Morbus Behçet – a rare disease in Central Europe

Behçet's disease (BD) is a multiorgan inflammatory disease of complex and not entirely elucidated etiology, which was originally diagnosed in patients with aphthous stomatitis, genital ulcerations and ocular manifestations. The entity is endemic in countries of Eastern and Central Asia, especially Turkey and Iran, but rarely seen in Central Europe. As there are no specific diagnostic laboratory tests or histopathologic findings which confirm the preliminary diagnosis, the final diagnosis should be based on clinical criteria. Frequently a definitive diagnosis is established within several years or months after the first manifestations appear. The increased number of cases, recently described worldwide also in the Polish population, indicates that the disease could spread out of endemic areas. The aim of this manuscript is to present the clinical picture, diagnosis criteria and therapeutic approaches of this “international disease” which currently is observed not only in emigrants from Asia but also in native Polish citizens.

[1]  D. Valla,et al.  Behcet’s disease in budd-chiari syndrome , 2013, Orphanet Journal of Rare Diseases.

[2]  Queiroz,et al.  The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria , 2014, Journal of the European Academy of Dermatology and Venereology : JEADV.

[3]  E. Cunningham,et al.  Differential Diagnosis of Behçet Uveitis , 2013, Ocular immunology and inflammation.

[4]  S. Bonin,et al.  Analysis of Diagnostic Criteria in Adamantiades-Behçet Disease: A Retrospective Study , 2013, Indian journal of dermatology.

[5]  F. Shahram,et al.  Impact of the positive pathergy test on the performance of classification/diagnosis criteria for Behcet’s disease , 2013, Modern rheumatology.

[6]  M. Liang,et al.  Oral aphthosis: management gaps and recent advances. , 2012, Annals of the Academy of Medicine, Singapore.

[7]  N. Shen,et al.  Cytokines and Behcet's disease. , 2012, Autoimmunity reviews.

[8]  M. K. Latinis The Washington manual rheumatology subspecialty consult , 2012 .

[9]  C. Zouboulis,et al.  [Epidemiology and clinical aspects of Adamantiades-Behçet disease in Gemany. Current data]. , 2012, Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft.

[10]  O. Köse Development of Immunopathogenesis Strategies to Treat Behçet's Disease , 2012, Pathology research international.

[11]  S. Moretti,et al.  Potential Infectious Etiology of Behçet's Disease , 2011, Pathology research international.

[12]  A. Kokturk Clinical and Pathological Manifestations with Differential Diagnosis in Behçet's Disease , 2011, Pathology research international.

[13]  F. Davatchi Diagnosis/Classification Criteria for Behcet's Disease , 2011, Pathology research international.

[14]  M. Ustun,et al.  Cerebral abscesses in Behcet's disease: a case report. , 2010, Turkish neurosurgery.

[15]  O. Hiz,et al.  Effects of Behçet's disease on sexual function and psychological status of male patients. , 2011, The journal of sexual medicine.

[16]  F. Shahram,et al.  Behcet’s disease: from east to west , 2010, Clinical Rheumatology.

[17]  F. Gogus,et al.  Prevalence of Behçet's syndrome in patients presenting with venous thrombosis: prospective study in a cardiovascular outpatient clinic , 2009 .

[18]  L. Guillevin,et al.  HLA-B51/B5 and the risk of Behçet's disease: a systematic review and meta-analysis of case-control genetic association studies. , 2009, Arthritis and rheumatism.

[19]  W. Placek,et al.  Case report Behçet disease – case report and review , 2009 .

[20]  F. Shahram,et al.  Colchicine versus placebo in Behçet’s disease: randomized, double-blind, controlled crossover trial , 2009, Modern rheumatology.

[21]  P. Neri,et al.  Long-term control of cystoid macular oedema in noninfectious uveitis with Mycophenolate Mofetil , 2009, International Ophthalmology.

[22]  J. Kempen,et al.  Ocular inflammation in Behçet disease: incidence of ocular complications and of loss of visual acuity. , 2008, American journal of ophthalmology.

[23]  J. Coste,et al.  Population-based prevalence study of Behçet's disease: differences by ethnic origin and low variation by age at immigration. , 2008, Arthritis and rheumatism.

[24]  A. Silman,et al.  EULAR recommendations for the management of Behçet disease , 2008, Annals of the rheumatic diseases.

[25]  F. Shahram,et al.  Prevalence of Behcet's disease in Iran: a WHO‐ILAR COPCORD stage I study , 2007 .

[26]  G. Mumcu,et al.  The role of infectious agents in the pathogenesis, clinical manifestations and treatment strategies in Behçet's disease. , 2007, Clinical and experimental rheumatology.

[27]  İ. Tuğal-Tutkun,et al.  Pro-inflammatory cellular immune response in Behçet’s disease , 2007, Rheumatology International.

[28]  B. Jaremin,et al.  [Behçet's disease--diagnostic difficulties]. , 2007, Polskie Archiwum Medycyny Wewnetrznej.

[29]  P. Martus,et al.  Epidemiology and clinical manifestations of Adamantiades‐Behçet disease in Germany – Current pathogenetic concepts and therapeutic possibilities , 2006, Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG.

[30]  M. Schirmer,et al.  Major vessel involvement in Behçet disease , 2005, Current opinion in rheumatology.

[31]  F. Shahram,et al.  PULSE CYCLOPHOSPHAMIDE IN OCULAR MANIFESTATIONS OF BEHCET'S DISEASE: A DOUBLE BLIND CONTROLLED CROSSOVER STUDY , 2004 .

[32]  H. Kikuchi,et al.  Low dose MTX for progressive neuro-Behçet's disease. A follow-up study for 4 years. , 2003, Advances in experimental medicine and biology.

[33]  C. Ustün A famous Turkish dermatologist, Dr. Hulusi Behçet. , 2002, European journal of dermatology : EJD.

[34]  O. Ozcebe,et al.  Behçet's disease. , 2019, The New England journal of medicine.

[35]  G. Akman-Demir,et al.  Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group. , 1999, Brain : a journal of neurology.

[36]  P. Jego Le thalidomide dans le traitement des lésions cutanéomuqueuses de la maladie de Behçet. Une étude randomisée, à double insu, versus placebo , 1998 .

[37]  S. Yamamoto,et al.  Characterization of T cells specific for an epitope of human 60‐kD heat shock protein (hsp) in patients with Behçet's disease (BD) in Japan , 1997, Clinical and experimental immunology.

[38]  Y. Tüzün,et al.  Azathioprine in Behcet's syndrome: effects on long-term prognosis. , 1997, Arthritis and rheumatism.

[39]  K. Hoshi,et al.  Increased peripheral blood gamma delta+ T cells and natural killer cells in Behçet's disease. , 1992, The Journal of rheumatology.

[40]  Calvin G. Barnes,et al.  A controlled trial of azathioprine in Behçet's syndrome. , 1990, The New England journal of medicine.

[41]  E. Atra,et al.  [Behcet's syndrome]. , 1968, Revista paulista de medicina.

[42]  E. Schulze [Behçet disease]. , 1958, Deutsche medizinische Wochenschrift.