection, pulmonary vasculitis, [[Pulmonary Fibrosis|pulmonary fibrosis]], pleuritis, and [[Pulmonary Embolism|pulmonary emboli]]. <ref name=":3" />


For the disease that's confined to the mucocutaneous areas (mouth, genitals, skin) use topical steroids and non immunosuppressive medications.&nbsp;


Moderate dosage of corticosteroids are used for exacerbations. For some patients with chronic pain, low doses of prednisone are indicated.&nbsp;Serious organ involvement calls for high doses of Prednisone and immunosuppressive treatment. Immunosuppressive medications include: azathrioprine, cyclosporine, cyclophosphamide, and chlorambucil&nbsp;<ref name="4." />
== Diagnostic Procedures  ==
[[Image:Neuro.jpg|MRI of central nervous system involvement in BD (white matter changes in the pons)|thumb]]Since there is no specific biomarker for BD, the diagnosis process is based on the clinical picture. <ref name=":1">Akkoç N. Update on the epidemiology, risk factors and disease outcomes of Behçet's disease. [https://www.sciencedirect.com/science/article/abs/pii/S1521694218300470 Best Practice & Research Clinical Rheumatology]. 2018 Apr 1;32(2):261-70.</ref> Although more than fifteen classification criteria have been proposed for BD to date <ref>Davatchi F, Sadeghi Abdollahi B, Chams‐Davatchi C, Shahram F, Shams H, Nadji A, Faezi T, Akhlaghi M, Ghodsi Z, Mohtasham N, Ashofteh F. The saga of diagnostic/classification criteria in Behcet's disease. International journal of rheumatic diseases. 2015 Jul;18(6):594-605.</ref>, the most frequently used one in studies has been the International Study Group (ISG) Criteria with 92% sensitivity and 97% specificity<ref>Wechsler FB, Davatchi F. Criteria for diagnosis of Behcet's disease. The Lancet. 1990;335(8697):1078-80.</ref><ref>International Study Group for Behçet's Disease, Wechsler B, Davatchi F, Mizushima Y, Hamza M, Dilsen N, Kansu E, yazici H, Barnes CG, Chamberlain MA, James DG. Evaluation of diagnostic (‘classification’) criteria in Behçet's disease—towards internationally agreed criteria. Rheumatology. 1992 May 1;31(5):299-308.</ref>, which was set in 1990. Accordingly, the criteria to be met are: 


== Diagnostic Tests/Lab Tests/Lab Values  ==
* Oral ulcerations at least three times in one year.


Diagnosis is very difficult and may takes months or even years to get an accurate diagnosis. Key symptoms are: [[Image:Behcet's_D.JPG|frame|right|Skin lesion on Scrotum]]
* Plus any two of the following:
* Recurring genital ulceration
* Eye lesions
* Skin lesions
* Positive pathergy test <ref>Wechsler FB, Davatchi F. Criteria for diagnosis of Behcet's disease. The Lancet. 1990;335(8697):1078-80.</ref>


*Mouth sores at least three times in one year.  
== Outcome Measures ==
*Any two of the following:  
A recent study <ref name=":1" /> summarised the outcome measures based on a systematic review by The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group <ref>Hatemi G, Merkel PA, Hamuryudan V, Boers M, Direskeneli H, Aydin SZ, Yazici H. [https://www.jrheum.org/content/41/3/599 Outcome measures used in clinical trials for Behçet syndrome: a systematic review]. The Journal of rheumatology. 2014 Mar 1;41(3):599-612.
*Recurring genital sores
*Eye inflammation with loss of vision
*Characteristic skin lesions
*Positive pathergy test


<br>
</ref>:


A dermatologist may do a biopsy of the mouth, genitals, and skin lesions.&nbsp;<ref name="3." />&nbsp;
* Behçet's Disease Current Activity Form (BDCAF)
== Etiology/Causes  ==
* Clinical Disease Activity Index
* Clinical Manifestations Index
* Iranian BD Dynamic Activity Measure,
* 1994 Criteria for Disease Activity of BD
* Krause's Total Severity Score
* BD Quality of Life
* Oral Ulcer Composite Index


The etiology is unknown but thought to be secondary to an autoimmune reaction, where the immune system attacks and harms the body's tissues, resulting in inflammation. Some thoughts are that it could have a genetic component making some more susceptible to a possible environmental trigger such as a virus or bacterium.&nbsp;&nbsp;<ref name="3." />
However, it also indicated that:


