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Classification and Diagnosis Criteria for Behçet’s Disease

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Behçet's Syndrome

Part of the book series: Rare Diseases of the Immune System ((RDIS))

Abstract

Behçet’s disease is relatively a young disease, described in 1937. Nevertheless, it has already 17 sets of diagnosis/classification criteria. The first set was created in 1946 and the last in 2010. The first created by an international collaboration was the International Study Group criteria (ISG) in 1990 with the collaboration of 7 countries, and the last the International Criteria for Behçet’s Disease (ICBD) in 2010 with the collaboration of 27 countries. In ICBD, the following signs get each 2 points: oral aphthosis, genital aphthosis, and ocular manifestations. Skin manifestations, vascular lesions, neurologic manifestations, and a positive pathergy test get each 1 point. To classify or diagnose a patient as Behçet’s disease, the patient must totalize 4 or more points. As pathergy test is not performed in some countries, mainly western countries, its use is facultative. However, in patients not fulfilling the ICBD it is recommended to perform it. It is also recommended to classify or diagnose a patient if no other disease could explain the symptoms.

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Authors and Affiliations

  1. Rheumatology Research Center, Tehran University of Medical Sciences, Jalal Al-Ahmad Avenue, 14117, Tehran, Iran

    Fereydoun Davatchi, Bahar Sadeghi Abdollahi, Farhad Shahram & Abdolhadi Nadji

  2. Rheumatology Research Center and Department of Dermatology, Tehran University of Medical Sciences, Jalal Al-Ahmad Avenue, 14117, Tehran, Iran

    Cheyda Chams-Davatchi

  3. Rheumatology Research Center and Department of Ophthalmology, Tehran University of Medical Sciences, Jalal Al-Ahmad Avenue, 14117, Tehran, Iran

    Hormoz Shams

Authors
  1. Fereydoun Davatchi
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  2. Bahar Sadeghi Abdollahi
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  3. Farhad Shahram
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  4. Cheyda Chams-Davatchi
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  5. Hormoz Shams
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  6. Abdolhadi Nadji
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Correspondence to Fereydoun Davatchi .

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Editors and Affiliations

  1. SOD Patologia Medica, Center for Autoimmune Systemic Diseases, Behçet Center and Lupus Clinic - AOU Careggi, Florence, Italy

    Lorenzo Emmi

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Davatchi, F., Sadeghi Abdollahi, B., Shahram, F., Chams-Davatchi, C., Shams, H., Nadji, A. (2014). Classification and Diagnosis Criteria for Behçet’s Disease. In: Emmi, L. (eds) Behçet's Syndrome. Rare Diseases of the Immune System. Springer, Milano. https://doi.org/10.1007/978-88-470-5477-6_17

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  • DOI: https://doi.org/10.1007/978-88-470-5477-6_17

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  • Publisher Name: Springer, Milano

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  • Online ISBN: 978-88-470-5477-6

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