Behçet's disease pathophysiology: Difference between revisions
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==Overview== | ==Overview== | ||
It is understood that Behçet disease is the result of [[vasculitis]]. It involves all sizes of [[Blood vessel|blood vessels]] ( small, medium, and large). [[Artery|Arteries]] and veins are both involved in Behçet disease. Major mechanisms in pathogenesis of Behçet disease include environmental factors such as [[bacteria]], [[Virus|viruses]], and [[heat shock proteins]] (present in some [[bacteria]] and some of the [[Bacteria|bacterial]] HSPs share similaritis with human HSPs). [[Streptococcus sanguinis]], [[streptococcus pyogenes]], and [[mycobacterium tuberculosis]] produce HSPs that trigger anti HSP60 and anti HSP65 [[antibodies]] and then they target human HSPs and [[Immunity (medical)|immune]] response such as [[uveitis]] in [[Parenchyma|parenchymal]] neuro-Behçet disease, [[T helper cell|CD4+ T cells]] activation, secretion of [[Cytokine|cytokines]] and [[inflammation]]. [[Gene|Genes]] involved in the pathogenesis of Behçet disease include [[Human leukocyte antigen|human leukocyte antigens]], particularly [[Human leukocyte antigen|HLA]]-B51. | |||
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==Pathophysiology== | ==Pathophysiology== | ||
===Pathogenesis=== | ===Pathogenesis=== | ||
*It is understood that | *It is understood that Behçet disease is the result of [[vasculitis]]. It involves all sizes of [[Blood vessel|blood vessels]] ( small, medium, and large). [[Artery|Arteries]] and [[Vein|veins]] are both involved in Behçet disease. Major mechanisms in pathogenesis of Behçet disease include:<ref name="pmid26868128">{{cite journal |vauthors=Zeidan MJ, Saadoun D, Garrido M, Klatzmann D, Six A, Cacoub P |title=Behçet's disease physiopathology: a contemporary review |journal=Auto Immun Highlights |volume=7 |issue=1 |pages=4 |date=December 2016 |pmid=26868128 |pmc=4751097 |doi=10.1007/s13317-016-0074-1 |url=}}</ref> | ||
**Polygenic | **[[Quantitative trait locus|Polygenic]] | ||
** Environmental factors such as bacteria, viruses, and [[heat shock proteins]] ( present in some bacteria and some of the bacterial HSPs share similaritis with human HSPs )<ref name="pmid24490075">{{cite journal |vauthors=Direskeneli H |title=Innate and Adaptive Responses to Heat Shock Proteins in Behcet's Disease |journal=Genet Res Int |volume=2013 |issue= |pages=249157 |date=2013 |pmid=24490075 |pmc=3893747 |doi=10.1155/2013/249157 |url=}}</ref> | ** Environmental factors such as [[bacteria]], [[Virus|viruses]], and [[heat shock proteins]] (present in some [[bacteria]] and some of the [[Bacteria|bacterial]] HSPs share similaritis with human HSPs).<ref name="pmid24490075">{{cite journal |vauthors=Direskeneli H |title=Innate and Adaptive Responses to Heat Shock Proteins in Behcet's Disease |journal=Genet Res Int |volume=2013 |issue= |pages=249157 |date=2013 |pmid=24490075 |pmc=3893747 |doi=10.1155/2013/249157 |url=}}</ref> | ||
*** | *** [[Streptococcus sanguinis]], [[streptococcus pyogenes]], and [[mycobacterium tuberculosis]] produce HSPs that trigger anti HSP60 and anti HSP65 [[antibodies]] and then they target human HSPs and [[Immune system|immune response]] such as [[uveitis]] in [[Parenchyma|parenchymal]] neuro-Behçet disease.<ref name="pmid10420201">{{cite journal |vauthors=Tanaka T, Yamakawa N, Koike N, Suzuki J, Mizuno F, Usui M |title=Behçet's disease and antibody titers to various heat-shock protein 60s |journal=Ocul. Immunol. Inflamm. |volume=7 |issue=2 |pages=69–74 |date=June 1999 |pmid=10420201 |doi= |url=}}</ref> | ||
** CD4+ T cells activation, secretion of | ** [[T helper cell|CD4+ T cells]] activation, secretion of [[Cytokine|cytokines]] and [[inflammation]]. | ||
==Genetics== | ==Genetics== | ||
*The development of | *The development of Behçet disease is the result of multiple [[Genetics|genetic]] [[Mutation|mutations]]. | ||
*Genes involved in the pathogenesis of | *Genes involved in the [[pathogenesis]] of Behçet disease include [[Human leukocyte antigen|human leukocyte antigens]], particularly [[Human leukocyte antigen|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 | *[[Family|Familial]] cases of Behçet disease have higher rates of [[Human leukocyte antigen|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> | ||
