Behcet's syndrome: Difference between revisions
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In [[rheumatology]], '''Behcet's Syndrome''', or '''Behçet's Disease''', is a rare chronic inflammatory disease involving the small blood vessels. It is of unknown etiology and characterized by mucocutaneous ulceration in the mouth and genital region, and [[uveitis]] with hypopyon. Other symptoms | {{TOC|right}} | ||
In [[rheumatology]], '''Behcet's Syndrome''', or '''Behçet's Disease''', is a rare chronic inflammatory disease involving the small blood vessels. It is of unknown etiology, although it may be an [[autoimmune disease]] and have genetic components. | |||
The syndrome is characterized by mucocutaneous ulceration in the mouth and genital region, and [[uveitis]] with hypopyon. Recurrent oral ulcers are a key marker.<ref>International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. Lancet. May 5 1990;335(8697):1078-80.</ref> Other symptoms include painful, stiff and swollen joints <ref>http://www.nlm.nih.gov/medlineplus/behcetssyndrome.html</ref>. The neuro-ocular form may cause blindness and death. [[Synovitis]]; [[thrombophlebitis]]; gastrointestinal ulcerations; [[retinal vasculitis]];[[optic atrophy]] and [[meningitis]] may occur as well."<ref>{{MeSH}}</ref> | |||
A variation may be Hughes-Stovin Syndrome which is pulmonary artery aneurysms associated with systemic [[thrombosis]].<ref name="pmid17457658">{{cite journal| author=Emad Y, Ragab Y, Shawki Ael-H, Gheita T, El-Marakbi A, Salama MH| title=Hughes-Stovin syndrome: is it incomplete Behçet's? Report of two cases and review of the literature. | journal=Clin Rheumatol | year= 2007 | volume= 26 | issue= 11 | pages= 1993-6 | pmid=17457658 | A variation may be Hughes-Stovin Syndrome which is pulmonary artery aneurysms associated with systemic [[thrombosis]].<ref name="pmid17457658">{{cite journal| author=Emad Y, Ragab Y, Shawki Ael-H, Gheita T, El-Marakbi A, Salama MH| title=Hughes-Stovin syndrome: is it incomplete Behçet's? Report of two cases and review of the literature. | journal=Clin Rheumatol | year= 2007 | volume= 26 | issue= 11 | pages= 1993-6 | pmid=17457658 | ||
| url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=clinical.uthscsa.edu/cite&email=badgett@uthscdsa.edu&retmode=ref&cmd=prlinks&id=17457658 | doi=10.1007/s10067-007-0609-y }} <!--Formatted by http://sumsearch.uthscsa.edu/cite/--></ref><ref name="pmid15515789">{{cite journal| author=Erkan D, Yazici Y, Sanders A, Trost D, Yazici H| title=Is Hughes-Stovin syndrome Behçet's disease? | journal=Clin Exp Rheumatol | year= 2004 Jul-Aug | volume= 22 | issue= 4 Suppl 34 | pages= S64-8 | pmid=15515789 | | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=clinical.uthscsa.edu/cite&email=badgett@uthscdsa.edu&retmode=ref&cmd=prlinks&id=17457658 | doi=10.1007/s10067-007-0609-y }} <!--Formatted by http://sumsearch.uthscsa.edu/cite/--></ref><ref name="pmid15515789">{{cite journal| author=Erkan D, Yazici Y, Sanders A, Trost D, Yazici H| title=Is Hughes-Stovin syndrome Behçet's disease? | journal=Clin Exp Rheumatol | year= 2004 Jul-Aug | volume= 22 | issue= 4 Suppl 34 | pages= S64-8 | pmid=15515789 | ||
| url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=clinical.uthscsa.edu/cite&email=badgett@uthscdsa.edu&retmode=ref&cmd=prlinks&id=15515789 }} <!--Formatted by http://sumsearch.uthscsa.edu/cite/--></ref> | | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=clinical.uthscsa.edu/cite&email=badgett@uthscdsa.edu&retmode=ref&cmd=prlinks&id=15515789 }} <!--Formatted by http://sumsearch.uthscsa.edu/cite/--></ref> | ||
==Epidemiology== | |||
Apropos of the latter, it was first described in [[Turkey]] and [[Greece]], then a cluster of cases was found in [[Japan]], and further studies found an increased concentration along the traditional [[Silk Road]]. | |||
==Diagnosis== | |||
==Treatment== | |||
Topical steroids or [[sucralfate]] can help oral or skin lesions.<ref name=eMed-treat>{{citation | |||
| title = Behcet Disease: Treatment & Medication | |||
