Contents

History

Initial Contributions of Adamantiades and Behget 3

A Historical Review of Adamantiades-Behget's Disease 7

Ch. C. Zouboulis and W. Kietel

Epidemiology

Multicentre Clinical Registries 17

P. Martus

Epidemiology of Behcet's Disease in Asian Countries and Japan 25

F. Kaneko, K. Nakamura, M. Sato, M. Tojo, X. Zheng, and J.-Z. Zhang

Recent Epidemiological Data on Behcet's Disease in Iran 31

F. Shahram, F. Davatchi, A. Nadji, A. Jamshidi, H. Chams, C. Chams, N. Shafaie, M. Akbarian, and F. Gharibdoost

Prevalence of Behcet's Disease among Iraqis 37

Current Epidemiological Data from the German Registry of

Adamantiades-Behgets Disease 43

Ch. C. Zouboulis, I. Kötter, D. Djawari, L. Krause, U. Pleyer, R. Stadler, W. Kirch, U. Wollina, P.K. Kohl, W. Keitel, xiii

F.R. Ochsendorf, H.P.M. Gollnick, H. Borgmann, J.R. Turnbull,

R. Keitzer, E. Hölzle, E. Proksch, R. Söhnchen, H. Blech, R. Glosemeyer,

G.E. Gross, Y. Hoch, E.G. Jung, G. Koch, B. Pfeif, J. Reichrath, W. Schaffartzik, H. Weber, K. Fritz, and C.E. Orfanos

Heterogenity of Clinical Manifestations in Behcet's Disease among Different Ethnic Groups 49

A. Yelonakov, Z. Alekberova, and V. Yasdovsky

Behcet's Disease in Patients of German and Turkish Origin - A Comparative Study 53

I. Kötter, R.. Vonthein, I. Günaydin, C. Müller, L. Kanz, M. Zierhut, and N. Stübiger

Influence of Sex on Patients with Behcet's Disease in Korea 59

The Influence of Gender on the Frequency of Clinical Symptoms in Behcet's Disease 65

F. Davatchi, F. Shahram, C. Chams, H. Chams, A. Nadji, A. R. Jamshidi, M. Akbarian, and F. Gharibdoost

The Influence of Gender on the Severity, and the Outcome of Ocular Lesions in Behcet's Disease 67

F. Davatchi, F. Shahram, H. Chams, A. Nadji, A. R. Jamshidi, C. Chams, M. Akbarian, and F. Gharibdoost

The Implications of Nonaphthous Beginning of Behcet's Disease 73

N. Dilsen

Clinical Manifestations and Course of 200 Japanese Patients with Behcet's Disease 77

C. Tanaka, T. Matsuda, E. Hayashi, Y. Imamura, and S. Ozaki

Clinical Presentation of Behcet's Disease in Internal Medicine 81

B. Dianati, S. Saliba, G. Etienne, X. Delbrel, M. Grenouillet, P. Mercie, J. L. Pellegrin, M. Lebras, and M. Longy-Boursier

The Change of Clinical Manifestations of Patients with Behcet's Disease in Japan 83

S. Kotake, K. Namba, K. Higashi, C. Goda, T. Ariga, A. Ogawa, and S. Ohno

Chronology of Clinical Manifestations in Behget's Disease 85

F. Shahram, K. Assadi, F. Davatchi, A. Nadji, A. Jamshidi, C. Chams, and M. Akbarian

General Aspects and Clinical Manifestations of 100 Iraqi Patients with Behget's Disease 91

M. M. Al-Waiz, I. Kh. Al-Joofy, R. K. Muslih, and K. M. Mousawy

The Prevalence of Behget's Syndrome and its Neurological Complications in Hertfordshire, U.K. 95

D. Kidd

Clinical and Genetic Characteristics of Late-Onset Behget's Disease 99 A. Weinberger, T. Klein, and I. Krause

Diagnostic criteria, prognostic parameters, assessment of disease activity, and quality of life

The Importance of the Manifestations Besides the Ones Included in International Criteria for Behget's Disease 105

