白塞病43例临床分析
摘要
目的:总结和分析白塞病的临床及治疗特点,以加强对白塞病的认识,提高临床诊治水平。
方法:分析43例白塞病患者初发部位、临床表现、实验室和组织病理检查、治疗及疗效的情况。
结果:本组白塞病以女性、慢性型多见,好发于青壮年。9例急性型发病诱因以上呼吸道感染为主,应注意病毒或细菌感染在急性型发病机制中的作用。表现为口腔溃疡有41例(95.35%),以其为首发症状的有37例(90.24%)。表现为生殖器溃疡有19例(44.19%),眼部损害有15例(34.88%),针刺反应阳性有20例(46.51%),皮肤损害以结节性红斑最多见17例(39.53%),关节受累11例(25.58%)。除此外,白塞病尚可累及消化道和神经系统等。实验室检查无特异性,病理检查符合血管炎改变。应用糖皮质激素、免疫抑制剂或中西医结合治疗,病情得到控制或缓解。
结论:白塞病是累及全身多系统器官以血管炎改变为基础的免疫性疾病。诊断主要依靠临床症状,反复口腔溃疡是诊断白塞病的主要依据,针刺反应有辅助诊断意义。白塞病治疗目前尚无统一方法,糖皮质激素、免疫抑制剂、中西医结合治疗能很好地控制病情。
Objective: To improve the diagnosis and treatment of Behcet’s disease via analyzing its clinical manifestations and treatment outcomes.
Methods: The clinical manifestations,laboratory,pathological results, treatments and curative effects were analyzed in 43 patients with Behcet’s disease.
Results: Most cases in this group were middle-aged females with chronic onset.Nine patients with acute onset were predisposed by upper respiretory infection.The role of virus or bacterial infection in the pathogenesis of acute stage should be emphasized.Oral ulcer was found in 41 cases (95.35%),which was the initial clinical sign in 37 cases (90.24%).Genital ulcer was found in 19 cases (44.19%),while ocular involvement in 15 cases (34.88%).20 cases (46.51%) suffered form needling reaction.Erythema nodosum were seen in 17 cases (39.53%) and arthritis were seen in 11 cases (25.58%).Some patients were complicated with alimentary tract and neurologic manifestations.Laboratory examinations were no specificity. Histopathological examinations of skin revea- led the changes in vasculitis. All of the patients had idea response to glucocor- ticoids,immunosuppressive agents or combined therapies.
Conclusion: Behcet’s disease is an autoimmunologic disorder charact- erized with vasculitis which can involve multisysterm.The diagnose depen- ds mainly on clinical symptoms.Repeated oral ulcer is the main diagnosis evidence,and needling reaction assist the diagnose.At present, although a consensus on treatment of Behcet’s disease has not been reached,it is wide- ly accepted that glucocorticoids and immunosuppressive agents have good curative effects.
方法:分析43例白塞病患者初发部位、临床表现、实验室和组织病理检查、治疗及疗效的情况。
结果:本组白塞病以女性、慢性型多见,好发于青壮年。9例急性型发病诱因以上呼吸道感染为主,应注意病毒或细菌感染在急性型发病机制中的作用。表现为口腔溃疡有41例(95.35%),以其为首发症状的有37例(90.24%)。表现为生殖器溃疡有19例(44.19%),眼部损害有15例(34.88%),针刺反应阳性有20例(46.51%),皮肤损害以结节性红斑最多见17例(39.53%),关节受累11例(25.58%)。除此外,白塞病尚可累及消化道和神经系统等。实验室检查无特异性,病理检查符合血管炎改变。应用糖皮质激素、免疫抑制剂或中西医结合治疗,病情得到控制或缓解。
结论:白塞病是累及全身多系统器官以血管炎改变为基础的免疫性疾病。诊断主要依靠临床症状,反复口腔溃疡是诊断白塞病的主要依据,针刺反应有辅助诊断意义。白塞病治疗目前尚无统一方法,糖皮质激素、免疫抑制剂、中西医结合治疗能很好地控制病情。
Objective: To improve the diagnosis and treatment of Behcet’s disease via analyzing its clinical manifestations and treatment outcomes.
Methods: The clinical manifestations,laboratory,pathological results, treatments and curative effects were analyzed in 43 patients with Behcet’s disease.
Results: Most cases in this group were middle-aged females with chronic onset.Nine patients with acute onset were predisposed by upper respiretory infection.The role of virus or bacterial infection in the pathogenesis of acute stage should be emphasized.Oral ulcer was found in 41 cases (95.35%),which was the initial clinical sign in 37 cases (90.24%).Genital ulcer was found in 19 cases (44.19%),while ocular involvement in 15 cases (34.88%).20 cases (46.51%) suffered form needling reaction.Erythema nodosum were seen in 17 cases (39.53%) and arthritis were seen in 11 cases (25.58%).Some patients were complicated with alimentary tract and neurologic manifestations.Laboratory examinations were no specificity. Histopathological examinations of skin revea- led the changes in vasculitis. All of the patients had idea response to glucocor- ticoids,immunosuppressive agents or combined therapies.
