Skip to main content
Erschienen in: Autoimmunity Highlights 1/2016

Open Access 01.12.2016 | Review Article

Behçet’s disease physiopathology: a contemporary review

verfasst von: Mohamad J. Zeidan, David Saadoun, Marlene Garrido, David Klatzmann, Adrien Six, Patrice Cacoub

Erschienen in: Autoimmunity Highlights | Ausgabe 1/2016

Einloggen, um Zugang zu erhalten

Abstract

Behçet’s disease, also known as the Silk Road Disease, is a rare systemic vasculitis disorder of unknown etiology. Recurrent attacks of acute inflammation characterize Behçet’s disease. Frequent oral aphthous ulcers, genital ulcers, skin lesions and ocular lesions are the most common manifestations. Inflammation is typically self-limiting in time and relapsing episodes of clinical manifestations represent a hallmark of Behçet’s disease. Other less frequent yet severe manifestations that have a major prognostic impact involve the eyes, the central nervous system, the main large vessels and the gastrointestinal tract. Behçet’s disease has a heterogeneous onset and is associated with significant morbidity and premature mortality. This study presents a current immunological review of the disease and provides a synopsis of clinical aspects and treatment options.
Anhänge
Nur mit Berechtigung zugänglich
Literatur
1.
Zurück zum Zitat Mendes D, Correia M, Barbedo M, Vaio T, Mota M, Gonçalves O, Valente J (2009) Behçet’s disease—a contemporary review. J Autoimmun 32(3–4):178–188CrossRefPubMed Mendes D, Correia M, Barbedo M, Vaio T, Mota M, Gonçalves O, Valente J (2009) Behçet’s disease—a contemporary review. J Autoimmun 32(3–4):178–188CrossRefPubMed
2.
Zurück zum Zitat Verity DH, Marr JE, Ohno S, Wallace GR, Stanford MR (1999) Behçet’s disease, the silk road and HLA-B51: historical and geographical perspectives. Tissue Antigens 54(3):213–220CrossRefPubMed Verity DH, Marr JE, Ohno S, Wallace GR, Stanford MR (1999) Behçet’s disease, the silk road and HLA-B51: historical and geographical perspectives. Tissue Antigens 54(3):213–220CrossRefPubMed
3.
Zurück zum Zitat Sfikakis PP, Markomichelakis N, Alpsoy E, Assaad-Khalil S, Bodaghi B, Gul A et al (2007) Anti-TNF therapy in the management of behçet’s disease-review and basis for recommendations. Rheumatology (Oxford) 46(5):736–741CrossRef Sfikakis PP, Markomichelakis N, Alpsoy E, Assaad-Khalil S, Bodaghi B, Gul A et al (2007) Anti-TNF therapy in the management of behçet’s disease-review and basis for recommendations. Rheumatology (Oxford) 46(5):736–741CrossRef
4.
Zurück zum Zitat Saadoun D, Wechsler B, Desseaux K, Le Thi Huong D, Amoura Z, Resche-Rigon M, Cacoub P (2010) Mortality in Behçet’s disease. Arthritis Rheum 62(9):2806–2812CrossRefPubMed Saadoun D, Wechsler B, Desseaux K, Le Thi Huong D, Amoura Z, Resche-Rigon M, Cacoub P (2010) Mortality in Behçet’s disease. Arthritis Rheum 62(9):2806–2812CrossRefPubMed
5.
6.
Zurück zum Zitat Sfikakis PP (2002) Behçet’s disease: a new target for anti-tumor necrosis factor treatment. Ann Rheum Dis 61(Suppl 2):51–53CrossRef Sfikakis PP (2002) Behçet’s disease: a new target for anti-tumor necrosis factor treatment. Ann Rheum Dis 61(Suppl 2):51–53CrossRef
7.
Zurück zum Zitat Yazici H, Akokan G, Yalçin B, Müftüoğlu A (1977) The high prevalence of HLA-B5 in Behçet’s disease. Clin Exp Immunol 30(2):259–261PubMedPubMedCentral Yazici H, Akokan G, Yalçin B, Müftüoğlu A (1977) The high prevalence of HLA-B5 in Behçet’s disease. Clin Exp Immunol 30(2):259–261PubMedPubMedCentral
8.
Zurück zum Zitat Lawton G, Bhakta BB, Chamberlain MA, Tennant A (2004) The Behçet’s disease activity index. Rheumatology (Oxford) 43(1):73–78CrossRef Lawton G, Bhakta BB, Chamberlain MA, Tennant A (2004) The Behçet’s disease activity index. Rheumatology (Oxford) 43(1):73–78CrossRef
9.
Zurück zum Zitat Savey L, Resche-Rigon M, Wechsler B, Comarmond C, Piette JC, Cacoub P, Saadoun D (2014) Ethnicity and association with disease manifestations and mortality in Behçet’s disease. Orphanet J Rare Dis 27(9):42CrossRef Savey L, Resche-Rigon M, Wechsler B, Comarmond C, Piette JC, Cacoub P, Saadoun D (2014) Ethnicity and association with disease manifestations and mortality in Behçet’s disease. Orphanet J Rare Dis 27(9):42CrossRef
10.
11.
Zurück zum Zitat Remmers EF, Cosan F, Kirino Y, Ombrello MJ, Abaci N, Satorius C et al (2010) Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behçet’s disease. Nat Genet 42(8):698–702CrossRefPubMedPubMedCentral Remmers EF, Cosan F, Kirino Y, Ombrello MJ, Abaci N, Satorius C et al (2010) Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behçet’s disease. Nat Genet 42(8):698–702CrossRefPubMedPubMedCentral
12.
