Behçet's disease pathophysiology: Difference between revisions
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*Genetic anticipation: earlier age of onset of disease in children of pateints with behcet disease.<ref name="pmid9536823">{{cite journal |vauthors=Fresko I, Soy M, Hamuryudan V, Yurdakul S, Yavuz S, Tümer Z, Yazici H |title=Genetic anticipation in Behçet's syndrome |journal=Ann. Rheum. Dis. |volume=57 |issue=1 |pages=45–8 |date=January 1998 |pmid=9536823 |pmc=1752455 |doi= |url=}}</ref> | *Genetic anticipation: earlier age of onset of disease in children of pateints with behcet disease.<ref name="pmid9536823">{{cite journal |vauthors=Fresko I, Soy M, Hamuryudan V, Yurdakul S, Yavuz S, Tümer Z, Yazici H |title=Genetic anticipation in Behçet's syndrome |journal=Ann. Rheum. Dis. |volume=57 |issue=1 |pages=45–8 |date=January 1998 |pmid=9536823 |pmc=1752455 |doi= |url=}}</ref> | ||
*Some studies shows that major histocompatibility complex class I chain related gene A (MICA) A6 allele is related to behcet disease. <ref name="pmid27284416">{{cite journal |vauthors=Wei F, Zhang YU, Li W |title=A meta-analysis of the association between Behçet's disease and MICA-A6 |journal=Biomed Rep |volume=4 |issue=6 |pages=741–745 |date=June 2016 |pmid=27284416 |pmc=4887777 |doi=10.3892/br.2016.644 |url=}}</ref> | *Some studies shows that major histocompatibility complex class I chain related gene A (MICA) A6 allele is related to behcet disease. <ref name="pmid27284416">{{cite journal |vauthors=Wei F, Zhang YU, Li W |title=A meta-analysis of the association between Behçet's disease and MICA-A6 |journal=Biomed Rep |volume=4 |issue=6 |pages=741–745 |date=June 2016 |pmid=27284416 |pmc=4887777 |doi=10.3892/br.2016.644 |url=}}</ref> | ||
* | *Non-HLA genes als have roles in behcet diesease. They include: <ref name="pmid12632436">{{cite journal |vauthors=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 |title=Mapping the HLA association in Behçet's disease: a role for tumor necrosis factor polymorphisms? |journal=Arthritis Rheum. |volume=48 |issue=3 |pages=807–13 |date=March 2003 |pmid=12632436 |doi=10.1002/art.10815 |url=}}</ref> | ||
**Intercellular adhesion molecule (ICAM)-1 gene | |||
**The endothelial nitric oxide synthase gene | |||
**TNF genes | |||
**The vascular endothelial growth factor (VEGF) gene | |||
**Manganese superoxide dismutase gene | |||
**Cytochrome P450 gene | |||
**Interleukin (IL)-10 gene | |||
**The IL-23 receptor gene | |||
** | |||
* Non-HLA genes also play a role in determining susceptibility to disease. Genome-wide screening of affected families with more than one affected member has identified additional, non-HLA regions of potential interest [44]. Examples include associations between Behçet syndrome and: | |||
* | |||
*The development of behcet disease is the result of multiple genetic mutations. | *The development of behcet disease is the result of multiple genetic mutations. | ||
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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>[[File:Behcet's syndrome 11.jpeg|502x502px]]</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> | ||
==Microscopic Pathology== | ==Microscopic Pathology== | ||
Revision as of 15:56, 22 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: Cafer Zorkun, M.D., Ph.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
- [Disease name] is transmitted in [mode of genetic transmission] pattern.
- 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 als have roles in behcet diesease. They include: [5]
- Intercellular adhesion molecule (ICAM)-1 gene
- The endothelial nitric oxide synthase gene
- TNF genes
- The vascular endothelial growth factor (VEGF) gene
- Manganese superoxide dismutase gene
- Cytochrome P450 gene
- Interleukin (IL)-10 gene
- The IL-23 receptor gene
- Non-HLA genes also play a role in determining susceptibility to disease. Genome-wide screening of affected families with more than one affected member has identified additional, non-HLA regions of potential interest [44]. Examples include associations between Behçet syndrome and:
- 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>
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.
- ↑ 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.