Conclusion: Behcet’s disease is an autoimmunologic disorder charact- erized with vasculitis which can involve multisysterm.The diagnose depen- ds mainly on clinical symptoms.Repeated oral ulcer is the main diagnosis evidence,and needling reaction assist the diagnose.At present, although a consensus on treatment of Behcet’s disease has not been reached,it is wide- ly accepted that glucocorticoids and immunosuppressive agents have good curative effects.
引文
1.侯小荫,张卓莉,董怡.中国白塞病患者的临床特点.中华全科医师杂志,2005,4(11):666-667
2.张卓莉,彭劲民,侯小萌,等.1996例白塞病患者的临床荟萃分析.北京医学,2007,29(1):10-12
3. Niedzielska A,Chelminska K,Jaremin B.Behcet’s disease diagnostic difficulties [J].Pol Arch Med Wewn,2007,117(9):427-429
4. Salas Cabrera R,Sague Larrea J,Laurencio Mena A.Behcet’s disease [J].Case Re- port Arch Esp Urol,2007,60(1):67-68
5. Ahn JK,Park YG.Human leukocyte antigen B27 and B5l double-positive Behcet uveitis[J].Arch Ophthalmol,2007,l25(10):l375-l380
6. Gul A,Hajeer AH,Worthington J,et a1.Evidence for linkage of the HLA-B locus in Behcet’s disease obtained using transmission disequlibrium tests.Arthritis Rheum, 2001,44(1):239-240
7.周航,马烈,马智鸿,等.白塞病血清中免疫指标分析.中国麻风皮肤病杂志,2002,18(4):370-371
8. Lenher T.Immunopathogenesis of Bechet’s syndrome.Ann Med Interne(Paris), 1999,150:482-487
9.徐春兴.白塞病患者外周血T淋巴细胞亚群变化研究.中国麻风皮肤病杂志,2007,23(7):599-600
10.曹庆科,于志湖.白塞病患者外周血CD4+CD25+调节性T细胞的检测及其临床意义.中国麻风皮肤病杂志,2007,23(5):376-378
11. Mizuki N,Ota M,Katsuyama Y,et a1.HLA class I genotyping including HLA-B 5l allele typing in the Iranian patients with Behcet’s disease.Tissue Antigens,2001, 57(5):457-462
12.王培光,张学军.白塞病的遗传学与免疫学研究进展.国外医学遗传学分册,2003,26(2):116-118
13. Mizuki N,Ota M,Katsuyama Y,et a1.Association analysis between the MIC-A and HLA-B alleles in Japanese patients with Behcet’s disease.Arthritis Rheum,1999, 42(9):1961-1966
14. Wallace GR,Verity DH,Delamaine LJ,et a1.MIC-A allele profiles and HLA class Ⅰassociations in Behcet’s disease.Immunogenetics,1999,49(7-8):613-617
15. Cohen R,Metzger S,Nahir M,et a1.Association of the MIC-A gene and HLA-B51with Behcet’s disease in Arabs and non-Ashkenazi Jews in Israel.Ann Rheum Dis, 2002,61(2):157-160
16. Amoura Z,Guillaume M,Caillat-Zucman S,et a1.Pathophysiology of Behcet’s disease[J].Rev Med Interne,2006,27(11):843-853
17. Sakane T,Takeno M.Current therapy in Behcet’s disease.Skin Therapy Lett,2000, 5(6):3-5
18. Kattsambis AD.European Handbook of Dermatological Treatment.Berlin Springer, 1999,21-30
19. Yazici H,Fresko I,Yurdakul S.Behcet’s syndrome:disease manifestations,mana- gement,and advances in treatment.[J]Nat Clin Pract Rheumatol,2007,3(3):148- 155
20. Evereklioglu C.The treatment schedule and historical naming process of Behcet disease.[J]Eur Acad Dermatol Venereol,2007,21(3):427-428
21. Pipitone N,Olivieri I,Cantini F,et a1.New approaches in the treatment of Adaman- tiades-Behcet’s disease.[J]Curr Opin Rheumato1,2006,l8(1):3-9
22. Sakane T,Takeno M,Suzuki N,et a1.Behcet’s disease.N Engl J Med,1999,341(17): 1284-1291
23. Guillaume-Czitrom S,Berger C,Pajot C,et al.Efficacy and safety of interferon alpha in the treatment of corticodependent uveitis of paediatric Behcet’s disease. [J]Rheumatology (Oxford),2007,46(10):1570-1573