N. Dilsen

Influence of Pathergy Test on the Accuracy of Different Diagnosis Criteria for Behget's Disease 109

F. Davatchi, F. Shahram, A. Nadji, A. R. Jamshidi, C. Chams, H. Chams, M. Akbarian, and F. Gharibdoost

Measuring Health Related Quality of Life 113

M. Bullinger

Univariate and Multivariate Analyses Comparing Demographic, Genetic, Clinical, and Serological Risk Factors for Severe Adamantiades-Behget's Disease 123

Is Long Term Observation of Patients with Recurrent Aphthous Stomatitis Necessary? 127

P. Ekmekqi, A. Boyvat, Özdemir, A. Gürler, and E. Gürgey

Acetylator Phenotype in Behget's Disease 131

HLA Antigen, Severity and Acetylator Phenotype in Behget's

Disease Patients with Non-Relative Parents 139

Risk Factors of Neuro-Behget 143

H. Houman, I. Ben Ghorbel, A. Braham-Sfaxi, M. Khanfir, M. Ben Ahmed, M. Lamloum, and M. Miled

Association of HLA-B51 with Clinical Expression of Behcet's Disease 147

F. Shahram, F. Davatchi, A. Nadji, A. Jamshidi, K. Bahar, M. Akbarian, and F. Gharibdoost

The Behget's Disease Activity Index 149

G. Lawton, M. A. Chamberlain, B. B. Bhakta, and A. Tennant

Development of Clinical Activity Form for Korean Patients with Behget's Disease 153

E.-S. Lee, H. S. Kim, D. Bang, H. G. Yu, HChung, D. H. Shin,

Y. W. Song, Y. B. Park, S. K. Lee, S. K. Shin, W. H. Kim, J. Choi, B. J. Park, and S. Lee

Living with Behget's Disease 157

G. Gilworth, M. A. Chamberlain, B. Bhakta, A. Tennant, A. Silman, and D. Haskard

Pathogenesis

Pathogenesis of Adamantiades-Behget's disease 161

Immunopathogenesis and Prevention of Uveitis with the Behget's Disease-Specific Peptide Linked to Cholera Toxin B 173

T. Lehner, M. R. Stanford, P. A. Phipps, J. B. Sun, B. G. Xiao, J. Holmgren, T. Shinnick, A. Hasan, and Y. Mizushima

The Role of Infectious Agents in the Pathogenesis of Behget's Disease 181

Detection of Microbial DNA in Skin Lesions from Patients with Behget's Disease 185

M. Tojo, H. Yanagihori, X. Zheng, N. Oyama, E. Isogai, K. Nakamura, and F. Kaneko

Association of Mycoplasma fermentans with Adamantiades-Behçet's

Disease 191

Ch. C. Zouboulis, J. R. Turnbull, and P. F. Mühlradt

Antimicrobial Activity of Synthetic Human CAP18 Peptides to Streptococcus sanguis Isolated from Patients with Behcet's Disease 195 E. Isogai, M. Hirata, H. Isogai, K. Matuo, K. Kimura, K. Yokota, K. Oguma, M. Tojo, F. Kaneko, S. Kotake, and S. Ohno

Anti-Saccharomyces Cerevisiae Antibodies 201

I. Krause, Y. Monselise, G. Milo, and A. Weinberger

Genetics

The Human Genome Project: What We have Learnt about the MHC Region on Chromosome 6 and its Potential to Behcet's Disease 207

S. Beck

Comparison of Proteome Map Between Sera of Patients with Behcet's Disease and Controls 213

HLA Typing for Class I and Class II Antigens in Iraqi Patients with Behcet's Disease (Sporadic and Familial Cases) 217

I. Kh. Al-Joofy, R. K. Muslih, K. M. Mousawy, and M. M. Al-Waiz

Strong Association of MIC-A*009 of Extracellular Domains and MIC-A*A6 of Transmembrane Domain in Korean Patients with Behcet's Disease 221

MICA Transmembrane Region Polymorphism and HLA B51 in Tunisian Behcet's Disease Patients 225