[[Image:Lungs.jpg|frame|Behcet's affecting the lungs]]  
* Most of the measures used in the studies were not properly validated, and standard definitions were absent for outcome variables like response, relapse, or remission.
* Even though specific outcome measures were developed for BD, they were not widely used in studies. For instance, BDCAF, which is the most commonly used disease activity measure, has been used in only 10% of the studies.
* Internet-based surveys conducted by the OMERACT Vasculitis Working Group among expert physicians from relevant specialities from multiple countries <ref>Hatemi G, Ozguler Y, Direskeneli H, Mahr A, Gul A, Levi V, Aydin SZ, Mumcu G, Sertel-Berk O, Stevens RM, Yazici H. [https://www.jrheum.org/content/42/12/2436 Current status, goals, and research agenda for outcome measures development in Behçet syndrome: report from OMERACT 2014]. The Journal of rheumatology. 2015 Dec 1;42(12):2436-41.


[[Image:GI.jpeg|frame|Ulcers in GI tract]]
</ref> have emphasized the need for widely acceptable and properly validated outcome measures for BD.  


== Systemic Involvement  ==
== Management / Interventions ==


Can affect the eyes, mouth, skin, lungs, joints, brain, genitals, and GI tract.&nbsp;
=== Multidisciplinary Team ===


*Eyes: May cause anterior/ posterior uveitis; anterior uveitis results in pain, blurry vision, tearing, redness, and light sensitivity; posterior uveitis causes fewer symptoms but damages the retina.  
* Treatment and follow-up are ideally coordinated by a paediatrician or adult practice physician with expertise in BD. This is most often an internal medicine specialist, dermatologist, or rheumatologist. The general physician or pediatrician plays a crucial role in optimizing the coordination of the proposed care plan. <ref name=":4">Kone-Paut I, Barete S, Bodaghi B, Deiva K, Desbois AC, Galeotti C, Gaudric J, Kaplanski G, Mahr A, Noel N, Piram M. [https://link.springer.com/article/10.1186/s13023-020-01620-4 French recommendations for the management of Behçet’s disease]. Orphanet journal of rare diseases. 2021 Feb;16:1-28.
*Mouth: Painful sores called aphthous ulcers can be found on the lips, tongue, and inside of the cheek.  
*Skin: Erythema nodosum which are red, tender nodules that can occur on the legs and ankles commonly, but also on the face, neck, and arms or pustular skin lesions which resemble acne.&nbsp;
*Lungs: Aneurysms in arteries of lungs--&gt; which can lead to hemorrhage.&nbsp;
*Joints: Arthralgias
*Brain: Headaches, confusion, strokes, personality changes, dementia, and aseptic meningitis. [[Image:Neuro.jpg|frame|MRI of CNS involvement (white matter changes in the pons)]]
*Genitals: painful lesions that form on the scrotum or vulva.  
*Gastrointestional: Ulcerations that occur from the mouth to the anus. The most common sites are the ileum and cecum.&nbsp;<ref name="4." />


== Medical Management ==
</ref>
* Other specialized physicians such as ophthalmologists, neurologists, vascular physicians, gastroenterologists, and pulmonologists may also be involved in patient care. <ref name=":4" />
* Other health professionals such as those in therapeutic education, dietitians, state-certified nurses, physiotherapists, occupational therapists, psychologists, and social welfare assistants may also be involved upon as necessary. <ref name=":4" />


Steroid gels, pastes, and creams can be used for mouth and genital ulcers. Colchicine helps to reduce recurrent ulcers. Pentoxifylline can be used to maintain the healed ulcers for up to 29 months. Ibuprofen can be used to decrease joint inflammation. Colchicine and cortisone are used for inflammation in patients. Sulfasalazine is used to decrease the symptoms for arthritis. Immediate attention has to be given to treat the eyes and reduce inflammation. Research has indicated that by blocking TNF, a protein that initiates inflammation, physicians can successfully manage inflammation. TNF blocking medications include infliximab and etanercept. Immunosuppressive drugs have been used to treat the more severe symptoms associated with the brain, eyes, and arteries. The immunosuppressive agents include: chlorambucil, azathioprine, and cyclophosphamide. Current literature is looking at the effects of thalidomide for treating and preventing ulcerations in the mouth and genitals.&nbsp;<ref name="5.">MedicineNet.com. Behcet's syndrome. http://www.medicinenet.com (accessed March 15 2011).</ref>
=== Medical Management ===


Research indicates that the prognosis is variable and depending on the severity and where the symptoms occur, some will diminish over time. Usually symptoms of the CNS will not diminish, however.
* In the treatment and secondary prevention of thrombosis in Behcet's Disease, immunosuppressants are currently preferred over anticoagulants. <ref>Bettiol A, Alibaz-Oner F, Direskeneli H, Hatemi G, Saadoun D, Seyahi E, Prisco D, Emmi G. Vascular Behçet syndrome: from pathogenesis to treatment. Nature Reviews Rheumatology. 2023 Feb;19(2):111-26.</ref>
* Recently, Apremilast has been approved for the treatment of oral ulcers in Behcet's Syndrome in the United States. The efficacy of Apremilast in controlling non-oral ulcer features of the syndrome is still under investigation. <ref name=":5">Yazici Y. Management of Behçet syndrome. Current opinion in rheumatology. 2020 Jan 1;32(1):35-40.