24. Takamoto M,Kaburaki T,Numaga J,et al.Long-term infliximab treatment for Beh- cet’s disease.[J]Jpn J Ophthalmol,2007,51(3):239-240
25. Mydlarski PR,Mittmunn N,Shear NH.Intravenous immunoglobulin:use in derma- tology.Skin Therapy Lett,2004,9:1-6
26. Hamuryudan V,Mat C,Saip S,et a1.Thalidomide in the treatment of the mucocut- aneous lesions of the Behcet syndrome.A randomized,double-blind, placebo-con- trolled tria1.Ann Intern Med,1998,128(6):443-450
27.马百芳,梁玉华,孟克,等.复方甘草酸苷治疗Behcet病临床观察.中国麻风皮肤病杂志,2007,23(3):219-220
28. Kanekura T,Gushi A,Iwata M,et al.Treatment of behcet’s disease with granulocyte and monocyte adsorption apheresis.[J]AM ACAD Dermatol,2004,51(2):83-87
29.王冬英.中西医结合治疗白塞病50例.现代中西医结合杂志,2008,17(5):692
30.陈岚,叶岚,陈兴平,等.58例白塞病回顾性分析.中国麻风皮肤病杂志,2003,19(1):25-27
1.赵辨.临床皮肤病学[M].第3版.南京:江苏科学技术出版社,2001,867
2. Sakane T,Takeno M,Suzuki N,et al.Behcet’s disease.N Engl J Med,1999,341: 1284-1291
3.曹光玲,凌红忠,李欢,等.白塞病64例临床分析.岭南皮肤性病科杂志,2001,8(3):134-136
4.陈岚,叶岚,陈兴平,等.58例白塞病回顾性分析.中国麻风皮肤病杂志,2003,1(1):25-27
5.赵辨.临床皮肤病学[M].第3版.南京:江苏科学技术出版社,2001,865
6.苏彦果,台立稳,董瑞芳.神经白塞氏病2例报告.脑与神经疾病杂志,2007,15(6):465-466
7.郭立琳,刘永太,田庄,等.白塞病合并心脏血栓4例临床分析.北京医学,2008,30(6):342-344
8.刘永太,田庄,方理刚,等.白塞病血管受累的临床分析.北京医学,2007,29(8):452-454
9. Frassnito MA,Dammacco R,Cafforio P,et al.Th1 polarization of the immune response in Behcet’s disease:a putative pathogenic role interleukin-12.Arthritis Rheum,1999,26:510-512
10. Zouboulis CC,Drtanous CE.Treatment of Adamantiades Behcet’s disease with systemic interferon alfa.Arch Dermatol,1998,134:1010
11. Hamuryudan V,Mat C,Saip S,et a1.Thalidomide in the treatment of the mucocu- taneous lesions of the Behcet syndrome.A randomized,double-blind, placebo- controlled tria1.Ann Intern Med,1998,128(6):443-450
2.张卓莉,彭劲民,侯小萌,等.1996例白塞病患者的临床荟萃分析.北京医学,2007,29(1):10-12
3. Niedzielska A,Chelminska K,Jaremin B.Behcet’s disease diagnostic difficulties [J].Pol Arch Med Wewn,2007,117(9):427-429
4. Salas Cabrera R,Sague Larrea J,Laurencio Mena A.Behcet’s disease [J].Case Re- port Arch Esp Urol,2007,60(1):67-68
5. Ahn JK,Park YG.Human leukocyte antigen B27 and B5l double-positive Behcet uveitis[J].Arch Ophthalmol,2007,l25(10):l375-l380
6. Gul A,Hajeer AH,Worthington J,et a1.Evidence for linkage of the HLA-B locus in Behcet’s disease obtained using transmission disequlibrium tests.Arthritis Rheum, 2001,44(1):239-240
7.周航,马烈,马智鸿,等.白塞病血清中免疫指标分析.中国麻风皮肤病杂志,2002,18(4):370-371
8. Lenher T.Immunopathogenesis of Bechet’s syndrome.Ann Med Interne(Paris), 1999,150:482-487
9.徐春兴.白塞病患者外周血T淋巴细胞亚群变化研究.中国麻风皮肤病杂志,2007,23(7):599-600
10.曹庆科,于志湖.白塞病患者外周血CD4+CD25+调节性T细胞的检测及其临床意义.中国麻风皮肤病杂志,2007,23(5):376-378
11. Mizuki N,Ota M,Katsuyama Y,et a1.HLA class I genotyping including HLA-B 5l allele typing in the Iranian patients with Behcet’s disease.Tissue Antigens,2001, 57(5):457-462
12.王培光,张学军.白塞病的遗传学与免疫学研究进展.国外医学遗传学分册,2003,26(2):116-118
13. Mizuki N,Ota M,Katsuyama Y,et a1.Association analysis between the MIC-A and HLA-B alleles in Japanese patients with Behcet’s disease.Arthritis Rheum,1999, 42(9):1961-1966