*Genetic anticipation: earlier age of onset of disease in children of | *[[Genetics|Genetic]] [[Anticipation (genetics)|anticipation]]: earlier [[Ageing|age]] of onset of [[disease]] in [[Child|children]] of patients with Behçet 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 | *Some studies shows that major [[histocompatibility]] complex class I chain related gene A (MICA) A6 [[allele]] is related to Behçet 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 also have roles in | *Non-[[HLA]] [[Gene|genes]] also have roles in Behçet diesease. They include: <ref name="pmid11060788">{{cite journal |vauthors=Sakane T, Takeno M |title=Novel approaches to Behçet's disease |journal=Expert Opin Investig Drugs |volume=9 |issue=9 |pages=1993–2005 |date=September 2000 |pmid=11060788 |doi=10.1517/13543784.9.9.1993 |url=}}</ref> | ||
**Intercellular adhesion molecule (ICAM)-1 gene | **[[Intercellular adhesion molecule]] ([[ICAM-1|ICAM]])-1 [[gene]] | ||
** | **[[Endothelial]] [[nitric oxide]] [[synthase]] [[gene]]<ref name="pmid15705632">{{cite journal |vauthors=Karasneh JA, Hajeer AH, Silman A, Worthington J, Ollier WE, Gul A |title=Polymorphisms in the endothelial nitric oxide synthase gene are associated with Behçet's disease |journal=Rheumatology (Oxford) |volume=44 |issue=5 |pages=614–7 |date=May 2005 |pmid=15705632 |doi=10.1093/rheumatology/keh561 |url=}}</ref> | ||
**TNF genes<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> | **[[Tumor necrosis factors|Tumor necrosis factor (TNF)]] [[Gene|genes]]<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> | ||
** | **[[Vascular endothelial growth factor]] ([[Vascular endothelial growth factor|VEGF]]) [[gene]]<ref name="pmid15338501">{{cite journal |vauthors=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 |title=Vascular endothelial growth factor gene polymorphisms in Behçet's disease |journal=J. Rheumatol. |volume=31 |issue=9 |pages=1785–9 |date=September 2004 |pmid=15338501 |doi= |url=}}</ref> | ||
**Manganese superoxide dismutase gene<ref name="pmid17296902">{{cite journal |vauthors=Nakao K, Isashiki Y, Sonoda S, Uchino E, Shimonagano Y, Sakamoto T |title=Nitric oxide synthase and superoxide dismutase gene polymorphisms in Behçet disease |journal=Arch. Ophthalmol. |volume=125 |issue=2 |pages=246–51 |date=February 2007 |pmid=17296902 |doi=10.1001/archopht.125.2.246 |url=}}</ref> | **[[Manganese]] [[superoxide dismutase]] [[gene]]<ref name="pmid17296902">{{cite journal |vauthors=Nakao K, Isashiki Y, Sonoda S, Uchino E, Shimonagano Y, Sakamoto T |title=Nitric oxide synthase and superoxide dismutase gene polymorphisms in Behçet disease |journal=Arch. Ophthalmol. |volume=125 |issue=2 |pages=246–51 |date=February 2007 |pmid=17296902 |doi=10.1001/archopht.125.2.246 |url=}}</ref> | ||
**Cytochrome P450 gene<ref name="pmid17269966">{{cite journal |vauthors=Tursen U, Tamer L, Api H, Yildirim H, Baz K, Ikizoglu G, Atik U |title=Cytochrome P450 polymorphisms in patients with Behcet's disease |journal=Int. J. Dermatol. |volume=46 |issue=2 |pages=153–6 |date=February 2007 |pmid=17269966 |doi=10.1111/j.1365-4632.2007.02957.x |url=}}</ref> | **[[Cytochrome P450]] [[gene]]<ref name="pmid17269966">{{cite journal |vauthors=Tursen U, Tamer L, Api H, Yildirim H, Baz K, Ikizoglu G, Atik U |title=Cytochrome P450 polymorphisms in patients with Behcet's disease |journal=Int. J. Dermatol. |volume=46 |issue=2 |pages=153–6 |date=February 2007 |pmid=17269966 |doi=10.1111/j.1365-4632.2007.02957.x |url=}}</ref> | ||
**Interleukin (IL)-10 gene<ref name="pmid26097239">{{cite journal |vauthors=Sousa I, Shahram F, Francisco D, Davatchi F, Abdollahi BS, Ghaderibarmi F, Nadji A, Mojarad Shafiee N, Xavier JM, Oliveira SA |title=Brief report: association of CCR1, KLRC4, IL12A-AS1, STAT4, and ERAP1 With Behçet's disease in Iranians |journal=Arthritis Rheumatol |volume=67 |issue=10 |pages=2742–8 |date=October 2015 |pmid=26097239 |doi=10.1002/art.39240 |url=}}</ref> | **[[Interleukin 10|Interleukin (IL)-10]] [[gene]]<ref