Behcet Disease: Difference between revisions

m (One case study hyperlinked. Another case study with the wrong reference changed with a study from the same journal mentioned before.)
m ("Clinical Presentation" is updated.)
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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:
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''': Painful sores called aphthous ulcers can be found on the lips, tongue, and inside of the cheek. 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. Genital sores less common, but still occur in more than half of the patients with this disease and they are usually mistaken as genital herpes and can be on the external or internal surface of the genital.
* '''Mucocutaneous involvement''': Painful sores called aphthous ulcers can be found on the lips, tongue, and inside of the cheek. 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. Genital sores less common, but still occur in more than half of the patients with this disease and they are usually mistaken as genital herpes and can be on the external or internal surface of the genital.
* '''Ocular involvement''': It 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.
* '''Ocular involvement''': It 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. 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 (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.), arthralgia, enthesopathy, avascular necrosis, myalgia, and myositis <ref name=":3" />.
* '''Musculoskeletal involvement''': 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.), arthralgia, enthesopathy, avascular necrosis, myalgia, and myositis <ref name=":3" />.
* '''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. <ref name=":3" />
* '''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. <ref name=":3" />
* '''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. <ref name=":3" />
* '''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. <ref name=":3" />
* '''Gastrointestinal (GI) involvement''': It 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.
* '''Gastrointestinal (GI) involvement''': It 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.
* '''Neurologic involvement''': Patient may experience fever, headache, stiff neck, and difficulty coordinating movements secondary to inflammation in the meninges in the brain and spinal cord.
* '''Neurologic involvement''': Patient may experience fever, headache, stiff neck, and difficulty coordinating movements secondary to inflammation in the meninges in the brain and spinal cord. 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.
 
BibTeXEndNoteRefManRefWorks</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, pleuritis, and pulmonary emboli. <ref name=":3" />
* '''Pulmonary involvement:''' Pulmonary artery aneurism, pulmonary infection, pulmonary vasculitis, pulmonary fibrosis, pleuritis, and pulmonary emboli. <ref name=":3" />


Revision as of 11:35, 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  

This article is currently under review and may not be up to date. Please come back soon to see the finished work! (05/09/2023)

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]

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: Painful sores called aphthous ulcers can be found on the lips, tongue, and inside of the cheek. 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. Genital sores less common, but still occur in more than half of the patients with this disease and they are usually mistaken as genital herpes and can be on the external or internal surface of the genital.
  • Ocular involvement: It 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. Ocular involvement is the most common cause of morbidity in people with BD. [10]
  • Musculoskeletal involvement: 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.), 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]
  • Gastrointestinal (GI) involvement: It 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.
  • Neurologic involvement: Patient may experience fever, headache, stiff neck, and difficulty coordinating movements secondary to inflammation in the meninges in the brain and spinal cord. The combination of neurologic symptoms and/or signs in patients diagnosed with BD is called Neuro-Behcet's disease (NBD). [11] NBD is a serious risk for mortality and usually occurs in males. [12][4]
  • Pulmonary involvement: Pulmonary artery aneurism, pulmonary infection, pulmonary vasculitis, pulmonary fibrosis, pleuritis, and pulmonary emboli. [9]

Diagnostic Procedures[edit | edit source]

File:Lungs.jpg
Behcet's affecting the lungs
MRI of CNS involvement (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 [13], the most frequently used one in studies has been the International Study Group (ISG) Criteria with 92% sensitivity and 97% specificity[14][15], 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 [16]

    A dermatologist may do a biopsy of the mouth, genitals, and skin lesions. [17]


Management / Interventions[edit | edit source]

Medical Management[edit | edit source]

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. [18]

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.

Medications[edit | edit source]

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

Moderate dosage of corticosteroids are used for exacerbations. For some patients with chronic pain, low doses of prednisone are indicated. Serious organ involvement calls for high doses of Prednisone and immunosuppressive treatment. Immunosuppressive medications include: azathrioprine, cyclosporine, cyclophosphamide, and chlorambucil [19]

Physical Therapy Management[edit | edit source]

Energy conservation techniques, low-moderate intensity exercise endurance and strengthening, ROM, education on disease process, relaxation techniques, modalities for pain control

Differential Diagnosis[edit | edit source]

Behcet's is rare in the U.S. Many times it is misdiagnosed for other conditions based on the symptoms.

File:Skin.JPG
Skin lesions on forearm

If lesions appear on the mouth, skin, or genitals, it can be mistaken for:

  • Cold sores
  • Canker sores
  • Genital Herpes
  • Herpes
  • Ache

If lesions appear in the GI tract, these are possible incorrect diagnosis:

Other possible diagnosis:

  • Chancroid 
  • Chronic Fatigue Syndrome
  • Encephalitis
  • Granuloma inguinale
  • Mosquito-borne diseases
  • Multiple Sclerosis
  • Oral Ulcer
  • Superficial thrombophlebitis
  • Vulvar cancer
  • Vulvar lesions[20]

Case Studies[edit | edit source]

Resources[edit | edit source]

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 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. BibTeXEndNoteRefManRefWorks
  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. BibTeXEndNoteRefManRefWorks
  9. ↑ 9.0 9.1 9.2 9.3 9.4 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. ↑ 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. BibTeXEndNoteRefManRefWorks
  12. ↑ 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.
  13. ↑ 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.
  14. ↑ Wechsler FB, Davatchi F. Criteria for diagnosis of Behcet's disease. The Lancet. 1990;335(8697):1078-80.
  15. ↑ 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.
  16. ↑ Wechsler FB, Davatchi F. Criteria for diagnosis of Behcet's disease. The Lancet. 1990;335(8697):1078-80.
  17. ↑ Arthritis Foundation. Behcet's disease: how is it diagnosed? http://www.arthritis.org (accessed February 22 2011).
  18. ↑ MedicineNet.com. Behcet's syndrome. http://www.medicinenet.com (accessed March 15 2011).
  19. ↑ The John Hopkins Vasculitis Center. Hellmann D. Behcet's disease. www.hopkinsvasculitis.org (accessed 22 February 2011).
  20. ↑ WD. Misdiagnosis of behcet’s disease. www.wrongdiagnosis.com. (accessed 31 March 2011).
  21. ↑ 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.
  22. ↑ Gurler A, Boyvat A, Tursen U. Clinical manifestations of Behcet’s disease: an analysis of 2147 patients. Yonsei Medical Journal. 1997; 38:423-427.


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