M. Ben Ahmed, H. Houman, S. Abdelhak, I. Ben Ghorbel, M. Miled, K. Dellagi, and H. Louzir

HLA-B51 Frequency in Iranian Patients with Behcet's Disease 229

F. Shahram, F. Davatchi, A. Nadji, N. Jamshidi, K. Bahar, M. Akbarian, and C. Chams

Frequency of HLA in Patients with Behçet's Disease and Association with Occlusive Retinal Vasculitis 231

N. A. Ermakova, Z. S. Alekberova, T. B. Prokaeva, and I. B. Poljanskaja

The ICAM1469*E is Associated with Susceptibility to Ocular Lesions and Vasculitis in Korean Patients with Behcet's Disease 235

E. H. Kim, J. W. Mok, D. Bang, E.-S. Lee, S. Lee, and K. S. Park

Immunology

Chemokines in Behcet's Disease, a Field to Be Explored as a Potential Basis for Therapy 239

M. Baggiolini

Expression of CCR5 in Behcet's Disease 245

Immunophenotype and Th1/Th2 Cytokines in Patients with Adamantiades-Behget's Disease 249

K. Psarra, V. Kapsimali, G. Vaiopoulos, M. Nikolopoulou, PH. Kaklamanis, G. Palimeris, E. Kaklamani, and C. Papasteriades

Cytokine Production of Peripheral Blood Mononuclear Cells Stimulated with Streptococcus Sanguis Antigen in Patients with Behget's Disease 255

Serum Levels of Soluble TNF-a Receptor-II (P75), Circulating y8 T-Cells and Adamantiades-Behget's Disease Activity 261

A. V. Elezoglou, P. P. Sfikakis, G. Vaiopoulos, V. Kapsimali, and Ph. G. Kaklamanis

T-y8 Receptor Restriction in Peripheral Lymphocytes of Patients with Behget's Disease 267

P. M. Van Hagen, H. Hooijkaas, M. W. M. VD Beemd, G. Verjans, and G.S. Baarsma

Lymphocyte Subsets and Activated Neutrophils in Iraqi Patients with Behget's Disease 269

K. M. Mousawy, I. Kh. Al-Joofy, R. K. Muslih, andM. M. Al-Waiz

L-Selectin Expression on Leukocytes of Patients with Behget's Disease 273 S. H. Assaad-Khalil, M. Abou-Seif, I. Youssef, and N. Farahat

Autoimmunity to S-Antigen and Retinal Vasculitis in Patients with Behget's Disease 279

N. A. Ermakova, Z. S. Alekberova, and T. B. Prokaeva

Nerve Growth Factor in Behget's Disease 283

M. Accorinti, A. Bruscolini, A. Lambiase, S. Speranza, S. Bonini, and P. Pivetti-Pezzi

Low Serum Mannose-Binding Lectin Levels in Behcet's Disease 287

N. Inanc, E. Birtas, S. Yavuz, T. Ergun, and H. Direskeneli

Concepts for Research

ISBD Basic Research Perspectives 293

Mucocutaneous manifestations

Complex Aphthosis: Evaluation for Behcet's Disease? 303

Complex Aphthosis 311

Comparison of Oral Aphthae in Behcet's Disease and Idiopathic

Recurrent Aphthous Stomatitis 317

C. Chams-Davatchi, M. Shizarpour, F. Davatchi, F. Shahram, H. Chams, A. Nadji, and A. R. Jamshidi

Bipolar Aphthosis. A Forme Fruste of Behcet's Disease 321

The Effect of Smoking on the Clinical Features of

Adamantiades-Behget's Disease 323

V. G. Kaklamani, A. Tzonou, N. Markomichelakis, S. Papazoglou and Ph. G. Kaklamanis

Muco-Cutaneous Lesions of Behget's Disease 329

C. Chams-Davatchi

Dilsen's Pathergy Test in Behget's Disease: Positive Correlation with Clinical Manifestations 331

Is Behget's Disease a Kobner Positive Disorder? M. Önder, M. Oztas, and A. Aral

Extensive Pyoderma Gangrenosum-Like Lesion in Two Cases of Behget's Disease, Responding only to Cyclosporin 337

