Behçet's Syndrome Apart From the Triple Symptom Complex: Vascular, Neurologic, Gastrointestinal, and Musculoskeletal Manifestations. A Mini Review

Overview

Journal Front Med (Lausanne)

Specialty General Medicine

Date 2021 Apr 26

PMID 33898481

Citations 8

Authors

Ina Kotter

Fabian Lotscher

Affiliations

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Abstract

Behçet's Syndrome (BS) is a variable vessel vasculitis according to the Chapel Hill Consensus Nomenclature (1) and may thus affect any organ, including major and minor arterial and venous vessels to a varying degree and with varying frequency. Although the main features of BS are recurrent oral and genital aphthous ulcers, cutaneous lesions, ocular inflammation and arthritis-major vessel and life-or organ threatening involvement of internal organs and the central and peripheral nervous system occur. In general, BS in Europe appears to form six phenotypes of clinical manifestations (2), which are (1) mucocutaneous only, (2) predominant arthritis/articular involvement, (3) vascular phenotype, (4) ocular manifestations, which are most likely associated with CNS manifestations and HLA-B51, (5) dominant parenchymal CNS manifestations (being associated with the ocular ones), and (6) gastrointestinal involvement. Mucocutaneous manifestations are present in almost all patients/all phenotypes. In the following review, we summarize the current knowledge concerning vascular, neurologic, gastrointestinal and musculoskeletal manifestations of the disease.

Citing Articles

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Thoughts and Experiences of Behçet Disease From Participants on a Reddit Subforum: Qualitative Online Community Analysis.

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[Hughes-Stovin syndrome: a life-threatening manifestation of Behçet's syndrome].

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