name="pmid26097239">{{cite journal |vauthors=Sousa I, Shahram F, Francisco D, Davatchi F, Abdollahi BS, Ghaderibarmi F, Nadji A, Mojarad Shafiee N, Xavier JM, Oliveira SA |title=Brief report: association of CCR1, KLRC4, IL12A-AS1, STAT4, and ERAP1 With Behçet's disease in Iranians |journal=Arthritis Rheumatol |volume=67 |issue=10 |pages=2742–8 |date=October 2015 |pmid=26097239 |doi=10.1002/art.39240 |url=}}</ref> | ||
** | **IL-23 [[Receptor (biochemistry)|receptor]] [[gene]]<ref name="pmid25156021">{{cite journal |vauthors=Yalçin B, Atakan N, Dogan S |title=Association of interleukin-23 receptor gene polymorphism with Behçet disease |journal=Clin. Exp. Dermatol. |volume=39 |issue=8 |pages=881–7 |date=December 2014 |pmid=25156021 |doi=10.1111/ced.12400 |url=}}</ref> | ||
**Missense mutations of the familial Mediterranean fever (MEFV) gene that encodes protein pyrin on the surface of neutrophils<ref name="pmid11313758">{{cite journal |vauthors=Livneh A, Aksentijevich I, Langevitz P, Torosyan Y, G-Shoham N, Shinar Y, Pras E, Zaks N, Padeh S, Kastner DL, Pras M |title=A single mutated MEFV allele in Israeli patients suffering from familial Mediterranean fever and Behçet's disease (FMF-BD) |journal=Eur. J. Hum. Genet. |volume=9 |issue=3 |pages=191–6 |date=March 2001 |pmid=11313758 |doi=10.1038/sj.ejhg.5200608 |url=}}</ref> | **[[Missense mutation|Missense mutations]] of the [[Family|familial]] [[Mediterranean fever]] (MEFV) [[gene]] that encodes [[protein]] pyrin on the surface of [[Neutrophil|neutrophils]]<ref name="pmid11313758">{{cite journal |vauthors=Livneh A, Aksentijevich I, Langevitz P, Torosyan Y, G-Shoham N, Shinar Y, Pras E, Zaks N, Padeh S, Kastner DL, Pras M |title=A single mutated MEFV allele in Israeli patients suffering from familial Mediterranean fever and Behçet's disease (FMF-BD) |journal=Eur. J. Hum. Genet. |volume=9 |issue=3 |pages=191–6 |date=March 2001 |pmid=11313758 |doi=10.1038/sj.ejhg.5200608 |url=}}</ref> | ||
==Gross Pathology== | ==Gross Pathology== | ||
*On gross pathology, [ | *On gross pathology [[Oral ulcer|oral ulcers]], [[Sex organ|genital]] [[Ulcer|ulcers]], [[uveitis]], and [[Skin lesion|skin lesions]] are characteristic findings of Behçet disease. | ||
[[File:Behcet's syndrome 11.jpeg| | [[File:Behcet's syndrome 11.jpeg|thumb|left|369x369px|Oral ulcers [https://commons.wikimedia.org/wiki/File%3ABullae_and_Bleb.JPG Source:By Samuel Freire da Silva, M.D. in homage to The Master And Professor Delso Bringel Calheiros]]] | ||
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==Microscopic Pathology== | ==Microscopic Pathology== | ||
*On microscopic histopathological analysis, neutrophilic and CD4+ T lymphocytes infiltrate and vasculitis are characteristic findings of | *On microscopic histopathological analysis, [[Neutrophil|neutrophilic]] and [[T helper cell|CD4+ T lymphocytes]] infiltrate and [[vasculitis]] are characteristic findings of Behçet disease. | ||
==References== | ==References== | ||
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[[Category:Medicine]] | |||
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[[Category:Dermatology]] | [[Category:Dermatology]] | ||
[[Category:Rheumatology]] | [[Category:Rheumatology]] | ||
Latest revision as of 20:36, 29 July 2020
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2], Mahda Alihashemi M.D. [3]
Overview
It is understood that Behçet disease is the result of vasculitis. It involves all sizes of blood vessels ( small, medium, and large). Arteries and veins are both involved in Behçet disease. Major mechanisms in pathogenesis of Behçet disease include environmental factors such as bacteria, viruses, and heat shock proteins (present in some bacteria and some of the bacterial HSPs share similaritis with human HSPs). Streptococcus sanguinis, streptococcus pyogenes, and mycobacterium tuberculosis produce HSPs that trigger anti HSP60 and anti HSP65 antibodies and then they target human HSPs and immune response such as uveitis in parenchymal neuro-Behçet disease, CD4+ T cells activation, secretion of cytokines and inflammation. Genes involved in the pathogenesis of Behçet disease include human leukocyte antigens, particularly HLA-B51.