== Physical Therapy Management  ==
</ref>
* Tumor necrosis factor inhibitors have proven to be safe and effective for treating eye disease in Behcet's Syndrome with long-term use. <ref name=":5" />
* While new treatment options such as ustekinumab, secukinumab, tocilizumab, and others show promising early data, more studies are needed to better understand their role in Behcet's management. <ref name=":5" />


Energy conservation techniques, low-moderate intensity exercise endurance and strengthening, ROM, education on disease process, relaxation techniques, modalities for pain control
=== Physical Therapy ===
To prevent cortisone myopathy, patients should be advised to engage in regular physical activities, such as fast walking for 30 to 45 minutes each day. If they are diagnosed with amyotrophy, they may even need to undergo kinesiology therapy to strengthen their muscles. Engaging in physical activity is also crucial in preventing cortisone-induced [[osteoporosis]]. <ref name=":4" />


== Differential Diagnosis ==
== Differential Diagnosis ==
 
{| class="wikitable"
Behcet's is rare in the U.S. Many times it is misdiagnosed for other conditions based on the symptoms. [[Image:Skin.JPG|frame|right|Skin lesions on forearm]]  
|+Table 1: The differential diagnoses of BD according to system involvement
 
!'''System Involvement'''
If lesions appear on the mouth, skin,&nbsp;or genitals, it can be mistaken for:  
!'''Differential Diagnoses'''
 
|-
*Cold sores
|Mucocutaneous involvement
*Canker sores
|[[Herpes Zoster|Herpes]], [[neutropenia]], pemphigus, Chronic Intestinal Inflammatory Disease (CIID), drugs, vitamin deficiencies <ref name=":4" />
*Genital Herpes
|-
*Herpes
|Musculoskeletal involvement
*Ache
|[[Systemic Lupus Erythematosus]], [[Reactive Arthritis]], [[Psoriatic Arthritis]], [[Ankylosing Spondylitis (Axial Spondyloarthritis)|Ankylosing Spondylitis]], [[Juvenile Idiopathic Arthritis]], [[Sarcoidosis]], Inflammatory Bowel Diseases <ref name=":3" />
 
|-
If lesions appear in the GI tract, these are possible incorrect diagnosis:
|Vascular involvement
 
|Septic aneurysms, atrophic polychondritis, Takayasu <ref name=":4" />
*[[Crohn's Disease|Crohn's Disease]]  
|-
*[[Peptic Ulcers|Peptic Ulcer]]  
|Gastrointestinal involvement
*Ulcerative Colitis
|Inflammatory Bowel Disease, [[Crohn's Disease]], [[Peptic Ulcers|Peptic Ulcer]], Reiter's Syndrome, Ulcerative Colitis, CIID, NSAID toxicity, infectious colitis <ref name=":3" /><ref name=":4" />
*Inflammatory Bowel Disease
|-
*Reiter's syndrome
|Neurologic involvement
 
|[[Multiple Sclerosis (MS)|Multiple Sclerosis]], [[sarcoidosis]], tumoral pathologies, [[Lymphoma|lymphomas]], infectious [[meningoencephalitis]]  <ref name=":4" />
Other possible diagnosis:  
|}
 
*Chancroid&nbsp;
*Chronic Fatigue Syndrome
*Encephalitis
*Granuloma inguinale
*Mosquito-borne diseases
*[[MS Multiple Sclerosis|Multiple Sclerosis]]  
*Oral Ulcer
*Superficial thrombophlebitis
*Vulvar cancer
*Vulvar lesions<ref name="6.">WD. Misdiagnosis of behcet’s disease. www.wrongdiagnosis.com. (accessed 31 March 2011).</ref>  


