Behçet's disease pathophysiology: Difference between revisions
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==Genetics== | ==Genetics== | ||
* | *The development of behcet disease is the result of multiple genetic mutations. | ||
*Genes involved in the pathogenesis of behcet disease include human leukocyte antigens, particularly HLA-B51. <ref name="pmid19790126">{{cite journal |vauthors=de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A |title=HLA-B51/B5 and the risk of Behçet's disease: a systematic review and meta-analysis of case-control genetic association studies |journal=Arthritis Rheum. |volume=61 |issue=10 |pages=1287–96 |date=October 2009 |pmid=19790126 |pmc=3867978 |doi=10.1002/art.24642 |url=}}</ref> | *Genes involved in the pathogenesis of behcet disease include human leukocyte antigens, particularly HLA-B51. <ref name="pmid19790126">{{cite journal |vauthors=de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A |title=HLA-B51/B5 and the risk of Behçet's disease: a systematic review and meta-analysis of case-control genetic association studies |journal=Arthritis Rheum. |volume=61 |issue=10 |pages=1287–96 |date=October 2009 |pmid=19790126 |pmc=3867978 |doi=10.1002/art.24642 |url=}}</ref> | ||
*Familial cases of behcet disease have higher rates of HLA-B51 in compare to sporadic cases.<ref name="pmid1341477">{{cite journal |vauthors=Akpolat T, Koç Y, Yeniay I, Akpek G, Güllü I, Kansu E, Kiraz S, Ersoy F, Batman F, Kansu T |title=Familial Behçet's disease |journal=Eur J Med |volume=1 |issue=7 |pages=391–5 |date=November 1992 |pmid=1341477 |doi= |url=}}</ref> | *Familial cases of behcet disease have higher rates of HLA-B51 in compare to sporadic cases.<ref name="pmid1341477">{{cite journal |vauthors=Akpolat T, Koç Y, Yeniay I, Akpek G, Güllü I, Kansu E, Kiraz S, Ersoy F, Batman F, Kansu T |title=Familial Behçet's disease |journal=Eur J Med |volume=1 |issue=7 |pages=391–5 |date=November 1992 |pmid=1341477 |doi= |url=}}</ref> | ||
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==Gross Pathology== | ==Gross Pathology== | ||
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name]. | *On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name]. | ||
<figure-inline><figure-inline><figure-inline><figure-inline><figure-inline><figure-inline><figure-inline>[[File:Behcet's syndrome 11.jpeg|502x502px]]</figure-inline></figure-inline></figure-inline></figure-inline></figure-inline></figure-inline></figure-inline> | <figure-inline><figure-inline><figure-inline><figure-inline><figure-inline><figure-inline><figure-inline><figure-inline>[[File:Behcet's syndrome 11.jpeg|502x502px]]</figure-inline></figure-inline></figure-inline></figure-inline></figure-inline></figure-inline></figure-inline></figure-inline> | ||
==Microscopic Pathology== | ==Microscopic Pathology== | ||
Revision as of 19:10, 23 March 2018
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Risk calculators and risk factors for Behçet's disease pathophysiology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]
Overview
The exact pathogenesis of [disease name] is not fully understood.
OR
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
OR
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
OR
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
OR
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
OR
The progression to [disease name] usually involves the [molecular pathway].
OR
The pathophysiology of [disease/malignancy] depends on the histological subtype.
Pathophysiology
Pathogenesis
- The exact pathogenesis of [disease name] is not fully understood.
OR
- It is understood that behcet disease is the result of vasculitis. It involves all sizes of blood vessels ( small, medium, and large). Arteries and venis are both involved in behcet disease. Major mechanisms in pathogenesis of behcet disease include:
- Polygenic
- s with other autoimmune diseases, the disorder may represent aberrant immune activity triggered by exposure to an agent, perhaps infectious, in patients with a genetic predisposition to develop the disease. Major disease mechanisms in Behçet syndrome include the following
- [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
- Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
- [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
- The progression to [disease name] usually involves the [molecular pathway].
- The pathophysiology of [disease/malignancy] depends on the histological subtype.
Genetics
- The development of behcet disease is the result of multiple genetic mutations.
- Genes involved in the pathogenesis of behcet disease include human leukocyte antigens, particularly HLA-B51. [1]
- Familial cases of behcet disease have higher rates of HLA-B51 in compare to sporadic cases.[2]
- Genetic anticipation: earlier age of onset of disease in children of pateints with behcet disease.[3]
- Some studies shows that major histocompatibility complex class I chain related gene A (MICA) A6 allele is related to behcet disease. [4]
- Non-HLA genes also have roles in behcet diesease. They include: [5]
- Intercellular adhesion molecule (ICAM)-1 gene
- The endothelial nitric oxide synthase gene[6]
- TNF genes[7]
- The vascular endothelial growth factor (VEGF) gene[8]
- Manganese superoxide dismutase gene[9]
- Cytochrome P450 gene[10]
- Interleukin (IL)-10 gene[11]
- The IL-23 receptor gene[12]
- Missense mutations of the familial Mediterranean fever (MEFV) gene that encodes protein pyrin on the surface of neutrophils[13]
- The development of behcet disease is the result of multiple genetic mutations.
