Behcet syndrome
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Introduction
Chronic, relapsing, multisystemic inflammatory disorder named after Turkish dermatologist who described it. A clinical diagnosis.
Etiology
- unknown
- autoimmune
Epidemiology
- increased prevalence in a belt from east Asia to Turkey[4]
- incidence 1:10,000 in Japan, 1: 500,000 in USA
- affects mainly young adults
- males have more severe disease than females
Pathology
- vasculitis
- circulating autoantibodies to oral mucus membrane in 50% of patients
Genetics
Clinical manifestations
- mucocutaneous lesions
- erythema nodosum
- recurrent painful oral ulcers (aphthous stomatitis)
- initial resolution in 1-3 weeks[4]
- recurrent painful genital ulcers
- folliculitis
- rash, acneiform lesions[4]
- odynophagia[9]
- pathergy is common in Turkish, but rare in American patients
- formation of sterile pustules at sites of venipuncture
- inflammation &/or ulceration at the site of minor trauma[4]
- recurrent eye manifestations (eye pain, photophobia)
- iritis
- uveitis (posterior & hypopyon)
- optic neuritis
- retinal vasculitis, occlusion of retinal vessels
- oligoarthritis/synovitis
- thrombophlebitis: superficial or deep [DVT] (25%)
- vasculitis
- aortitis
- aneurysm
- pulmonary artery vasculitis
- dyspnea
- cough
- chest pain
- hemoptysis
- recurrent
- cause of death in 40% of patients
- cardiac
- central nervous system
- intracranial hypertension
- psychiatric disturbances
- meningoencephalitis
- GI mucosal ulcerations resembling inflammatory bowel disease
- mononeuritis multiplex & respiratory tract disease are not features
Diagnostic criteria
- recurrent painful oral ulcers + >= 2 of the following
- recurrent genital ulcers
- eye disease (uveitis, retinal vasculitis)
- skin disease (erythema nodosum, pseudofolliculitis, acneiform lesions)
- pathergy[4]
Laboratory
- complete blood count (CBC)
- elevated inflammatory markers
- elevated erythrocyte sedimentation rate [ESR]
- elevated serum C-reactive protein [CRP]
- antibodies to oral mucosa (50%)
- see ARUP consult[5]
Diagnostic procedures
Radiology
- chest X-ray changes which may be present
Complications
- blindness[4]
- pulmonary embolism
- bronchovascular anastomosis (pulmonary artery aneurysm communicating with bronchial tree)
- aneurysmal rupture is a major cause of death
- also see clinical manifestations[4]
Management
- treatment is symptomatic & empirical
- mucous membrane lesions may respond to topical glucocorticoids
- apremilast FDA-approved for oral ulcers, thalidomide also acceptable[4]
- colchicine 1ts line for prevention of mucocutaneous disease[12]
- may be better for arthritis than oral ulcer[4]
- topical &/or oral 5-aminosalicylates (salfasalzine, mesalamine) for GI involvement[4]
- for resistant ulcers, ophthalmic or CNS involvement[4]
- DMARDs: azathioprine, cyclosporine
- hydroxychloroquine not effective for prevention of mucocutaneous ulcers[12]
- TNF-alpha-inhibitors: infliximab, adalimumab
- cyclophosphamide may be an option in severe cases
- DMARDs: azathioprine, cyclosporine
- acute thrombosis should be treated with immunosuppressive agents rather than anticoagulants because the cause of thrombosis is inflammatory[4]
- hemoptysis may respond initially to glucocorticoids, but tends to recur
More general terms
Additional terms
References
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1669-70
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1910
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 783
- ↑ 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 4.11 4.12 4.13 4.14 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ 5.0 5.1 ARUP Consult: Behcet Syndrome The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/behcet-syndrome
- ↑ Calamia KT, Kaklamanis PG. Behcet's disease: recent advances in early diagnosis and effective treatment. Curr Rheumatol Rep. 2008 Oct;10(5):349-55. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18817637
- ↑ de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A. HLA-B51/B5 and the risk of Behcet's disease: a systematic review and meta-analysis of case-control genetic association studies. Arthritis Rheum. 2009 Oct 15;61(10):1287-96 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19790126
- ↑ Saadoun D, Wechsler B, Desseaux K et al Mortality in Behcet's disease. Arthritis Rheum. 2010 Sep;62(9):2806-12 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20496419
- ↑ 9.0 9.1 Unizony SH, Kim ND, Hoang MP. Case Records of the Mass General Hospital. Case 7-2015: A 25-year-old man with oral ulcers, rash, and odynophagia. N Engl J Med. 2015 Feb 26;372(9):864-72 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/25714165 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMcpc1413303
- ↑ Dalvi SR, Yildirim R, Yazici Y. Behcet's Syndrome. Drugs. 2012 Dec 3;72(17):2223-41 PMID: https://www.ncbi.nlm.nih.gov/pubmed/23153327
- ↑ International Team for the Revision of the International Criteria for Behcet's Disease (ITR-ICBD). The International Criteria for Behcet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014 Mar;28(3):338-47 PMID: https://www.ncbi.nlm.nih.gov/pubmed/23441863
- ↑ 12.0 12.1 12.2 NEJM Knowledge+ Rheumatology
- ↑ National Institute of Arthritis and Muscluloskeletal and Skin Diseases (NIAMS) Behcet's Syndrome: https://www.niams.nih.gov/health-topics/behcets-disease
Patient information
Behcet' syndreom patient information