Zurück zum Zitat Saadoun D, Wechsler B, Terrada C, Hajage D, Le Thi Huong D, Resche-Rigon M et al (2010) Azathioprine in severe uveitis of Behçet’s disease. Arthritis Care Res (Hoboken). 62(12):1733–1738CrossRefPubMed Saadoun D, Wechsler B, Terrada C, Hajage D, Le Thi Huong D, Resche-Rigon M et al (2010) Azathioprine in severe uveitis of Behçet’s disease. Arthritis Care Res (Hoboken). 62(12):1733–1738CrossRefPubMed
13.
Zurück zum Zitat Desbois AC, Wechsler B, Resche-Rigon M, Piette JC, Huong D Le T, Amoura Z et al (2012) Immunosuppressants reduce venous thrombosis relapse in Behçet’s disease. Arthritis Rheum 64(8):2753–2760CrossRefPubMed Desbois AC, Wechsler B, Resche-Rigon M, Piette JC, Huong D Le T, Amoura Z et al (2012) Immunosuppressants reduce venous thrombosis relapse in Behçet’s disease. Arthritis Rheum 64(8):2753–2760CrossRefPubMed
14.
Zurück zum Zitat Kump LI, Moeller KL, Reed GF, Kurup SK, Nussenblatt RB, Levy-Clarke GA (2008) Behçet’s disease: comparing 3 decades of treatment response at the national eye institute. Can J Ophthalmol 43(4):468–472CrossRefPubMedPubMedCentral Kump LI, Moeller KL, Reed GF, Kurup SK, Nussenblatt RB, Levy-Clarke GA (2008) Behçet’s disease: comparing 3 decades of treatment response at the national eye institute. Can J Ophthalmol 43(4):468–472CrossRefPubMedPubMedCentral
16.
Zurück zum Zitat Alpsoy E, Donmez L, Bacanli A, Apaydin C, Butun B (2003) Review of the chronology of clinical manifestations in 60 patients with Behçet’s disease. Dermatology 207(4):354–356CrossRefPubMed Alpsoy E, Donmez L, Bacanli A, Apaydin C, Butun B (2003) Review of the chronology of clinical manifestations in 60 patients with Behçet’s disease. Dermatology 207(4):354–356CrossRefPubMed
17.
Zurück zum Zitat Al-Araji A (2009) Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol 8(2):192CrossRefPubMed Al-Araji A (2009) Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol 8(2):192CrossRefPubMed
18.
Zurück zum Zitat Arai Y, Kohno S, Takahashi Y, Miyajima Y, Tsutusi Y (2006) Autopsy case of neuro-Behçet’s disease with multifocal neutrophilic perivascular inflammation. Neuropathology 26(6):579–585CrossRefPubMed Arai Y, Kohno S, Takahashi Y, Miyajima Y, Tsutusi Y (2006) Autopsy case of neuro-Behçet’s disease with multifocal neutrophilic perivascular inflammation. Neuropathology 26(6):579–585CrossRefPubMed
19.
Zurück zum Zitat Hadfield MG, Aydin A, Lippman HR, Sanders KM (1997) Neuro-Behçet’s disease. Clin Neuropathol 16(2):55–60PubMed Hadfield MG, Aydin A, Lippman HR, Sanders KM (1997) Neuro-Behçet’s disease. Clin Neuropathol 16(2):55–60PubMed
20.
Zurück zum Zitat Noel N, Bernard R, Wechsler B, Resche-Rigon M, Depaz R, Le Thi Huong Boutin D et al (2014) Long-term outcome of neuro-Behçet’s disease. Arthritis Rheum 66(5):1306–1314CrossRef Noel N, Bernard R, Wechsler B, Resche-Rigon M, Depaz R, Le Thi Huong Boutin D et al (2014) Long-term outcome of neuro-Behçet’s disease. Arthritis Rheum 66(5):1306–1314CrossRef
21.
Zurück zum Zitat Kidd D (2012) Neurological complications of Behçet’s syndrome. Curr Neurol Neurosc Rep 12(6):675–679CrossRef Kidd D (2012) Neurological complications of Behçet’s syndrome. Curr Neurol Neurosc Rep 12(6):675–679CrossRef
22.
Zurück zum Zitat Siva A, Kantarci OH, Saip S, Altintas A, Hamuryudan V, Islak C et al (2001) Behçet’s disease: diagnostic and prognostic aspects of neurological involvement. J Neurol 248(2):95–103CrossRefPubMed Siva A, Kantarci OH, Saip S, Altintas A, Hamuryudan V, Islak C et al (2001) Behçet’s disease: diagnostic and prognostic aspects of neurological involvement. J Neurol 248(2):95–103CrossRefPubMed
23.
Zurück zum Zitat Kikuchi H, Aramaki K, Hirohata S (2008) Effect of infliximab in progressive neuro-Behçet’s syndrome. J Neurol Sci 272(1):99–105CrossRefPubMed Kikuchi H, Aramaki K, Hirohata S (2008) Effect of infliximab in progressive neuro-Behçet’s syndrome. J Neurol Sci 272(1):99–105CrossRefPubMed
24.
Zurück zum Zitat Saadoun D, Wechsler B, Resche-Rigon M, Trad S, Le Thi Huong D, Sbai A et al (2009) Cerebral venous thrombosis in Behçet’s disease. Arthritis Rheum 61(4):518–526CrossRefPubMed Saadoun D, Wechsler B, Resche-Rigon M, Trad S, Le Thi Huong D, Sbai A et al (2009) Cerebral venous thrombosis in Behçet’s disease. Arthritis Rheum 61(4):518–526CrossRefPubMed
25.