Associated Conditions
Gross Pathology
- On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
<figure-inline><figure-inline><figure-inline><figure-inline><figure-inline><figure-inline><figure-inline><figure-inline>
</figure-inline></figure-inline></figure-inline></figure-inline></figure-inline></figure-inline></figure-inline></figure-inline>
Microscopic Pathology
- On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
References
- ↑ de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A (October 2009). "HLA-B51/B5 and the risk of Behçet's disease: a systematic review and meta-analysis of case-control genetic association studies". Arthritis Rheum. 61 (10): 1287–96. doi:10.1002/art.24642. PMC 3867978. PMID 19790126.
- ↑ Akpolat T, Koç Y, Yeniay I, Akpek G, Güllü I, Kansu E, Kiraz S, Ersoy F, Batman F, Kansu T (November 1992). "Familial Behçet's disease". Eur J Med. 1 (7): 391–5. PMID 1341477.
- ↑ Fresko I, Soy M, Hamuryudan V, Yurdakul S, Yavuz S, Tümer Z, Yazici H (January 1998). "Genetic anticipation in Behçet's syndrome". Ann. Rheum. Dis. 57 (1): 45–8. PMC 1752455. PMID 9536823.
- ↑ Wei F, Zhang YU, Li W (June 2016). "A meta-analysis of the association between Behçet's disease and MICA-A6". Biomed Rep. 4 (6): 741–745. doi:10.3892/br.2016.644. PMC 4887777. PMID 27284416.
- ↑ Sakane T, Takeno M (September 2000). "Novel approaches to Behçet's disease". Expert Opin Investig Drugs. 9 (9): 1993–2005. doi:10.1517/13543784.9.9.1993. PMID 11060788.
- ↑ Karasneh JA, Hajeer AH, Silman A, Worthington J, Ollier WE, Gul A (May 2005). "Polymorphisms in the endothelial nitric oxide synthase gene are associated with Behçet's disease". Rheumatology (Oxford). 44 (5): 614–7. doi:10.1093/rheumatology/keh561. PMID 15705632.
- ↑ Ahmad T, Wallace GR, James T, Neville M, Bunce M, Mulcahy-Hawes K, Armuzzi A, Crawshaw J, Fortune F, Walton R, Stanford MR, Welsh KI, Marshall SE, Jewell DP (March 2003). "Mapping the HLA association in Behçet's disease: a role for tumor necrosis factor polymorphisms?". Arthritis Rheum. 48 (3): 807–13. doi:10.1002/art.10815. PMID 12632436.
- ↑ Salvarani C, Boiardi L, Casali B, Olivieri I, Cantini F, Salvi F, Malatesta R, La Corte R, Triolo G, Ferrante A, Filippini D, Paolazzi G, Sarzi-Puttini P, Nicoli D, Farnetti E, Chen Q, Pulsatelli L (September 2004). "Vascular endothelial growth factor gene polymorphisms in Behçet's disease". J. Rheumatol. 31 (9): 1785–9. PMID 15338501.
- ↑ Nakao K, Isashiki Y, Sonoda S, Uchino E, Shimonagano Y, Sakamoto T (February 2007). "Nitric oxide synthase and superoxide dismutase gene polymorphisms in Behçet disease". Arch. Ophthalmol. 125 (2): 246–51. doi:10.1001/archopht.125.2.246. PMID 17296902.
- ↑ Tursen U, Tamer L, Api H, Yildirim H, Baz K, Ikizoglu G, Atik U (February 2007). "Cytochrome P450 polymorphisms in patients with Behcet's disease". Int. J. Dermatol. 46 (2): 153–6. doi:10.1111/j.1365-4632.2007.02957.x. PMID 17269966.
- ↑ Sousa I, Shahram F, Francisco D, Davatchi F, Abdollahi BS, Ghaderibarmi F, Nadji A, Mojarad Shafiee N, Xavier JM, Oliveira SA (October 2015). "Brief report: association of CCR1, KLRC4, IL12A-AS1, STAT4, and ERAP1 With Behçet's disease in Iranians". Arthritis Rheumatol. 67 (10): 2742–8. doi:10.1002/art.39240. PMID 26097239.
- ↑ Yalçin B, Atakan N, Dogan S (December 2014). "Association of interleukin-23 receptor gene polymorphism with Behçet disease". Clin. Exp. Dermatol. 39 (8): 881–7. doi:10.1111/ced.12400. PMID 25156021.
- ↑ Livneh A, Aksentijevich I, Langevitz P, Torosyan Y, G-Shoham N, Shinar Y, Pras E, Zaks N, Padeh S, Kastner DL, Pras M (March 2001). "A single mutated MEFV allele in Israeli patients suffering from familial Mediterranean fever and Behçet's disease (FMF-BD)". Eur. J. Hum. Genet. 9 (3): 191–6. doi:10.1038/sj.ejhg.5200608. PMID 11313758.