Recent advances in the diagnosis and management of Behçet’s syndrome uveitis

ABSTRACT

Introduction

Behcet’s syndrome (BS) is a chronic multisystem disease that mainly occurs along the ancient Silk Road. Uveitis is a major manifestation of BS with potentially a high visual morbidity. Early diagnosis of BS uveitis (BSU) based on characteristic ocular findings can be helpful in appropriate management of the patients.

Areas covered

A comprehensive literature review was conducted in PubMed database. New advances in the diagnosis and management of BSU were reviewed.

Expert opinion

The diagnosis of BSU is based on a combination of clinical findings. New sets of diagnostic/classification criteria have been developed including the International Criteria for Behçet Disease and the Standardization of Uveitis Nomenclature (SUN) working group criteria. An algorithm for the diagnosis of BSU based on characteristic ocular findings, has been recently proposed to help ophthalmologists in predicting BS diagnosis in patients presenting with isolated uveitis.

Treatment of BSU is currently based on the European Alliance of Associations for Rheumatology (EULAR) and the French recommendations. Treatment of anterior uveitis is based on topical corticosteroids with mydriatics unless high risk factors of poor visual outcomes. Posterior segment involvement requires the combination of systemic corticosteroids, and immunosuppressive or immunomodulating agents. Anti TNFα or INFα2a should be considered in non-responsive patients or in those with acute severe inflammatory attack.

KEYWORDS:

• Behcet’s syndrome
• uveitis
• biologic agents
• corticosteroids
• immunosuppressors
• blindness

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Article highlights

• •The eye is the most frequently affected organ in Behcet’s syndrome, and a potentially blinding uveitis (typically panuveitis with retinal vasculitis), is one of the major manifestations of the disease

• •Diagnosis of BS uveitis remains clinical based on a combination of extraocular and ocular features, and various sets of criteria have been proposed over the years including the new International diagnostic criteria for BD and the SUN working group classification criteria for BD uveitis

• •As uveitis can be an initial or presenting manifestation of the disease in 10-20% of patients with BS uveitis, the recognition of typical ocular changes by the ophthalmologist can be helpful in early diagnosis and prompt initiation of appropriate treatment to prevent irreversible vision loss

• •In patients with isolated anterior uveitis, topical treatment can be sufficient to control inflammation, but concomitant use of immunosuppressors should be considered in patients with high risk factors for poor visual outcome including young age, early onset of the disease and male gender.

• •Posterior segment involvement definitely requires the use of azathioprine in combination with systemic corticosteroids, and ciclosporine A can be added, if needed

• •Patients who are non-responsive to conventional treatment and those who present with an initial or recurrent acute severe inflammatory attack should be treated with anti TNFα or INF α2a in association with glucocorticoids.

• •Periocular or intravitreal corticosteroid injection may be used as an adjunct to systemic treatment in patients with unilateral exacerbation or macular edema.

• •In case of anti-TNF α failure, switching to another anti-TNFα agent or to anti-interleukin 6 is an effective option.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Authors’ contributions statement

All authors contributed to the article concept and design. All authors collected PubMed articles upon Behçet disease. Melek Kechida, Magdalena Bazewicz, Wijdene Nabi and Syrine Daadaa drafted the manuscript. François Willermain, Nesrine Abroug, Dorine Makhoul, and Imen Ksiaa provided critical manuscript revisions and supervised the study. Bechir Jelliti, Sana Khochtali and Moncef Khairallah made a significant contribution to the work, reviewed and agreed on all versions of the article before submission, during revision, the final version accepted for publication, and any significant changes introduced at the proofing stage. All authors read and approved the final manuscript.

Additional information

Funding

This work has been supported by the Ministry of Higher Education and Research of Tunisia