Zurück zum Zitat Akman-Demir G (1999) Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. Brain 122(11):2171–2182CrossRefPubMed Akman-Demir G (1999) Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. Brain 122(11):2171–2182CrossRefPubMed
26.
Zurück zum Zitat Kizilkilic O, Albayram S, Adaletli I, Ak H, Islak C, Kocer N (2003) Endovascular treatment of Behçet’s disease-associated intracranial aneurysms: report of two cases and review of the literature. Neuroradiology 45(5):328–334CrossRefPubMed Kizilkilic O, Albayram S, Adaletli I, Ak H, Islak C, Kocer N (2003) Endovascular treatment of Behçet’s disease-associated intracranial aneurysms: report of two cases and review of the literature. Neuroradiology 45(5):328–334CrossRefPubMed
27.
Zurück zum Zitat Öktem-Tanör Ö, Baykan-Kurt B, Gürvit IH, Akman-Demir G, Serdaroğlu P (1999) Neuropsychological follow-up of 12 patients with neuro-Behçet’s disease. J Neurol 246(2):113–119CrossRefPubMed Öktem-Tanör Ö, Baykan-Kurt B, Gürvit IH, Akman-Demir G, Serdaroğlu P (1999) Neuropsychological follow-up of 12 patients with neuro-Behçet’s disease. J Neurol 246(2):113–119CrossRefPubMed
28.
Zurück zum Zitat Saadoun D, Asli B, Wechsler B, Houman H, Geri G, Desseaux K et al (2012) Long-term outcome of arterial lesions in Behçet’s disease: a series of 101 patients. Medicine (Baltimore) 91(1):18–24CrossRef Saadoun D, Asli B, Wechsler B, Houman H, Geri G, Desseaux K et al (2012) Long-term outcome of arterial lesions in Behçet’s disease: a series of 101 patients. Medicine (Baltimore) 91(1):18–24CrossRef
29.
Zurück zum Zitat Desbois A, Wechsler B, Cluzel P, Helft G, Boutin D, Piette JC et al (2014) Cardiovascular involvement in Behçet’s disease. Rev Med Int 35(2):103–111CrossRef Desbois A, Wechsler B, Cluzel P, Helft G, Boutin D, Piette JC et al (2014) Cardiovascular involvement in Behçet’s disease. Rev Med Int 35(2):103–111CrossRef
30.
Zurück zum Zitat Tursen U, Gurler A, Boyvat A (2003) Evaluation of clinical findings according to sex in 2313 turkish patients with Behçet’s disease. Int J Dermatol 42(5):346–351CrossRefPubMed Tursen U, Gurler A, Boyvat A (2003) Evaluation of clinical findings according to sex in 2313 turkish patients with Behçet’s disease. Int J Dermatol 42(5):346–351CrossRefPubMed
31.
Zurück zum Zitat Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V et al (2003) The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine 82(1):60–76CrossRefPubMed Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V et al (2003) The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine 82(1):60–76CrossRefPubMed
32.
Zurück zum Zitat Desbois A, Rautou P, Biard L, Belmatoug N, Wechsler B, Resche-Rigon M et al (2014) Behçet’s disease in Budd-Chiari syndrome. Orphanet J Rare Dis 9(1):104CrossRefPubMedPubMedCentral Desbois A, Rautou P, Biard L, Belmatoug N, Wechsler B, Resche-Rigon M et al (2014) Behçet’s disease in Budd-Chiari syndrome. Orphanet J Rare Dis 9(1):104CrossRefPubMedPubMedCentral
33.
Zurück zum Zitat Hamuryudan V, Yurdakul S, Moral F et al (1994) Pulmonary arterial aneurysms in behçet’s syndrome: a report of 24 cases. Br J Rheumatol 33(1):48–51CrossRefPubMed Hamuryudan V, Yurdakul S, Moral F et al (1994) Pulmonary arterial aneurysms in behçet’s syndrome: a report of 24 cases. Br J Rheumatol 33(1):48–51CrossRefPubMed
34.
Zurück zum Zitat Geri G, Terrier B, Rosenzwajg M, Wechsler B, Touzot M, Seilhean D et al (2011) Critical role of IL-21 in modulating Th17 and regulatory T cells in Behçet’s disease. J Allergy Clin Immunol 128(3):655CrossRefPubMed Geri G, Terrier B, Rosenzwajg M, Wechsler B, Touzot M, Seilhean D et al (2011) Critical role of IL-21 in modulating Th17 and regulatory T cells in Behçet’s disease. J Allergy Clin Immunol 128(3):655CrossRefPubMed
35.
Zurück zum Zitat Atzeni F, Sarzi-Puttini P, Doria A, Boiardi L, Pipitone N, Salvarani C (2005) Behçet’s disease and cardiovascular involvement. Lupus 14(9):723–726CrossRefPubMed Atzeni F, Sarzi-Puttini P, Doria A, Boiardi L, Pipitone N, Salvarani C (2005) Behçet’s disease and cardiovascular involvement. Lupus 14(9):723–726CrossRefPubMed
36.
Zurück zum Zitat Ait Badi MA, Zyani M, Kaddouri S, Niamane R, Hda A, Algayres J (2008) Les manifestations articulaires de la maladie de Behçet. À propos de 79 cas. Rev Med Int 29(4):277–282CrossRef Ait Badi MA, Zyani M, Kaddouri S, Niamane R, Hda A, Algayres J (2008) Les manifestations articulaires de la maladie de Behçet. À propos de 79 cas. Rev Med Int 29(4):277–282CrossRef
37.