== '''Case Studies'''  ==
== '''Case Studies'''  ==


*&nbsp;Regional distinction for the clinical severity of Behcet's disease in Korea: four university-based medical centre studies. <ref name="9.">Choe JY, Chung WT, Lee SW, et al. Regional distinction for the clinical severity of Behcet’s disease in Korea: four university-based medical centre studies. Clinical and experimental rheumatology. 2010;28:S20-S26.</ref>[view article in Clinical and Experimental Rheumatology, 2010] <br>
*Regional distinction for the clinical severity of Behcet's disease in Korea: four university-based medical centre studies. <ref name="9.">Choe JY, Chung WT, Lee SW, et al. Regional distinction for the clinical severity of Behcet’s disease in Korea: four university-based medical centre studies. Clinical and experimental rheumatology. 2010;28:S20-S26.</ref>[view article in Clinical and Experimental Rheumatology, 2010]  
*Clinical manifestations of Behcet's disease: an analysis of 2147 patients<ref name="10.">Gurler A, Boyvat A, Tursen U. Clinical manifestations of Behcet’s disease: an analysis of 2147 patients. Yonsei Medical Journal. 1997; 38:423-427.</ref> [view article in Yonsei Medical Journal]  
*Clinical manifestations of Behcet's disease: an analysis of 2147 patients <ref name="10.">Gurler A, Boyvat A, Tursen U. Clinical manifestations of Behcet’s disease: an analysis of 2147 patients. Yonsei Medical Journal. 1997; 38:423-427.</ref> [view article in Yonsei Medical Journal]  
*Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients <ref name="12.">Akman-Demir G, Serdaroglu P, Tasci B. Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. Oxford Journals. 1999;122:2171-2182.</ref>&nbsp;[ view article in Oxford Journal]<br>
*[https://academic.oup.com/brain/article/122/11/2171/377380 Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients]
*Clinical studies on Behcet's syndrome. <ref name="12." />&nbsp;[view article at&nbsp;European Journal of&nbsp;Cardiothoracic Surgery]
*[https://watermark.silverchair.com/36-4-771.pdf?token=AQECAHi208BE49Ooan9kkhW_Ercy7Dm3ZL_9Cf3qfKAc485ysgAAA2EwggNdBgkqhkiG9w0BBwagggNOMIIDSgIBADCCA0MGCSqGSIb3DQEHATAeBglghkgBZQMEAS4wEQQM8yCCCBZehZjGfU47AgEQgIIDFFCopih_bqvDTNDcg3vCIsNZGthNUyofOMeaTSAflk2FEFWaRqpv65CoXRj_0jgj8h2XOr-mCjK1tMCEUG9h4QMCHffLTWZ_eDHdnEbF0PZ4ddOKBSXoBwevTW-rXW1UFSPa7vJfX1SMHovNpQlMfRz7bfUS7aM_N92IpBkuS7dc7zDE_sLEkDpxSnPUNAAa0CmZYcI3UnICl_9iFscz6M3YCFe3w7vTonEuz1Z-hqO8U9tuMKZwRVM3CfDE3xq2g-HOl31sl_Z0mD-kc5GtcMF8CwKvCApy9T4iK7Od4x4bvetjygs0G7p0D5CkEsemfe_YgkPqIN7-ak2Ux0rlS3dsbJ0b3YN4FQbId1Rv2QH9SWt9cpLlIoA1bTYNLnNCyEJtu4JmIcEmYCJkn0E4gP92kGg5iDNWipypg1aFuIIdMvd5kiRZ_wPbOPES-l_sBbYzUK6XJmd-TK-bzD_d-d3PCvZ_1570zmx0sMH5nF8LihA_ydQI3cQXMochvCQdRDpF918GMqjiC3xYWtQmJnMU1ooA2aP88PhtivmjIV8ZRhiJHMHjPYgD7BRPQR7jhIaJCITz6rke6pvpUfN52pAHEr0pqFTId4X6nePMfcWJK334M7aG8G2ck0KCeg7fyxECON8Wga4O3qvwP-Ch40CFY3m9ztuqv2-Q_gsrUg3MF8dfpCu-Ez3pW0kfw4TwqzniyGoavG3xJAEafrNihVO99BfE3KckHwS8Z8Oz2RzEldQtZaN3uU-cIaNSQ9W202h9uA8h-ZQFHZg-BPkSzgrEn-GrTOZj2WFfkxUAInK4gQud95WOdTuglFJtzsaUBYzDYM0vA2FOzkn-XIl2WyBakAV90nM7VR51E-Y0Zq_wtk6K_gpRWOKVcRJjRJjfRzIht8RDPV94Wu2U1sYhhUk8qST6KpdKIl_bz9XMHQFwbmaHk6qHSbBaIrckoaEZc8RKVbzHE-RSbuTWlladGjh-W-QXpA5EiRA2og0mZQi6O3liCXbJVptqxk97aQoQqQyHGCiS8ghYttHwKTHsLdWRt17g Aortic valve replacement in Behcet’s disease: surgical modification to prevent valve detachment]


