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Behçet's disease: How to diagnose and treat vascular involvement

Emire Seyahi1

  • 1University of Istanbul, Cerrahpasa Medical Faculty, Department of Medicine, Division of Rheumatology, Istanbul 81310, Turkey.

Best Practice & Research. Clinical Rheumatology
|November 26, 2016
PubMed

Insights

Behçet's disease causes recurrent lesions and sight-threatening inflammation. Vascular issues, especially blood clots in veins and arteries, are common and increase mortality risk.

Area of Science:

  • Rheumatology
  • Internal Medicine
  • Vascular Medicine

Background:

  • Behçet's disease is a multisystem disorder of unknown cause.
  • It presents with mucocutaneous lesions, panuveitis, and characteristic vascular involvement.
  • Vascular manifestations are more severe in males, often involving veins and leading to thrombosis.

Purpose of the Study:

  • To summarize the key features of vascular involvement in Behçet's disease.
  • To highlight the common types and consequences of vascular complications.
  • To emphasize the importance of immunosuppressive therapy.

Main Methods:

  • Review of clinical characteristics of Behçet's disease.
  • Analysis of vascular manifestations, including thrombosis and aneurysms.
  • Evaluation of mortality causes and treatment strategies.

Main Results:

  • Lower extremity vein thrombosis (LEVT) is the most frequent vascular manifestation.
  • Pulmonary artery involvement (PAI) and Budd-Chiari syndrome are major causes of mortality.
  • Individuals may present with multiple types of vascular involvement, often starting with LEVT or cerebral venous sinus thrombosis.

Conclusions:

  • Vascular involvement is a critical and potentially fatal aspect of Behçet's disease.
  • Early diagnosis and management of vascular complications are essential.
  • Immunosuppressive treatment is crucial for preventing attacks and improving survival in Behçet's disease patients.

Keywords:
AnticoagulationAzathioprineBehçet's diseaseLower extremity vein thrombosisPulmonary artery aneurysmsThrombosisVasculitisVena cava thrombosis

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