Zurück zum Zitat Kaklamani VG, Vaiopoulos G, Kaklamanis PG (1998) Behçet’s disease. Sem Arthritis Rheum 27(4):197–217CrossRef Kaklamani VG, Vaiopoulos G, Kaklamanis PG (1998) Behçet’s disease. Sem Arthritis Rheum 27(4):197–217CrossRef
38.
Zurück zum Zitat Kastner DL. Intermittent and periodic arthritic syndromes. In: Koopman WJ (ed.) Arthritis and allied conditions: a textbook of rheumatology. Vol. 1. 13th ed. Baltimore: Williams and Wilkins; 1997:1279–1306 Kastner DL. Intermittent and periodic arthritic syndromes. In: Koopman WJ (ed.) Arthritis and allied conditions: a textbook of rheumatology. Vol. 1. 13th ed. Baltimore: Williams and Wilkins; 1997:1279–1306
39.
Zurück zum Zitat Beales IL (1998) Gastrointestinal involvement in Behçet’s syndrome. Am J Gastroenterol 93(12):2633CrossRefPubMed Beales IL (1998) Gastrointestinal involvement in Behçet’s syndrome. Am J Gastroenterol 93(12):2633CrossRefPubMed
40.
41.
Zurück zum Zitat Lee KS, Kim SJ, Lee BC, Yoon DS, Lee WJ, Chi HS (1997) Surgical treatment of intestinal Behçet’s disease. Yonsei Med J 38(6):455–460CrossRefPubMed Lee KS, Kim SJ, Lee BC, Yoon DS, Lee WJ, Chi HS (1997) Surgical treatment of intestinal Behçet’s disease. Yonsei Med J 38(6):455–460CrossRefPubMed
42.
Zurück zum Zitat Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F et al (2013) 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 65:1–11CrossRefPubMed Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F et al (2013) 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 65:1–11CrossRefPubMed
43.
Zurück zum Zitat Link J, Söderström M, Olsson T, Höjeberg B, Ljungdahl A, Link H (1994) Increased transforming growth factor-beta, interleukin-4, and interferon-gamma in multiple sclerosis. Ann Neurology 36(3):379–386CrossRef Link J, Söderström M, Olsson T, Höjeberg B, Ljungdahl A, Link H (1994) Increased transforming growth factor-beta, interleukin-4, and interferon-gamma in multiple sclerosis. Ann Neurology 36(3):379–386CrossRef
44.
Zurück zum Zitat Cho SB, Cho S, Bang D (2012) New insights in the clinical understanding of Behçet’s disease. Yonsei Med J 53(1):35–42CrossRefPubMed Cho SB, Cho S, Bang D (2012) New insights in the clinical understanding of Behçet’s disease. Yonsei Med J 53(1):35–42CrossRefPubMed
45.
Zurück zum Zitat International study group for Behçet’s disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet 335(8697):1078–1080 International study group for Behçet’s disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet 335(8697):1078–1080
46.
Zurück zum Zitat Türsen Ü (2009) Activation markers in Behçet disease. Turkderm 43:74–86 Türsen Ü (2009) Activation markers in Behçet disease. Turkderm 43:74–86
47.
Zurück zum Zitat Kawai T, Akira S (2010) The role of pattern-recognition receptors in innate immunity: update on Toll-like receptors. Nat Immunol 11:373–384CrossRefPubMed Kawai T, Akira S (2010) The role of pattern-recognition receptors in innate immunity: update on Toll-like receptors. Nat Immunol 11:373–384CrossRefPubMed
48.
Zurück zum Zitat de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A (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):1287–1296CrossRefPubMed de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A (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):1287–1296CrossRefPubMed
49.
Zurück zum Zitat Ohno S, Ohguchi M, Hirose S, Matsuda H, Wakisaka A, Aizawa M (1982) Close association of HLA-Bw51 with Behçet’s disease. Arch Ophthalmol 100(9):1455–1458CrossRefPubMed Ohno S, Ohguchi M, Hirose S, Matsuda H, Wakisaka A, Aizawa M (1982) Close association of HLA-Bw51 with Behçet’s disease. Arch Ophthalmol 100(9):1455–1458CrossRefPubMed
50.
Zurück zum Zitat Shahneh FZ, Hamzavi F, Bayazi B, Bandehagh A, Baradaran B (2013) New insights into HLA class I association to Behçet’s syndrome in Iranian Azari patients. Auto Immun Highlights 4(3):101–102CrossRefPubMedPubMedCentral Shahneh FZ, Hamzavi F, Bayazi B, Bandehagh A, Baradaran B (2013) New insights into HLA class I association to Behçet’s syndrome in Iranian Azari patients. Auto Immun Highlights 4(3):101–102CrossRefPubMedPubMedCentral
51.
Zurück zum Zitat Gül A (2014) Genetics of Behçetʼs disease: lessons learned from genomewide association studies. Curr Opin Rheumatol 26:56–63CrossRefPubMed Gül A (2014) Genetics of Behçetʼs disease: lessons learned from genomewide association studies. Curr Opin Rheumatol 26:56–63CrossRefPubMed
52.
53.
Zurück zum Zitat Ombrello MJ, Kirino Y, de Paul Bakker IW, Gül A, Kastner DL, Remmers EF (2014) Behçet disease-associated MHC class I residues implicate antigen binding and regulation of cell-mediated cytotoxicity. Proc Natl Acad Sci USA 111:8867–8872CrossRefPubMedPubMedCentral Ombrello MJ, Kirino Y, de Paul Bakker IW, Gül A, Kastner DL, Remmers EF (2014) Behçet disease-associated MHC class I residues implicate antigen binding and regulation of cell-mediated cytotoxicity. Proc Natl Acad Sci USA 111:8867–8872CrossRefPubMedPubMedCentral
54.
