Behcet syndrome

From Aaushi
Jump to navigation Jump to search

Introduction

Chronic, relapsing, multisystemic inflammatory disorder named after Turkish dermatologist who described it. A clinical diagnosis.

Etiology

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

Genetics

  • familial cases reported
  • linked to HLA-B5 & DR5 in Japan & Mediterranean countries

Clinical manifestations

Diagnostic criteria

Laboratory

Diagnostic procedures

Radiology

Complications

Management

More general terms

Additional terms

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. 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. 5.0 5.1 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: https://www.ncbi.nlm.nih.gov/pubmed/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: https://www.ncbi.nlm.nih.gov/pubmed/19790126
  8. 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. 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
  10. 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
  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: https://www.ncbi.nlm.nih.gov/pubmed/23441863
  12. 12.0 12.1 12.2 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

Patient information

Behcet' syndreom patient information

Database