| author = Augusto C Posadas & Jeffrey R Lisse | |||
| date = 22 June 2009 | |||
| journal = eMedicine | |||
| url = http://emedicine.medscape.com/article/329099-treatment | |||
}}</ref> Colchicine has also been used to prevent mucocutaneous relapse,<ref>Aktulga E, Altac M, Muftüoglu A, et al. A double blind study of colchicine in Behçet's disease. Haematologica. Jun 1980;65(3):399-402</ref> and also for joint disease. | |||
Inhibitors of [[tumor necrosis factor-alpha]] have been of benefit, including [[etanercept]], [[infliximab]], and, with due regard for risk, [[thalidomide]] for severe lesions. <ref>{{citation | |||
| title = Thalidomide in the Treatment of the Mucocutaneous Lesions of the Behcet Syndrome | |||
A Randomized, Double-Blind, Placebo-Controlled Trial | |||
| author = Vedat Hamuryudan, ''et al.'' | |||
| journal = Ann Intern Med | |||
| date = 15 March 1998 | volume = 128 | issue = 6 | pages = 443-450 | |||
| url = http://www.annals.org/content/128/6/443.full}}</ref> | |||
==Notable cases== | ==Notable cases== | ||
Hughes-Stovin Syndrome was diagnosed in the "The Down Low" in season 6, episode 10 of the television series House. | Hughes-Stovin Syndrome was diagnosed in the "The Down Low" in season 6, episode 10 of the television series House. | ||
==References== | ==References== | ||
{{reflist|2}}} | |||
Latest revision as of 23:40, 18 August 2010
In rheumatology, Behcet's Syndrome, or Behçet's Disease, is a rare chronic inflammatory disease involving the small blood vessels. It is of unknown etiology, although it may be an autoimmune disease and have genetic components.
The syndrome is characterized by mucocutaneous ulceration in the mouth and genital region, and uveitis with hypopyon. Recurrent oral ulcers are a key marker.[1] Other symptoms include painful, stiff and swollen joints [2]. The neuro-ocular form may cause blindness and death. Synovitis; thrombophlebitis; gastrointestinal ulcerations; retinal vasculitis;optic atrophy and meningitis may occur as well."[3]
A variation may be Hughes-Stovin Syndrome which is pulmonary artery aneurysms associated with systemic thrombosis.[4][5]
Epidemiology
Apropos of the latter, it was first described in Turkey and Greece, then a cluster of cases was found in Japan, and further studies found an increased concentration along the traditional Silk Road.
Diagnosis
Treatment
Topical steroids or sucralfate can help oral or skin lesions.[6] Colchicine has also been used to prevent mucocutaneous relapse,[7] and also for joint disease.
Inhibitors of tumor necrosis factor-alpha have been of benefit, including etanercept, infliximab, and, with due regard for risk, thalidomide for severe lesions. [8]
Notable cases
Hughes-Stovin Syndrome was diagnosed in the "The Down Low" in season 6, episode 10 of the television series House.
References
- ↑ International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. Lancet. May 5 1990;335(8697):1078-80.
- ↑ http://www.nlm.nih.gov/medlineplus/behcetssyndrome.html
- ↑ Anonymous (2024), Behcet's syndrome (English). Medical Subject Headings. U.S. National Library of Medicine.
- ↑ Emad Y, Ragab Y, Shawki Ael-H, Gheita T, El-Marakbi A, Salama MH (2007). "Hughes-Stovin syndrome: is it incomplete Behçet's? Report of two cases and review of the literature.". Clin Rheumatol 26 (11): 1993-6. DOI:10.1007/s10067-007-0609-y. PMID 17457658. Research Blogging.
- ↑ Erkan D, Yazici Y, Sanders A, Trost D, Yazici H (2004 Jul-Aug). "Is Hughes-Stovin syndrome Behçet's disease?". Clin Exp Rheumatol 22 (4 Suppl 34): S64-8. PMID 15515789.
- ↑ Augusto C Posadas & Jeffrey R Lisse (22 June 2009), "Behcet Disease: Treatment & Medication", eMedicine
- ↑ Aktulga E, Altac M, Muftüoglu A, et al. A double blind study of colchicine in Behçet's disease. Haematologica. Jun 1980;65(3):399-402
- ↑ Vedat Hamuryudan, et al. (15 March 1998), "[http://www.annals.org/content/128/6/443.full Thalidomide in the Treatment of the Mucocutaneous Lesions of the Behcet Syndrome A Randomized, Double-Blind, Placebo-Controlled Trial]", Ann Intern Med 128 (6): 443-450
}