Uveitis in Behçet disease - an update

Abstract

Purpose of reviewUveitis is a major manifestation of Behc ¸et disease (BD) and potentially has a high morbidity. This article reviews recently published data on BD uveitis. Recent findingsA set of classification criteria and a diagnostic algorithm have been developed for BD uveitis. Recent reports have confirmed male predominance and posterior segment inflammation in the majority of BD uveitis patients. A high uveitis attack severity score, fluorescein angiographic leakage at the posterior pole, and disruption of outer retinal layers on optical coherence tomography (OCT) predict poor visual outcome. OCT-angiography studies have suggested subclinical changes of retinal capillaries in patients with or without ocular involvement. In a randomized controlled trial, interferon-a was superior to cyclosporine. Favorable outcomes were reported with earlier initiation, optimization, and withdrawal of infliximab after remission. Adalimumab as first-line was superior to conventional therapy. SummaryClassification criteria will be used to select a homogeneous group of patients for research and the diagnostic algorithm may help ophthalmologists predict the probability of BD uveitis based on ocular findings. Fluorescein angiography and OCT are the routine imaging modalities. Clinical relevance of OCT-angiography is unclear. Interferon-a, infliximab, and adalimumab have proven superior efficacy compared to conventional therapy.

Augenbeteiligung bei ausgewählten rheumatischen Erkrankungen – Klinik im Erwachsenenalter

“…In Germany, Behçetʼs disease is certainly the most relevant vasculitis in terms of the frequency of ocular involvement (affecting approximately 2 2 ⁄3 3 of all patients) [44,45]. The most common ocular manifestation is uveitis, manifesting either as acute anterior uveitis with a high degree of inflammation ("hypopyon iritis") or as panuveitis with occlusive retinal vasculitis.…”

“…In a few patients, low-dose oral steroids are sufficient as long-term relapse prophylaxis for posterior uveitis or panuveitis. As a rule, however, steroid-sparing DMARD therapy is indicated in order to prevent complications and recurrences and to ensure a good visual prognosis in the often young patients [45]. From an ophthalmological point of view, effective csDMARDs include azathioprine, MTX, or CsA, with azathioprine being preferred from a rheumatological perspective; CsA at therapeutically effective doses is often associated with typical adverse effects (nephrotoxicity, arterial hypertension, etc.).…”

“…Das Behçet-Syndrom ist in Deutschland die sicherlich relevanteste Vaskulitis bezogen auf die Häufigkeit der Augenbeteiligung (ca. 2 2 ⁄3 3 aller Patienten) [44,45]. Häufigste okuläre Manifestation ist die Uveitis, entweder als akute anteriore Uveitis mit hohem Entzündungsgrad ("Hypopyoniritis") oder als Panuveitis mit okklusiver retinaler Vaskulitis.…”

“…Bei wenigen Patienten sind niedrig dosierte orale Steroide als langfristige Rezidivprophylaxe bei posteriorer oder Panuveitis ausreichend. In der Regel besteht jedoch die Indikation zur steroidsparenden DMARD-Therapie, um Komplikationen und Rezidive zu verhindern und bei den oft jungen Patienten eine gute Visusprognose zu sichern [45]. Aus ophthalmologischer…”

“…Klassifikationskriterien der EULAR (European Alliance of Associations for Rheumatology) für das Sjögren-Syndrom (modifiziert nach[45]). Die Diagnose gilt als gesichert bei ≥ 4 Punkten nach Anwendung der Ein-und Ausschlusskriterien.Befund Score histologischer Nachweis einer fokalen lymphozytären Sialadenitis im Biopsat einer Speicheldrüse der Unterlippe; Fokus-Score ≥ 3-monatiges Bestehen einer Trockenheit von Augen und/oder Mundschleimhaut.…”