C. Chams-Davatchi, M.Shizarpour, F. Davatchi, F. Shahram, H. Chams. A. Nadji, and A. R. Jamshidi

A Case of SAPHO Syndrome with Pyoderma Gangrenosum and Inflammatory Bowel Disease Masquerading as Behcet's Disease 339

O. Yamasaki, K. Iwatsuki, and F. Kaneko

Cytokine Expression Within Mucocutaneous Lesions of Behget's Disease: Involvement of Proinflammatory and Th1 Cytokines 343

M. Ben Ahmed, H. Houman, I. Ben Ghorbel, A. Braham-Sfaxi, M. Miled, K. Dellagi, and H. Louzir

Ocular Manifestations

Origin and Outcome of Macular Edema in Behget's Disease 349

H. Chams, A. Lashei, A. Sadeghi-Tari, R. Karkhaneh, F. Davatchi, F. Shahram, C. Chams, and A. Abazari

Fluorescein Angiography and Optical Coherence Tomography in Ocular Behget's Disease 355

Cells of the Vitreous Body in Behget's Disease 361

Ocular Lesions Other than Behget's Disease in Behget's Disease-Affected Patients 365

M. Accorinti, M. P. Pirraglia, R. Corradi, M. Marino, and P. Pivetti-Pezzi

Neurological manifestations

Neuro-Behget's Disease (Isolated Cerebral Thrombophlebitis Excluded) 371 A. Sbai, B. Wechsler, P. Duhaut, L. T. H. Du-Boutin, Z. Amoura, P. Cacoub, P. Godeau, and J. C. Piette

The Prevalence of Headache in Behget's Syndrome 377

D. Kidd

Adamantiades-Behcet's Disease and Elevated Intracranial Pressure in a 12 Year Old Turkish Girl 381

U. Neudorf, R. Wieland, and J. Schaper

Sensory Symptoms in Behcet's Syndrome 383

M. Siepmann, and W. Kirch

Immunohistological Studies in Neuro-Behget's Disease 385

S. Hirohata, H. Arai, and T. Matsumoto

Soluble Fas Ligand Levels In Cerebrospinal Fluid in Neuro-Behget's Disease 389

E. Aoyama-Hayashi, T. Matsuda, N. Ohya, C. Tanaka, M. Kongozi, C. Kamo, M. Hotta, H. Ikejima, M. Shiraishi, and Y. Mizushima

Cardiovascular Involvement

Behçet's Disease With Vascular Involvement: The Contribution of Anticardiolipin Antibodies and Thrombomodulin 395

H. Nakano, H. Oka, T. Matsuda, S. Ooka, Y. Imamura, T. Suzuki, Y. Yamasaki, G. Itoh, K. Azuma, and S. Ozaki

Arterial Wall Characteristics in Patients with Adamantiades-Behget's Disease 399

A. Protogerou, J. Lekakis, K. Stamatelopoulos, K. Aznaouridis, E. Karatzis, I. Ikonomidis, Ch. Papamichael, N. Markomihelakis M. Mavrikakis, and Ph. G. Kaklamanis

The Influence of Plasma Fibrinogen and Serum Ferrum on Blood Viscosity in Adamantiades-Behget's Disease 405

S. Mineshita, L.M. Wang, A. Miura, S. Umezu, Y. Honda, and T. Satoh

Does Hyperhomocysteinemia Increase the Risk of Thrombosis in Behget's Disease? 413

H. Houman, M. Feki, I. B. Ghorbel, H. Gadhoum, M. Hsairi, S. Omar, M. Lamloum, N. Kaabachi, A. Mebazaa, and M. Miled

Pulmonary Artery Involvement in Behget's Disease 419

A. Akdag Köse, M. Kayabali, R. Sarica, E. Saglik, and G. Azizlerli

Cardiac Manifestation in Four Patients with Adamantiades-Behget's Disease 423

J. R. Turnbull, A. Tunsch, Y. D. Adler, L. Lehmann, H. Borgmann, and Ch.C. Zouboulis

Quantitative Evaluation of Microvessels in Behçet's Disease 427

M. Pastorelli, A. L. Pasqui, L. Puccetti, U. Beermann, F. Biagi, A. Camarri, A. Palazzuoli, M. Servi, A. Costa, D. Bischeri, F. Del Santo, F. Gelli, M. Saletti, F. Bruni, and A. Auteri