== Resources  ==
== Resources  ==


*American Behcet's Disease Association:&nbsp;&nbsp; [http://www.behcets.com/site/pp.asp?c=bhJIJSOCJrH&b=260521 http://www.behcets.com/site/pp.asp?c=bhJIJSOCJrH&amp;b=260521]  
*American Behcet's Disease Association:&nbsp;&nbsp; [https://www.behcets.com http://www.behcets.com/site/pp.asp?c=bhJIJSOCJrH&amp;b=260521 ]
*Medicine Net: Bechet's Disease:&nbsp;&nbsp; http://www.medicinenet.com/behcets_syndrome/article.htm  
*Medicine Net: Bechet's Disease:&nbsp;&nbsp; http://www.medicinenet.com/behcets_syndrome/article.htm  
*Mayo Clinic: Behcet's Disease:&nbsp;&nbsp;&nbsp;http://www.mayoclinic.com/health/behcets-disease/DS00822  
*Mayo Clinic: Behcet's Disease:&nbsp;&nbsp;&nbsp;http://www.mayoclinic.com/health/behcets-disease/DS00822  
*John Hopkins:&nbsp; Vasculitis Center:&nbsp;&nbsp;&nbsp;http://www.hopkinsvasculitis.org/types-vasculitis/behcets-disease/  
*John Hopkins:&nbsp; Vasculitis Center:&nbsp;&nbsp;&nbsp;http://www.hopkinsvasculitis.org/types-vasculitis/behcets-disease/  
*Essential Guide to Behcet's Disease:&nbsp;http://www.behcetsdisease.com/behcets-basics.html
*An Employer's Guide from the Behçet’s UK organisation: https://behcetsuk.org/wp-content/uploads/2019/06/BUK-Employers-Guide-v2.0-Jun19.pdf
*A presentation of Dr. Erkan Demirkaya from the 2nd NIH-WRNMMC Symposium on Autoinflammatory and Immunedysregulatory Diseases, held at NIH on August 22 and 23, 2019:
 
{{#ev:youtube|DOWOpBVx0k0}}<ref>The National Institute of Allergy and Infectious Diseases (NIAID). Diagnosis and Management of Behçet’s Disease. Available from: https://www.youtube.com/watch?v=DOWOpBVx0k0</ref>


== References  ==
== References  ==
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[[Category:Conditions]]
[[Category:Conditions]]
[[Category:Autoimmune Disorders]]
[[Category:Autoimmune Disorders]]
[[Category:Rheumatology]]

Latest revision as of 16:40, 9 September 2023

Original Editors - Jennifer Edwards from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Jennifer Edwards, Sehriban Ozmen, Elaine Lonnemann, Admin, Kim Jackson, 127.0.0.1, Laura Ritchie, Wendy Walker, WikiSysop and Lucinda hampton  


Definition/Description[edit | edit source]

"Behcet's Disease" (BD) is a chronic disease characterized by inflammation in the blood vessels and relapsing and remitting course. [1] It can also be referred as a "Behcet Syndrome" since it involves different body systems such as skin, mucosa, joints, nervous and gastrointestinal systems. [2]

The video below is a great introduction to the condition provided by the American Behcet's Disease Association (ABDA), featuring educational information and patients' stories.

[3]

Prevalence[edit | edit source]

Route of Silk Road

The prevalence is variable worldwide [4] but higher in the countries along the Silk Road. [1][4] A recent study calculated the prevalence of BD as 10.3 per 100,000 population globally and 119.8 for Turkey, 31.8 for the Middle East, 4.5 for Asia, 5.3 for Southern Europe, 2.1 for Northern Europe, and 3.8 for North America/Caribbean Islands. [5]



Pathological Process[edit | edit source]

The etiology is unknown [1] [6] and extensive debate existed on whether to classify it as autoimmune or autoinflammatory but recently classified as the major histocompatibility complex class I (MHC-I) opathy [6]. Genetic factors are thought to play a crucial role since strong association with HLA-B51 antigen is proved [7][8][1][4] as well as higher prevalence in specific ethnic populations, and high sibling recurrence rate. [4]

Clinical Presentation[edit | edit source]

Oral ulcer in Behcet Syndrome

Vasculitis in BD can involve any kind and size of vessels, and this explains why the disease can have an effect on multiple systems in the body. [9] So, the patient could present with any of the symptom(s) below:

