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Published on: March 14, 2014
[Adamantiades-Behcet's disease]
1Augenklinik, Campus Benjamin Franklin, Charité-Universitätsmedizin Berlin. lothar.krause@charite.de
Insights
Adamantiades-Behcet's disease (ABD) is a chronic vasculitis with unclear origins, affecting multiple organs. Ocular involvement is common and can lead to blindness, necessitating further research into advanced treatments.
Area of Science:
- Rheumatology
- Ophthalmology
- Immunology
Context:
- Adamantiades-Behcet's disease (ABD) is a multisystemic vasculitis of unknown etiology.
- It predominantly affects populations in Mediterranean, Middle Eastern, and Far Eastern countries but is increasingly recognized globally.
- Clinical manifestations include recurrent oral/genital ulcers, arthritis, skin lesions, and significant ocular involvement.
Purpose:
- To summarize the key clinical features and current understanding of Adamantiades-Behcet's disease.
- To highlight the prevalence and severity of ocular manifestations in ABD.
- To briefly mention existing and emerging treatment strategies for ocular involvement.
Summary:
- Adamantiades-Behcet's disease (ABD) is characterized by recurrent ulcers, arthritis, and skin issues.
- Ocular involvement, including retinal vasculitis and panuveitis, occurs in 60-80% of patients, potentially causing blindness in 20-50% of cases.
- Current treatments involve steroids, cyclosporin A, and azathioprine, with ongoing studies on interferon-alpha and TNF antibodies.
Impact:
- Increased global awareness of Adamantiades-Behcet's disease due to human mobility.
- Highlights the critical need for effective management of ocular complications to prevent vision loss.
- Underscores the importance of continued research into novel therapeutic targets for ABD.
Abstract:
Adamantiades-Behcet's disease (ABD) is a chronic recurrent vasculitis whose aetiology is still unclear. The first description goes back to B. Adamantiades und H. Behcet. The disease mainly occurs in Mediterranean, Middle and Far Eastern countries. However, it is not limited to these regions and can be found worldwide, and, as a result of increasing human mobility, is steadily becoming more significant. Clinical findings reveal recurrent oral and/or genital ulcers, arthritis and skin involvement in the form of erythema nodosum and superficial thrombophlebitis. Relapsing ocular involvement is one of the major manifestations in ABD and can be found in 60-80% of patients, resulting in retinal vasculitis, neuropathy or panuveitis. Eye involvement can lead to blindness in the affected eye in 20-50% of cases. Steroids, cyclosporin A und azathioprin are available to treat affected eyes. Studies of new treatment modalities including interferon-alpha or TNF antibodies are under way.
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