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Phenotypes in Behçet’s syndrome

  • Emire SeyahiEmail author
IM - REVIEW
Part of the following topical collections:
  1. Behcet disease

Abstract

Behçet syndrome (BS) is a multi-systemic complex disorder with unknown etiology and a unique geographic distribution. It could not be possible to include it into specific classification schemes and it is certainly not a uniform disease. Several cluster and association studies revealed that it has been composed of multiple phenotypes ascribing the principal problem such as skin-mucosa, joint, eye, vascular, neurological and gastrointestinal involvement. Each phenotype has its own characteristic demographic and clinical features as such their management strategies and prognosis differ substantially. Actually, the concept of phenotyping has been well known for some time and is considered one of the basic elements of the still continuing debate whether to call this entity ‘disease’ or ‘syndrome’. Further supporting evidence comes from the observation of the geographical differences of disease expression. In this setting, BS resembles rather a construction made of several dynamic and interactive LEGO pieces of different shapes and colors. These pieces presenting phenotypes with their own disease mechanism have presumably different genetic determinants. The analysis of phenotyping could help us to identify this disorder and hence could contribute to find better ways of treatment.

Keywords

Behçet’s syndrome Phenotypes Oral ulcers Uveitis Crohn’s disease Venous thrombosis Neurological involvement Ethnic differences 

Notes

Funding

None.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no competing interests.

Statements on human and animal rights

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

No informed consent was necessary for the study.

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© Società Italiana di Medicina Interna (SIMI) 2019

Authors and Affiliations

  1. 1.Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical FacultyUniversity of IstanbulIstanbulTurkey

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