  • Mucocutaneous involvement: Oral aphthosis, genital aphthosis, papulopustular lesions, pathergy phenomenon, erythema-nodosu-like lesions, superficial thrombophlebitis, pyodermagangrenosum-like lesions, erythemamultiforme-like lesions, nailfold capillary abnormalities. [9]
  • Ocular involvement: Uveitis, vitritis, retinal infiltrates, sheathing of retinal veins, occlusive vasculitis, neovascularization, secondary cataracts, glaucoma, frosted branch angiitis and macular oedema, conjunctivitis, and conjunctival aphthosis, and scleritis being less common. [9] Ocular involvement is the most common cause of morbidity in people with BD. [10]
  • Musculoskeletal involvement: Arthritis, arthralgia, enthesopathy, avascular necrosis, myalgia, and myositis. [9]
  • Vascular involvement: Pulmonary, carotid, aortic, iliac, femoral, and popliteal arterial involvement; pulmonary aneurysm; superficial and deep vein thrombosis; Budd-Chiari syndrome; superior and inferior vena cava occlusion; and dural sinus thrombosis. [9]
  • Cardiac involvement: Pericarditis, myocarditis, endocarditis with valvular regurgitation, coronary arteritis, coronary artery aneurysms, diastolic dysfunction, atrial septal aneurysm, conduction system disturbances, atrial and ventricular arrhythmias, mitral valve prolapse, intracardiac thrombosis, valvular insufficiency, and myocardial infarction. [9]
    Sigmoid sinus thrombosis presenting with posterior alexia in a patient with Behcet's disease and polycythaemia: a case report.
  • Gastrointestinal (GI) involvement: The most common symptoms are abdominal pain, nausea, vomiting, diarrhoea, and GI bleeding. The GI manifestations of BD are known to cause significant morbidity and mortality. [11] [12]
  • Neurologic involvement: The manifestations include focal or multifocal parenchymal involvement, isolated cerebral venous sinus thrombosis, intracranial hypertension, isolated behavioural syndromes, peripheral nervous system involvement, and secondary neurologic involvement. [9] The combination of neurologic symptoms and/or signs in patients diagnosed with BD is called Neuro-Behcet's disease (NBD). [13] NBD is a serious risk for mortality and usually occurs in males. [14][4]
  • Pulmonary involvement: Pulmonary artery aneurism, pulmonary infection, pulmonary vasculitis, pulmonary fibrosis, pleuritis, and pulmonary emboli. [9]


Diagnostic Procedures[edit | edit source]

MRI of central nervous system involvement in BD (white matter changes in the pons)

Since there is no specific biomarker for BD, the diagnosis process is based on the clinical picture. [4] Although more than fifteen classification criteria have been proposed for BD to date [15], the most frequently used one in studies has been the International Study Group (ISG) Criteria with 92% sensitivity and 97% specificity[16][17], which was set in 1990. Accordingly, the criteria to be met are:

  • Oral ulcerations at least three times in one year.
  • Plus any two of the following:
  • Recurring genital ulceration
  • Eye lesions
  • Skin lesions
  • Positive pathergy test [18]

Outcome Measures[edit | edit source]

A recent study [4] summarised the outcome measures based on a systematic review by The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group [19]:

  • Behçet's Disease Current Activity Form (BDCAF)
  • Clinical Disease Activity Index
  • Clinical Manifestations Index
  • Iranian BD Dynamic Activity Measure,
  • 1994 Criteria for Disease Activity of BD
  • Krause's Total Severity Score
  • BD Quality of Life
  • Oral Ulcer Composite Index

However, it also indicated that:

  • Most of the measures used in the studies were not properly validated, and standard definitions were absent for outcome variables like response, relapse, or remission.
  • Even though specific outcome measures were developed for BD, they were not widely used in studies. For instance, BDCAF, which is the most commonly used disease activity measure, has been used in only 10% of the studies.
  • Internet-based surveys conducted by the OMERACT Vasculitis Working Group among expert physicians from relevant specialities from multiple countries [20] have emphasized the need for widely acceptable and properly validated outcome measures for BD.

