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Behcet syndrome

Chronic, relapsing, multisystemic inflammatory disorder named after Turkish dermatologist who described it. A clinical diagnosis. Etiology: 1) unknown 2) autoimmune Pathology: 1) vasculitis - can affect veins as well as arteries [4] 2) circulating autoantibodies to oral mucus membrane in 50% of patients Epidemiology: 1) increased prevalence in a belt from east Asia to Turkey [4] - incidence 1:10,000 in Japan, 1: 500,000 in USA 2) affects mainly young adults 3) males have more severe disease than females Genetics: 1) familial cases reported 2) linked to HLA-B5 & DR5 in Japan & Mediterranean countries Clinical manifestations: 1) mucocutaneous lesions a) erythema nodosum b) recurrent painful oral ulcers (aphthous stomatitis) - initial resolution in 1-3 weeks [4] c) recurrent painful genital ulcers d) folliculitis e) rash, acneiform lesions [4] f) odynophagia [9] g) 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] 2) recurrent eye manifestations (eye pain, photophobia) a) iritis b) uveitis (posterior & hypopyon) c) optic neuritis d) retinal vasculitis, occlusion of retinal vessels 3) oligoarthritis/synovitis a) non deforming, asymmetric b) most frequently involves ankles & knees 4) thrombophlebitis: superficial or deep [DVT] (25%) 5) vasculitis a) aortitis b) aneurysm c) pulmonary artery vasculitis 1] dyspnea 2] cough 3] chest pain 4] hemoptysis a] recurrent b] cause of death in 40% of patients 6) cardiac a) aortic insufficiency b) myocarditis c) pericarditis d) cardiac conduction system abnormalities, arrhythmias [4] 7) central nervous system a) intracranial hypertension b) psychiatric disturbances c) meningoencephalitis 8) GI mucosal ulcerations resembling inflammatory bowel disease 9) 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: 1) complete blood count (CBC) - leukocytosis 2) elevated inflammatory markers - elevated erythrocyte sedimentation rate [ESR] - elevated serum C-reactive protein [CRP] 3) antibodies to oral mucosa (50%) 4) see ARUP consult [5] Special laboratory: - slit lamp examination - leukocytes in anterior chambers of eyes - pathergy skin test Radiology: - chest X-ray changes which may be present a) pulmonary infiltrates b) pleural effusion c) prominent pulmonary arteries d) pulmonary artery aneurysms Complications: 1) blindness [4] 2) pulmonary embolism 3) bronchovascular anastomosis (pulmonary artery aneurysm communicating with bronchial tree) 4) aneurysmal rupture is a major cause of death 5) also see clinical manifestations [4] Management: 1) treatment is symptomatic & empirical 2) mucous membrane lesions may respond to topical glucocorticoids 3) apremilast FDA-approved for oral ulcers, thalidomide also acceptable [4] 4) colchicine 1ts line for prevention of mucocutaneous disease [12] - may be better for arthritis than oral ulcer [4] 5) topical &/or oral 5-aminosalicylates (salfasalzine, mesalamine) for GI involvement [4] 6) 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 7) acute thrombosis should be treated with immunosuppressive agents rather than anticoagulants because the cause of thrombosis is inflammatory [4] 8) hemoptysis may respond initially to glucocorticoids, but tends to recur

Interactions

disease interactions

Related

Hughes-Stovin syndrome

General

autoimmune disease syndrome vasculitis

Properties

PATHOLOGY: LEUKOCYTOCLASTIC-VENULITIS

Database Correlations

OMIM 109650

References

  1. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1669-70
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1910
  3. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 783
  4. 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. ARUP Consult: Behcet Syndrome The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/behcet-syndrome
  6. 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: 18817637
  7. 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: 19790126
  8. Saadoun D, Wechsler B, Desseaux K et al Mortality in Behcet's disease. Arthritis Rheum. 2010 Sep;62(9):2806-12 PMID: 20496419
  9. 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 PMID: 25714165 http://www.nejm.org/doi/full/10.1056/NEJMcpc1413303
  10. Dalvi SR, Yildirim R, Yazici Y. Behcet's Syndrome. Drugs. 2012 Dec 3;72(17):2223-41 PMID: 23153327
  11. 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: 23441863
  12. NEJM Knowledge+ Rheumatology
  13. National Institute of Arthritis and Muscluloskeletal and Skin Diseases (NIAMS) Behcet's Syndrome: https://www.niams.nih.gov/health-topics/behcets-disease