Zurück zum Zitat Kobayashi M, Ito M, Nakagawa A, Matsushita M, Nishikimi N, Sakurai T, Nimura Y (2000) Neutrophil and endothelial cell activation in the vasa vasorum in vasculo-Behçet disease. Histopathology 36(4):362–371CrossRefPubMed Kobayashi M, Ito M, Nakagawa A, Matsushita M, Nishikimi N, Sakurai T, Nimura Y (2000) Neutrophil and endothelial cell activation in the vasa vasorum in vasculo-Behçet disease. Histopathology 36(4):362–371CrossRefPubMed
55.
Zurück zum Zitat Lehner T (1997) The role of heat shock protein, microbial and autoimmune agents in the aetiology of Behçet’s disease. Int Rev Immunol 14(1):21–32CrossRefPubMed Lehner T (1997) The role of heat shock protein, microbial and autoimmune agents in the aetiology of Behçet’s disease. Int Rev Immunol 14(1):21–32CrossRefPubMed
56.
Zurück zum Zitat Consolandi C, Turroni S, Emmi G, Severgnini M, Fiori J, Peano C et al (2015) Behçet’s syndrome patients exhibit specific microbiome signature. Autoimmun Rev 14(4):269–276CrossRefPubMed Consolandi C, Turroni S, Emmi G, Severgnini M, Fiori J, Peano C et al (2015) Behçet’s syndrome patients exhibit specific microbiome signature. Autoimmun Rev 14(4):269–276CrossRefPubMed
57.
Zurück zum Zitat Hirohata S, Oka H, Mizushima Y (1992) Streptococcal-related antigens stimulate production of IL6 and interferon-γ by T cells from patients with Behçet’s disease. Cell Immunol 140(2):410–419CrossRefPubMed Hirohata S, Oka H, Mizushima Y (1992) Streptococcal-related antigens stimulate production of IL6 and interferon-γ by T cells from patients with Behçet’s disease. Cell Immunol 140(2):410–419CrossRefPubMed
58.
Zurück zum Zitat The Matzinger P, Model Danger (2002) A Renewed Sense of Self. Science. 296:301–305 The Matzinger P, Model Danger (2002) A Renewed Sense of Self. Science. 296:301–305
59.
Zurück zum Zitat Medzhitov R, Janeway CA (2002) Decoding the patterns of self and non self by the innate immune system. Science 296:298–300CrossRefPubMed Medzhitov R, Janeway CA (2002) Decoding the patterns of self and non self by the innate immune system. Science 296:298–300CrossRefPubMed
60.
Zurück zum Zitat Direskeneli H (2006) Autoimmunity vs autoinflammation in Behcet’s disease: do we oversimplify a complex disorder? Rheumatology (Oxford) 45:1461–1465CrossRef Direskeneli H (2006) Autoimmunity vs autoinflammation in Behcet’s disease: do we oversimplify a complex disorder? Rheumatology (Oxford) 45:1461–1465CrossRef
62.
Zurück zum Zitat Hirohata S (2008) Histopathology of central nervous system lesions in Behçet’s disease. J Neurol Sci 267(1):41–47CrossRefPubMed Hirohata S (2008) Histopathology of central nervous system lesions in Behçet’s disease. J Neurol Sci 267(1):41–47CrossRefPubMed
63.
Zurück zum Zitat Kidd G, Kivisäkk P, Ransohoff RM (2003) Three or more routes for leukocyte migration into the central nervous system. Nat Rev Immunol 3(7):569–581CrossRefPubMed Kidd G, Kivisäkk P, Ransohoff RM (2003) Three or more routes for leukocyte migration into the central nervous system. Nat Rev Immunol 3(7):569–581CrossRefPubMed
64.
Zurück zum Zitat Ozaki K, Spolski R, Ettinger R, Kim HP, Wang G, Qi CF et al (2004) Regulation of B cell differentiation and plasma cell generation by IL-21, a novel inducer of blimp-1 and bcl-6. J Immunol 173(9):5361CrossRefPubMed Ozaki K, Spolski R, Ettinger R, Kim HP, Wang G, Qi CF et al (2004) Regulation of B cell differentiation and plasma cell generation by IL-21, a novel inducer of blimp-1 and bcl-6. J Immunol 173(9):5361CrossRefPubMed
65.
Zurück zum Zitat Tzartos JS, Friese MA, Craner MJ, Palace J, Newcombe J, Esiri MM, Fugger L (2008) Interleukin-17 production in central nervous system-infiltrating T cells and glial cells is associated with active disease in multiple sclerosis. Am J Pathol 172(1):146–155CrossRefPubMedPubMedCentral Tzartos JS, Friese MA, Craner MJ, Palace J, Newcombe J, Esiri MM, Fugger L (2008) Interleukin-17 production in central nervous system-infiltrating T cells and glial cells is associated with active disease in multiple sclerosis. Am J Pathol 172(1):146–155CrossRefPubMedPubMedCentral
66.
Zurück zum Zitat Anderson MS, Bluestone JA (2005) The NOD mouse: a model of immune dysregulation. Annu Rev Immunol 23(1):447–485CrossRefPubMed Anderson MS, Bluestone JA (2005) The NOD mouse: a model of immune dysregulation. Annu Rev Immunol 23(1):447–485CrossRefPubMed
67.
