Optic neuritis as a presenting feature of Behçet's disease: case-based review

Rheumatol Int. 2021 Jan;41(1):189-195. doi: 10.1007/s00296-020-04690-6. Epub 2020 Aug 29.

Abstract

Behçet's disease (BD) is vasculitis affecting vessels of variable sizes characterized with recurrent oral and/or genital aphthous ulcers accompanied by cutaneous, ocular, articular, gastrointestinal, and/or central nervous system inflammatory lesions. The disease is characterized by recurrent attacks and remissions of different durations, which is one of the reasons why the diagnosis is, in most cases, made several years after the onset of first symptoms. We present a 24-year old male, with South Eastern European heritage, with relapsing bilateral optic neuritis as a first symptom of the disease, followed by aseptic meningitis 2 years, and vascular manifestation 3 years after onset, which finally led to the diagnosis of Behçet's disease. Vascular symptoms were thromboembolism of the right leg and aneurism of the right popliteal artery that due to the size required surgical treatment. The patient was treated with glucocorticoids, azathioprine and anti-tumor necrosis factor-alpha therapy, that proved to be the best treatment options for all manifestations of the disease. Based on our literature review, optic neuritis is a known and rare clinical feature of BD. To our knowledge, there are only several literature reports in which optic neuritis is the initial symptom of BD. Our case report and literature review emphasize the importance of recognizing optic neuritis without inflammatory eye disease as a possible presenting symptom of BD and accentuate detailed medical history review at each patient's visit.

Keywords: Aneurysm; Behcet disease; Optic neuritis; Tumor necrosis factor inhibitors.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aneurysm / diagnostic imaging
  • Aneurysm / etiology*
  • Aneurysm / pathology
  • Aneurysm / surgery
  • Behcet Syndrome / diagnosis*
  • Disease Progression
  • Humans
  • Lower Extremity / diagnostic imaging
  • Male
  • Optic Neuritis / etiology*
  • Ultrasonography, Doppler, Color
  • Young Adult