Pathophysiology
Pathogenesis
- It is understood that Behçet disease is the result of vasculitis. It involves all sizes of blood vessels ( small, medium, and large). Arteries and veins are both involved in Behçet disease. Major mechanisms in pathogenesis of Behçet disease include:[1]
- Polygenic
- Environmental factors such as bacteria, viruses, and heat shock proteins (present in some bacteria and some of the bacterial HSPs share similaritis with human HSPs).[2]
- Streptococcus sanguinis, streptococcus pyogenes, and mycobacterium tuberculosis produce HSPs that trigger anti HSP60 and anti HSP65 antibodies and then they target human HSPs and immune response such as uveitis in parenchymal neuro-Behçet disease.[3]
- CD4+ T cells activation, secretion of cytokines and inflammation.
Genetics
- The development of Behçet disease is the result of multiple genetic mutations.
- Genes involved in the pathogenesis of Behçet disease include human leukocyte antigens, particularly HLA-B51.[4]
- Familial cases of Behçet disease have higher rates of HLA-B51 in compare to sporadic cases.[5]
- Genetic anticipation: earlier age of onset of disease in children of patients with Behçet disease.[6]
- Some studies shows that major histocompatibility complex class I chain related gene A (MICA) A6 allele is related to Behçet disease. [7]
- Non-HLA genes also have roles in Behçet diesease. They include: [8]
- Intercellular adhesion molecule (ICAM)-1 gene
- Endothelial nitric oxide synthase gene[9]
- Tumor necrosis factor (TNF) genes[10]
- Vascular endothelial growth factor (VEGF) gene[11]
- Manganese superoxide dismutase gene[12]
- Cytochrome P450 gene[13]
- Interleukin (IL)-10 gene[14]
- IL-23 receptor gene[15]
- Missense mutations of the familial Mediterranean fever (MEFV) gene that encodes protein pyrin on the surface of neutrophils[16]
Gross Pathology
- On gross pathology oral ulcers, genital ulcers, uveitis, and skin lesions are characteristic findings of Behçet disease.
Microscopic Pathology
- On microscopic histopathological analysis, neutrophilic and CD4+ T lymphocytes infiltrate and vasculitis are characteristic findings of Behçet disease.
References
- ↑ Zeidan MJ, Saadoun D, Garrido M, Klatzmann D, Six A, Cacoub P (December 2016). "Behçet's disease physiopathology: a contemporary review". Auto Immun Highlights. 7 (1): 4. doi:10.1007/s13317-016-0074-1. PMC 4751097. PMID 26868128.
- ↑ Direskeneli H (2013). "Innate and Adaptive Responses to Heat Shock Proteins in Behcet's Disease". Genet Res Int. 2013: 249157. doi:10.1155/2013/249157. PMC 3893747. PMID 24490075.
- ↑ Tanaka T, Yamakawa N, Koike N, Suzuki J, Mizuno F, Usui M (June 1999). "Behçet's disease and antibody titers to various heat-shock protein 60s". Ocul. Immunol. Inflamm. 7 (2): 69–74. PMID 10420201.
- ↑ 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.