Zurück zum Zitat Bucher C, Koch L, Vogtenhuber C, Goren E, Munger M, Panoskaltsis-Mortari A et al (2009) IL-21 blockade reduces graft-versus-host-disease mortality by supporting inducible T regulatory cell generation. Blood 114(26):5375–5384CrossRefPubMedPubMedCentral Bucher C, Koch L, Vogtenhuber C, Goren E, Munger M, Panoskaltsis-Mortari A et al (2009) IL-21 blockade reduces graft-versus-host-disease mortality by supporting inducible T regulatory cell generation. Blood 114(26):5375–5384CrossRefPubMedPubMedCentral
68.
Zurück zum Zitat O’Garra A, Rouleau M, Antonenko S, Rouleau M, Antonenko S, de Vries JE, Roncarolo MG (1997) A CD4+ T-cell subset inhibits antigen-specific T-cell responses and prevents colitis. Nature 389(6652):737–742CrossRefPubMed O’Garra A, Rouleau M, Antonenko S, Rouleau M, Antonenko S, de Vries JE, Roncarolo MG (1997) A CD4+ T-cell subset inhibits antigen-specific T-cell responses and prevents colitis. Nature 389(6652):737–742CrossRefPubMed
69.
Zurück zum Zitat Laurence A, Tato CM, Davidson TS, Kanno Y, Chen Z, Yao Z, Blank RB et al (2007) Interleukin-2 signaling via STAT5 constrains T helper 17 cell generation. Immunity 26(3):371–381CrossRefPubMed Laurence A, Tato CM, Davidson TS, Kanno Y, Chen Z, Yao Z, Blank RB et al (2007) Interleukin-2 signaling via STAT5 constrains T helper 17 cell generation. Immunity 26(3):371–381CrossRefPubMed
70.
Zurück zum Zitat Weaver CT, Hatton RD, Mangan PR, Harrington LE (2007) IL-17 family cytokines and the expanding diversity of effector T cell lineages. Annu Rev Immunol 25(1):821–852CrossRefPubMed Weaver CT, Hatton RD, Mangan PR, Harrington LE (2007) IL-17 family cytokines and the expanding diversity of effector T cell lineages. Annu Rev Immunol 25(1):821–852CrossRefPubMed
71.
Zurück zum Zitat Yang XO, Panopoulos AD, Nurieva R, Chang SH, Wang D, Watowich SS, Dong C (2007) STAT3 regulates cytokine-mediated generation of inflammatory helper T cells. J Biol Chem 282(13):9358–9363CrossRefPubMed Yang XO, Panopoulos AD, Nurieva R, Chang SH, Wang D, Watowich SS, Dong C (2007) STAT3 regulates cytokine-mediated generation of inflammatory helper T cells. J Biol Chem 282(13):9358–9363CrossRefPubMed
72.
Zurück zum Zitat Lee JW, Wang P, Kattah MG, Youssef S, Steinman L, DeFea K, Straus DS (2008) Differential regulation of chemokines by IL-17 in colonic epithelial cells. J Immunol 181(9):6536CrossRefPubMed Lee JW, Wang P, Kattah MG, Youssef S, Steinman L, DeFea K, Straus DS (2008) Differential regulation of chemokines by IL-17 in colonic epithelial cells. J Immunol 181(9):6536CrossRefPubMed
73.
Zurück zum Zitat Eastaff-Leung N, Mabarrack N, Barbour A, Cummins A, Barry S. Foxp3+ regulatory T cells, Th17 effector cells, and cytokine environment in inflammatory bowel disease. J Clin Immunol. 2010; 2009;30(1):80–9 Eastaff-Leung N, Mabarrack N, Barbour A, Cummins A, Barry S. Foxp3+ regulatory T cells, Th17 effector cells, and cytokine environment in inflammatory bowel disease. J Clin Immunol. 2010; 2009;30(1):80–9
74.
Zurück zum Zitat de Pineton Chambrun M, Wechsler B, Geri G, Cacoub P, Saadoun D (2012) New insights into the pathogenesis of Behçet’s disease. Autoimmun Rev 11(10):687–698CrossRef de Pineton Chambrun M, Wechsler B, Geri G, Cacoub P, Saadoun D (2012) New insights into the pathogenesis of Behçet’s disease. Autoimmun Rev 11(10):687–698CrossRef
75.
76.
77.
Zurück zum Zitat Kebir H, Kreymborg K, Ifergan I, Dodelet-Devillers A, Cayrol R, Bernard M et al (2007) Human Th17 lymphocytes promote blood-brain barrier disruption and central nervous system inflammation. Nat Med 13(10):1173–1175CrossRefPubMedPubMedCentral Kebir H, Kreymborg K, Ifergan I, Dodelet-Devillers A, Cayrol R, Bernard M et al (2007) Human Th17 lymphocytes promote blood-brain barrier disruption and central nervous system inflammation. Nat Med 13(10):1173–1175CrossRefPubMedPubMedCentral
78.
Zurück zum Zitat Saadoun D, Rosenzwajg M, Joly F, Six A, Carrat F, Thibault V et al (2011) Regulatory T-cell responses to low-dose interleukin-2 in HCV-induced vasculitis. N Engl J Med 365:2067–2077CrossRefPubMed Saadoun D, Rosenzwajg M, Joly F, Six A, Carrat F, Thibault V et al (2011) Regulatory T-cell responses to low-dose interleukin-2 in HCV-induced vasculitis. N Engl J Med 365:2067–2077CrossRefPubMed
79.
Zurück zum Zitat Nurieva R, Yang XO, Martinez G, Zhang Y, Panopoulos AD, Ma L, Schluns K et al (2007) Essential autocrine regulation by IL-21 in the generation of inflammatory T cells. Nature 448(7152):480–483CrossRefPubMed Nurieva R, Yang XO, Martinez G, Zhang Y, Panopoulos AD, Ma L, Schluns K et al (2007) Essential autocrine regulation by IL-21 in the generation of inflammatory T cells. Nature 448(7152):480–483CrossRefPubMed
80.