14. Wallace GR,Verity DH,Delamaine LJ,et a1.MIC-A allele profiles and HLA class Ⅰassociations in Behcet’s disease.Immunogenetics,1999,49(7-8):613-617
15. Cohen R,Metzger S,Nahir M,et a1.Association of the MIC-A gene and HLA-B51with Behcet’s disease in Arabs and non-Ashkenazi Jews in Israel.Ann Rheum Dis, 2002,61(2):157-160
16. Amoura Z,Guillaume M,Caillat-Zucman S,et a1.Pathophysiology of Behcet’s disease[J].Rev Med Interne,2006,27(11):843-853
17. Sakane T,Takeno M.Current therapy in Behcet’s disease.Skin Therapy Lett,2000, 5(6):3-5
18. Kattsambis AD.European Handbook of Dermatological Treatment.Berlin Springer, 1999,21-30
19. Yazici H,Fresko I,Yurdakul S.Behcet’s syndrome:disease manifestations,mana- gement,and advances in treatment.[J]Nat Clin Pract Rheumatol,2007,3(3):148- 155
20. Evereklioglu C.The treatment schedule and historical naming process of Behcet disease.[J]Eur Acad Dermatol Venereol,2007,21(3):427-428
21. Pipitone N,Olivieri I,Cantini F,et a1.New approaches in the treatment of Adaman- tiades-Behcet’s disease.[J]Curr Opin Rheumato1,2006,l8(1):3-9
22. Sakane T,Takeno M,Suzuki N,et a1.Behcet’s disease.N Engl J Med,1999,341(17): 1284-1291
23. Guillaume-Czitrom S,Berger C,Pajot C,et al.Efficacy and safety of interferon alpha in the treatment of corticodependent uveitis of paediatric Behcet’s disease. [J]Rheumatology (Oxford),2007,46(10):1570-1573
24. Takamoto M,Kaburaki T,Numaga J,et al.Long-term infliximab treatment for Beh- cet’s disease.[J]Jpn J Ophthalmol,2007,51(3):239-240
25. Mydlarski PR,Mittmunn N,Shear NH.Intravenous immunoglobulin:use in derma- tology.Skin Therapy Lett,2004,9:1-6
26. Hamuryudan V,Mat C,Saip S,et a1.Thalidomide in the treatment of the mucocut- aneous lesions of the Behcet syndrome.A randomized,double-blind, placebo-con- trolled tria1.Ann Intern Med,1998,128(6):443-450
27.马百芳,梁玉华,孟克,等.复方甘草酸苷治疗Behcet病临床观察.中国麻风皮肤病杂志,2007,23(3):219-220
28. Kanekura T,Gushi A,Iwata M,et al.Treatment of behcet’s disease with granulocyte and monocyte adsorption apheresis.[J]AM ACAD Dermatol,2004,51(2):83-87
29.王冬英.中西医结合治疗白塞病50例.现代中西医结合杂志,2008,17(5):692
30.陈岚,叶岚,陈兴平,等.58例白塞病回顾性分析.中国麻风皮肤病杂志,2003,19(1):25-27
1.赵辨.临床皮肤病学[M].第3版.南京:江苏科学技术出版社,2001,867
2. Sakane T,Takeno M,Suzuki N,et al.Behcet’s disease.N Engl J Med,1999,341: 1284-1291
3.曹光玲,凌红忠,李欢,等.白塞病64例临床分析.岭南皮肤性病科杂志,2001,8(3):134-136
4.陈岚,叶岚,陈兴平,等.58例白塞病回顾性分析.中国麻风皮肤病杂志,2003,1(1):25-27
5.赵辨.临床皮肤病学[M].第3版.南京:江苏科学技术出版社,2001,865
6.苏彦果,台立稳,董瑞芳.神经白塞氏病2例报告.脑与神经疾病杂志,2007,15(6):465-466
7.郭立琳,刘永太,田庄,等.白塞病合并心脏血栓4例临床分析.北京医学,2008,30(6):342-344
8.刘永太,田庄,方理刚,等.白塞病血管受累的临床分析.北京医学,2007,29(8):452-454
9. Frassnito MA,Dammacco R,Cafforio P,et al.Th1 polarization of the immune response in Behcet’s disease:a putative pathogenic role interleukin-12.Arthritis Rheum,1999,26:510-512
10. Zouboulis CC,Drtanous CE.Treatment of Adamantiades Behcet’s disease with systemic interferon alfa.Arch Dermatol,1998,134:1010
11. Hamuryudan V,Mat C,Saip S,et a1.Thalidomide in the treatment of the mucocu- taneous lesions of the Behcet syndrome.A randomized,double-blind, placebo- controlled tria1.Ann Intern Med,1998,128(6):443-450