Management / Interventions[edit | edit source]

Multidisciplinary Team[edit | edit source]

  • Treatment and follow-up are ideally coordinated by a paediatrician or adult practice physician with expertise in BD. This is most often an internal medicine specialist, dermatologist, or rheumatologist. The general physician or pediatrician plays a crucial role in optimizing the coordination of the proposed care plan. [21]
  • Other specialized physicians such as ophthalmologists, neurologists, vascular physicians, gastroenterologists, and pulmonologists may also be involved in patient care. [21]
  • Other health professionals such as those in therapeutic education, dietitians, state-certified nurses, physiotherapists, occupational therapists, psychologists, and social welfare assistants may also be involved upon as necessary. [21]

Medical Management[edit | edit source]

  • In the treatment and secondary prevention of thrombosis in Behcet's Disease, immunosuppressants are currently preferred over anticoagulants. [22]
  • Recently, Apremilast has been approved for the treatment of oral ulcers in Behcet's Syndrome in the United States. The efficacy of Apremilast in controlling non-oral ulcer features of the syndrome is still under investigation. [23]
  • Tumor necrosis factor inhibitors have proven to be safe and effective for treating eye disease in Behcet's Syndrome with long-term use. [23]
  • While new treatment options such as ustekinumab, secukinumab, tocilizumab, and others show promising early data, more studies are needed to better understand their role in Behcet's management. [23]

Physical Therapy[edit | edit source]

To prevent cortisone myopathy, patients should be advised to engage in regular physical activities, such as fast walking for 30 to 45 minutes each day. If they are diagnosed with amyotrophy, they may even need to undergo kinesiology therapy to strengthen their muscles. Engaging in physical activity is also crucial in preventing cortisone-induced osteoporosis. [21]

Differential Diagnosis[edit | edit source]

Table 1: The differential diagnoses of BD according to system involvement
System Involvement Differential Diagnoses
Mucocutaneous involvement Herpes, neutropenia, pemphigus, Chronic Intestinal Inflammatory Disease (CIID), drugs, vitamin deficiencies [21]
Musculoskeletal involvement Systemic Lupus Erythematosus, Reactive Arthritis, Psoriatic Arthritis, Ankylosing Spondylitis, Juvenile Idiopathic Arthritis, Sarcoidosis, Inflammatory Bowel Diseases [9]
Vascular involvement Septic aneurysms, atrophic polychondritis, Takayasu [21]
Gastrointestinal involvement Inflammatory Bowel Disease, Crohn's Disease, Peptic Ulcer, Reiter's Syndrome, Ulcerative Colitis, CIID, NSAID toxicity, infectious colitis [9][21]
Neurologic involvement Multiple Sclerosis, sarcoidosis, tumoral pathologies, lymphomas, infectious meningoencephalitis [21]

Case Studies[edit | edit source]

Resources[edit | edit source]

[26]

References[edit | edit source]