Zurück zum Zitat Vollmer TL, Liu R, Price M, Rhodes S, La Cava A, Shi F (2005) Differential effects of IL-21 during initiation and progression of autoimmunity against neuroantigen. J Immunol 174(5):2696–2701CrossRefPubMed Vollmer TL, Liu R, Price M, Rhodes S, La Cava A, Shi F (2005) Differential effects of IL-21 during initiation and progression of autoimmunity against neuroantigen. J Immunol 174(5):2696–2701CrossRefPubMed
81.
Zurück zum Zitat Brustle A, Heink S, Huber M, Rosenplänter C, Stadelmann C, Yu P et al (2007) The development of inflammatory Th-17 cells requires interferon-regulatory factor 4. Nat Immunol 8(9):958–966CrossRefPubMed Brustle A, Heink S, Huber M, Rosenplänter C, Stadelmann C, Yu P et al (2007) The development of inflammatory Th-17 cells requires interferon-regulatory factor 4. Nat Immunol 8(9):958–966CrossRefPubMed
82.
Zurück zum Zitat Miyara M, Yoshioka Y, Kitoh A, Shima T, Wing K, Niwa A et al (2009) Functional delineation and differentiation dynamics of human CD4+ T cells expressing the FoxP3 transcription factor. Immunity 30(6):899–911CrossRefPubMed Miyara M, Yoshioka Y, Kitoh A, Shima T, Wing K, Niwa A et al (2009) Functional delineation and differentiation dynamics of human CD4+ T cells expressing the FoxP3 transcription factor. Immunity 30(6):899–911CrossRefPubMed
83.
Zurück zum Zitat Bottinelli D, Uccelli A, Engelhardt B, Benvenuto F, Bottinelli D, Lira S et al (2009) C-C chemokine receptor 6-regulated entry of T H-17 cells into the CNS through the choroid plexus is required for the initiation of EAE. Nat Immunol 10(5):514–523CrossRefPubMed Bottinelli D, Uccelli A, Engelhardt B, Benvenuto F, Bottinelli D, Lira S et al (2009) C-C chemokine receptor 6-regulated entry of T H-17 cells into the CNS through the choroid plexus is required for the initiation of EAE. Nat Immunol 10(5):514–523CrossRefPubMed
84.
Zurück zum Zitat Engelhardt B, Wolburg-Buchholz K, Wolburg H (2001) Involvement of the choroid plexus in central nervous system inflammation. Micros Res Tech 52(1):112–129CrossRef Engelhardt B, Wolburg-Buchholz K, Wolburg H (2001) Involvement of the choroid plexus in central nervous system inflammation. Micros Res Tech 52(1):112–129CrossRef
85.
Zurück zum Zitat Chen Q, Yang W, Gupta S, Biswas P, Smith P, Bhagat G, Pernis AB (2008) IRF-4-binding protein inhibits interleukin-17 and interleukin-21 production by controlling the activity of IRF-4 transcription factor. Immunity 29(6):899–911CrossRefPubMedPubMedCentral Chen Q, Yang W, Gupta S, Biswas P, Smith P, Bhagat G, Pernis AB (2008) IRF-4-binding protein inhibits interleukin-17 and interleukin-21 production by controlling the activity of IRF-4 transcription factor. Immunity 29(6):899–911CrossRefPubMedPubMedCentral
86.
Zurück zum Zitat Fantini MC, Rizzo A, Fina D, Caruso R, Becker C, Neurath MF et al (2007) IL-21 regulates experimental colitis by modulating the balance between Treg and Th17 cells. Eur J Immunol 37(11):3155–3163CrossRefPubMed Fantini MC, Rizzo A, Fina D, Caruso R, Becker C, Neurath MF et al (2007) IL-21 regulates experimental colitis by modulating the balance between Treg and Th17 cells. Eur J Immunol 37(11):3155–3163CrossRefPubMed
87.
Zurück zum Zitat Matusevicius D, Kivisäkk P, He B, Kostulas N, Ozenci V, Fredrikson S, Link H (1999) Interleukin-17 mRNA expression in blood and CSF mononuclear cells is augmented in multiple sclerosis. Mult Scler 5(2):101–104CrossRefPubMed Matusevicius D, Kivisäkk P, He B, Kostulas N, Ozenci V, Fredrikson S, Link H (1999) Interleukin-17 mRNA expression in blood and CSF mononuclear cells is augmented in multiple sclerosis. Mult Scler 5(2):101–104CrossRefPubMed
88.
Zurück zum Zitat Dagur PK, Biancotto A, Stansky E, Sen HN, Nussenblatt RB, McCoy JP (2014) Secretion of interleukin-17 by CD8+ T cells expressing CD146 (MCAM). Clin Immunol 152:36–47CrossRefPubMedPubMedCentral Dagur PK, Biancotto A, Stansky E, Sen HN, Nussenblatt RB, McCoy JP (2014) Secretion of interleukin-17 by CD8+ T cells expressing CD146 (MCAM). Clin Immunol 152:36–47CrossRefPubMedPubMedCentral
89.
Zurück zum Zitat Ivanov II, McKenzie BS, Zhou L, Tadokoro CE, Lepelley A, Lafaille JJ et al (2006) The orphan nuclear receptor RORgammat directs the differentiation program of pro-inflammatory IL-17+ T helper cells. Cell 126(6):1121CrossRefPubMed Ivanov II, McKenzie BS, Zhou L, Tadokoro CE, Lepelley A, Lafaille JJ et al (2006) The orphan nuclear receptor RORgammat directs the differentiation program of pro-inflammatory IL-17+ T helper cells. Cell 126(6):1121CrossRefPubMed
90.