18F-FDG-Positron Emission Tomography for Diagnosis of Large Vessel Arteritis in Behçet's Disease 435

M. Wenger, M, Baltaci, P. Klein-Weigel, E. Donnemiller, R. Moncayo, and M. Schirmer

Spontaneous Platelet Aggregation in Patients with Behcet's Disease by Using Laser-Light Scattering Aggregometer 437

H. Nakano, H. Oka, T. Matsuda, S. Ooka, N. Suzuki, J. Hayashi, S. Ishida, T. Suzuki, K. Hirota, and S. Ozaki

Various Clinical Manifestations

New Perspectives of Imaging Techniques for Diagnosis of Organ Manifestations in Behçet's Disease 445

M. Schirmer, A. Klauser, M. Wenger, M. Baltaci, and R. Moncayo

The Comparison of Ankylosing Spondylitis in Behçet's Disease and Normal Population 449

A. Nadji, F. Davatchi, F. Shahram, A. Jamshidi, C. Chams, M. Akbarian, F. Gharibdoost, and K. Mowla

Gastro-Intestinal Manifestations of Behçet's Disease 455

W. Madanat, Z. Sharaiha, S. Khasawneh, H. Zureikat, and F. Fayyad

Gastrointestinal Disease in Behcet's Disease 459

E. M. Ward, T. A. Woodward, M. Mazlumzadeh, and K. T. Calamia

Clinical Features of Behcet's Disease Patients with Epididymitis 465

D. Bang, Y.-H. Cho, J. Jung, K.-H. Lee, E.-S. Lee, and S. Lee

Tonsillectomy and Behcet's Disease 471

M. Jalessi, F. Shahram, A. Safavi, F. Davatchi, A. Nadji, A. Jamshidi, C. Chams, and F. Gharibdoost

Destructive Sinusopathy and Middle Ear Involvement in Behcet's Syndrome 473

H. M. G. Martins, S. Santos, J. F. Lourenco, and J. A. Araujo

Urine Abnormalities in Behget's Disease 477

F. Shahram, F. Davatchi, A. Nadji, A. Jamshidi, M. Akbarian, F. Gharibdoost, and C. Chams

Treatment

Behget's Syndrome: From Aetiology to Treatment 481

Treatment of Ocular Manifestations of Behget's Disease 487

F. Davatchi

The Influence of the Delay of Aggressive Treatment on the Vision and its Outcome in Behget's Disease 493

F. Davatchi, F. Shahram, H. Chams, A. Nadji, A.R. Jamshidi, C. Chams, M. Akbarian, and F. Gharibdoost

The Clinical Outcome and Treatment in Behget's Disease with Deep Vein Thrombosis 495

A. Akdag Köse, M. Kayabali, R. Sarica, R. Kaymaz, and G.Azizlerli

The Use of Interferon-a in Behget's Disease - Review of the

Literature and Possible Mechanisms of Action 503

I. Kötter, I. Günaydin, M. Treusch, M. Zierhut, L. Kanz, and N. Stübiger

Interferon alfa-2a in the Treatment of Ocular Adamantiades-Behget's Disease 511

L. Krause, J. R. Turnbull, N. Torun, U. Pleyer, Ch. C. Zouboulis, and M. H. Foerster

Human Recombinant for the Treatment of

Behget's Disease with Sight-Threatening Retinal Vasculitis 521

I. Kötter, M. Zierhut, A. Eckstein, R. Vonthein, T. Ness, I. Günaydin,

B. Grimbacher, S. Blaschke, H.-H. Peter, L. Kanz, and N. Stübiger

Behget's Disease: Visual Acuity after 5 Years in Patients with alphaInterferon Treatment 525

C. M. E. Deuter, I. Kötter, I. Günaydin, M. Zierhut, and N. Stübiger

Multifocal ERG Changes in Patients with Ocular Behget's Disease During Therapy with Interferon alpha 2a 529