  1. ↑ 1.0 1.1 1.2 1.3 Alpsoy E, Bozca BC, Bilgic A. Behçet disease: an update for dermatologists. American Journal of Clinical Dermatology. 2021 Jul;22(4):477-502.
  2. ↑ Bettiol A, Prisco D, Emmi G. Behçet: the syndrome. Rheumatology. 2020 May 1;59(Supplement_3):iii101-7.
  3. ↑ Bechet's Disease Explained. Available from http://www.youtube.com/watch?v=Txf7cWW8HwI [accessed 28/98/19]
  4. ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 Akkoç N. Update on the epidemiology, risk factors and disease outcomes of Behçet's disease. Best Practice & Research Clinical Rheumatology. 2018 Apr 1;32(2):261-70.
  5. ↑ Maldini C, Druce K, Basu N, LaValley MP, Mahr A. Exploring the variability in Behçet’s disease prevalence: a meta-analytical approach. Rheumatology. 2018 Jan 1;57(1):185-95.
  6. ↑ 6.0 6.1 Rodríguez-Carrio J, Nucera V, Masala IF, Atzeni F. Behçet disease: from pathogenesis to novel therapeutic options. Pharmacological Research. 2021 May 1;167:105593.
  7. ↑ Ohno S, Ohguchi M, Hirose S, Matsuda H, Wakisaka A, Aizawa M. Close association of HLA-Bw51 with Behçet's disease. Archives of ophthalmology. 1982 Sep 1;100(9):1455-8.
  8. ↑ Ohno S, Aoki K, Sugiura S, Nakayama E, Itakura K, Aizawa M. HL-A5 and Behcet's disease. The Lancet. 1973 Dec 15;302(7842):1383-4.
  9. ↑ 9.0 9.1 9.2 9.3 9.4 9.5 9.6 9.7 9.8 9.9 Akdeniz N, Elmas ÖF, Karadağ AS. Behçet syndrome: A great imitator. Clinics in Dermatology. 2019 May 1;37(3):227-39.
  10. ↑ Davatchi F, Chams-Davatchi C, Shams H, Shahram F, Nadji A, Akhlaghi M, Faezi T, Ghodsi Z, Sadeghi Abdollahi B, Ashofteh F, Mohtasham N. Behcet’s disease: epidemiology, clinical manifestations, and diagnosis. Expert review of clinical immunology. 2017 Jan 2;13(1):57-65.
  11. ↑ Nguyen A, Upadhyay S, Javaid MA, Qureshi AM, Haseeb S, Javed N, Cormier C, Farooq A, Sheikh AB. Behcet’s disease: an in-depth review about pathogenesis, gastrointestinal manifestations, and management. Inflammatory Intestinal Diseases. 2021 Nov 4;6(4):175-85.
  12. ↑ Skef W, Hamilton MJ, Arayssi T. Gastrointestinal Behçet's disease: a review. World Journal of Gastroenterology: WJG. 2015 Apr 4;21(13):3801.
  13. ↑ Borhani-Haghighi A, Kardeh B, Banerjee S, Yadollahikhales G, Safari A, Sahraian MA, Shapiro L. Neuro-Behcet's disease: an update on diagnosis, differential diagnoses, and treatment. Multiple sclerosis and related disorders. 2020 Apr 1;39:101906.
  14. ↑ Benamour S, Naji T, Alaoui FZ, El Kabli H, El Aidouni S. Manifestations neurologiques de la maladie de Behçet. Revue Neurologique. 2006 Nov 1;162(11):1084-90.
  15. ↑ Davatchi F, Sadeghi Abdollahi B, Chams‐Davatchi C, Shahram F, Shams H, Nadji A, Faezi T, Akhlaghi M, Ghodsi Z, Mohtasham N, Ashofteh F. The saga of diagnostic/classification criteria in Behcet's disease. International journal of rheumatic diseases. 2015 Jul;18(6):594-605.
  16. ↑ Wechsler FB, Davatchi F. Criteria for diagnosis of Behcet's disease. The Lancet. 1990;335(8697):1078-80.
  17. ↑ International Study Group for Behçet's Disease, Wechsler B, Davatchi F, Mizushima Y, Hamza M, Dilsen N, Kansu E, yazici H, Barnes CG, Chamberlain MA, James DG. Evaluation of diagnostic (‘classification’) criteria in Behçet's disease—towards internationally agreed criteria. Rheumatology. 1992 May 1;31(5):299-308.
  18. ↑ Wechsler FB, Davatchi F. Criteria for diagnosis of Behcet's disease. The Lancet. 1990;335(8697):1078-80.
  19. ↑ Hatemi G, Merkel PA, Hamuryudan V, Boers M, Direskeneli H, Aydin SZ, Yazici H. Outcome measures used in clinical trials for Behçet syndrome: a systematic review. The Journal of rheumatology. 2014 Mar 1;41(3):599-612.
  20. ↑ Hatemi G, Ozguler Y, Direskeneli H, Mahr A, Gul A, Levi V, Aydin SZ, Mumcu G, Sertel-Berk O, Stevens RM, Yazici H. Current status, goals, and research agenda for outcome measures development in Behçet syndrome: report from OMERACT 2014. The Journal of rheumatology. 2015 Dec 1;42(12):2436-41.
  21. ↑ 21.0 21.1 21.2 21.3 21.4 21.5 21.6 21.7 Kone-Paut I, Barete S, Bodaghi B, Deiva K, Desbois AC, Galeotti C, Gaudric J, Kaplanski G, Mahr A, Noel N, Piram M. French recommendations for the management of Behçet’s disease. Orphanet journal of rare diseases. 2021 Feb;16:1-28.
  22. ↑ Bettiol A, Alibaz-Oner F, Direskeneli H, Hatemi G, Saadoun D, Seyahi E, Prisco D, Emmi G. Vascular Behçet syndrome: from pathogenesis to treatment. Nature Reviews Rheumatology. 2023 Feb;19(2):111-26.
  23. ↑ 23.0 23.1 23.2 Yazici Y. Management of Behçet syndrome. Current opinion in rheumatology. 2020 Jan 1;32(1):35-40.
  24. ↑ Choe JY, Chung WT, Lee SW, et al. Regional distinction for the clinical severity of Behcet’s disease in Korea: four university-based medical centre studies. Clinical and experimental rheumatology. 2010;28:S20-S26.
  25. ↑ Gurler A, Boyvat A, Tursen U. Clinical manifestations of Behcet’s disease: an analysis of 2147 patients. Yonsei Medical Journal. 1997; 38:423-427.
  26. ↑ The National Institute of Allergy and Infectious Diseases (NIAID). Diagnosis and Management of Behçet’s Disease. Available from: https://www.youtube.com/watch?v=DOWOpBVx0k0


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