Zurück zum Zitat Seder RA, Paul WE, Davis MM, de Fazekas St Groth B (1992) The presence of interleukin 4 during in vitro priming determines the lymphokine-producing potential of CD4+ T cells from T cell receptor transgenic mice. J Exp Med 176(4):1091–1098CrossRefPubMed Seder RA, Paul WE, Davis MM, de Fazekas St Groth B (1992) The presence of interleukin 4 during in vitro priming determines the lymphokine-producing potential of CD4+ T cells from T cell receptor transgenic mice. J Exp Med 176(4):1091–1098CrossRefPubMed
91.
Zurück zum Zitat Wu X, Tan Y, Xing Q, Wang S (2013) IL-21 accelerates xenogeneic graft-versus-host disease correlated with increased B-cell proliferation. Protein Cell 4(11):863–871CrossRefPubMedPubMedCentral Wu X, Tan Y, Xing Q, Wang S (2013) IL-21 accelerates xenogeneic graft-versus-host disease correlated with increased B-cell proliferation. Protein Cell 4(11):863–871CrossRefPubMedPubMedCentral
92.
Zurück zum Zitat Habib T, Senadheera S, Weinberg K, Kaushansky K (2002) The common gamma chain (gamma c) is a required signaling component of the IL-21 receptor and supports IL-21-induced cell proliferation via JAK3. Biochemistry 41(27):8725CrossRefPubMed Habib T, Senadheera S, Weinberg K, Kaushansky K (2002) The common gamma chain (gamma c) is a required signaling component of the IL-21 receptor and supports IL-21-induced cell proliferation via JAK3. Biochemistry 41(27):8725CrossRefPubMed
93.
Zurück zum Zitat Touzot M, Cacoub P, Bodaghi B, Soumelis V, Saadoun D (2015) IFN-α induces IL-10 production and tilt the balance between Th1 and Th17 in Behçet disease. Autoimmun Rev 14(5):370–375CrossRefPubMed Touzot M, Cacoub P, Bodaghi B, Soumelis V, Saadoun D (2015) IFN-α induces IL-10 production and tilt the balance between Th1 and Th17 in Behçet disease. Autoimmun Rev 14(5):370–375CrossRefPubMed
94.
Zurück zum Zitat Comarmond C, Wechsler B, Bodaghi B, Cacoub P, Saadoun D (2014) Biotherapies in Behçet’s disease. Autoimmun Rev 13(7):762–769CrossRefPubMed Comarmond C, Wechsler B, Bodaghi B, Cacoub P, Saadoun D (2014) Biotherapies in Behçet’s disease. Autoimmun Rev 13(7):762–769CrossRefPubMed
95.
Zurück zum Zitat Takamoto M, Kaburaki T, Numaga J, Fujino Y, Kawashima H (2007) Long-term infliximab treatment for behçet’s disease. Jpn J Ophthalmol 51(3):239–240CrossRefPubMed Takamoto M, Kaburaki T, Numaga J, Fujino Y, Kawashima H (2007) Long-term infliximab treatment for behçet’s disease. Jpn J Ophthalmol 51(3):239–240CrossRefPubMed
96.
Zurück zum Zitat Sbaï A, Wechsler B, Duhaut P, Du-Boutin LT, Amoura Z, Cacoub P et al (2003) Neuro-Behçet’s disease (isolated cerebral thrombophlebitis excluded). Clinical pattern, prognostic factors, treatment and long term follow-up. Adv Exp Med Biol 528:371–376CrossRefPubMed Sbaï A, Wechsler B, Duhaut P, Du-Boutin LT, Amoura Z, Cacoub P et al (2003) Neuro-Behçet’s disease (isolated cerebral thrombophlebitis excluded). Clinical pattern, prognostic factors, treatment and long term follow-up. Adv Exp Med Biol 528:371–376CrossRefPubMed
97.
Zurück zum Zitat Wechsler B, Lê Thi Huong DB, Saadoun D (2009) EULAR recommendations for the management of Behçet’s disease: evidence-based or experience-based medicine. Rev Med Interne 30(11):939–941CrossRefPubMed Wechsler B, Lê Thi Huong DB, Saadoun D (2009) EULAR recommendations for the management of Behçet’s disease: evidence-based or experience-based medicine. Rev Med Interne 30(11):939–941CrossRefPubMed
98.
Zurück zum Zitat Hatemi G, Melikoglu M, Tunc R et al (2015) Apremilast for Behçet’s syndrome–a phase 2, placebo-controlled study. N Engl J Med 372:1510CrossRefPubMed Hatemi G, Melikoglu M, Tunc R et al (2015) Apremilast for Behçet’s syndrome–a phase 2, placebo-controlled study. N Engl J Med 372:1510CrossRefPubMed
Metadaten
Titel
Behçet’s disease physiopathology: a contemporary review
verfasst von
Mohamad J. Zeidan
David Saadoun
Marlene Garrido
David Klatzmann
Adrien Six
Patrice Cacoub
Publikationsdatum
01.12.2016
Verlag
Springer International Publishing
Erschienen in
Autoimmunity Highlights / Ausgabe 1/2016
Print ISSN: 2038-0305
Elektronische ISSN: 2038-3274
DOI
https://doi.org/10.1007/s13317-016-0074-1

Weitere Artikel der Ausgabe 1/2016

Autoimmunity Highlights 1/2016 Zur Ausgabe

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.