N. Stübiger, D. Besch, C. M. E. Deuter, M. Zierhut, and I. Kötter

Influence of Interferon-alpha on Lymphocyte Subpopulations in Behget's Disease

M. Treusch, N. Stübiger, R. Vonthein, M. Baur, S. Koch, I. Günaydin, L. Kanz, M. Zierhut, and I. Kötter

Interferon alpha 2a in IRPB-Derived Peptide-Induced EAU - Part N. Stübiger, I. J. Crane, I. Kötter, G. R. Lamont, M. Zoeller, U. Günthert, J. V. Forrester, M. Zierhut, andR. H. Stiemer

A Case of Behget's Disease with Pathergy Reaction at Interferon Injection Site

Current and Future Use of Anti-TNF Agents in the Treatment of Autoimmune, Inflammatory Disorders K. T. Calamia

Successful Long-Term Treatment of Refractory Adamantiades-Behget's Disease (ABD) with Infliximab: Report of Two Patients 551

C. G. Katsiari, P. G. Theodossiadis, Ph. G. Kaklamanis, N. N. Markomichelakis, and P. P. Sfikakis

The Effect of Anti-Tumour Necrosis Factor Alpha (Infliximab) on Sight-Threatening Uveitis in a Patient with Behget's Disease 557

I 537

Antitumor Necrosis Factor Monoclonal Antibody Therapy in a Woman with Severe Adamantiades-Behget's Disease 561

F. Behrens, T. Zollner, B. Moeller, J. P. Kaltwasser, R. Kaufmann, and F. R. Ochsendorf

Efficacy of Corticosteroids and Cyclosporin in the Treatment of Retinal Vasculitis in Patients with Behget's Disease 563

N. A. Ermakova

Enhancement of Transmucosal Permeation of Cyclosporine by Benzalkonium Chloride 567

P. Van Der Bijl, A. D. Van Eyk, A. A. Gareis, and I. O. C. Thompson

Azathioprine and Low Dose Pulse Cyclophospha-Mide in Severe Ocular Lesions of Behget's Disease 571

F. Shahram, F. Davatchi, H. Chams, A. Nadji, A. Jamshidi, M. Akbarian, and F. Gharibdoost

Low Dose MTX for Progressive Neuro-Behget's Disease 575

H. Kikuchi, K. Aramaki, and S. Hirohata

High Dose Methotrexate for Ocular Lesions of Behcet's Disease 579

F. Davatchi, F. Shahram, H. Chams, A. R. Jamshidi, A. Nadji, C. Chams, M. Akbarian, and F. Gharibdoost

Therapeutic Effect of Thalidomide through Cytokine and Chemokine Regulation in Herpes Simplex Virus-Induced Behget's Disease-Like Animal Model 585

S. Sohn, E.-S. Lee, S. I. Lee, Y. A. Kim, H. J. Kwon, D. Bang, and S. Lee

Pentoxifylline Use for Behget's Disease 591

Cataract Surgery in Behget's Disease Patients 595

Do Metal Surgical Staples Induce Post-Surgical Intestinal Ulcer in Behget's Disease? 599

T. Matsuda, Y. Imamura, A. Shimizu, E. Hayashi, S. Tsukikawa, M. Enomoto, and T. Nakagawa

Patient - Physician Relationship

Clinicans, Scientists and Patients 603

Hopes of Patients with Behget's Disease in Japan towards

Researchers and the Relationship between Patients and Medical

Staff Concerning Social Work 605

T. Nishida, M. Nishida, M. Wakayama, M. Nishida, and S. Ohno

Jordan Friends of Behget's Disease Patients Society 609

J. Tamimi, and W. Madanat

The Relationship between Patient Groups and Physicians from a UK Support Group Perspective 613

G. Seaman, and R. Pearce

"Living With Behget" - A Young Patients' Group A. Müller

Private Practice Offices Serve as Centers for Adamantiades-Behget's Disease 621

K. Fritz

The Editor 627

Index 629

ADAMANTIADES-BEHQET'S DISEASE

HISTORY

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