Ocular sarcoidosis: clinical experience and recent pathogenetic and therapeutic advancements

Rosanna Dammacco,Tero T Kivelä,Dario Sisto,Jyotirmay Biswas,Alberto Mavilio,Patrizia Leone,Francesco Alfredo Zito,Franco Dammacco,Giovanni Alessio

doi:10.1007/s10792-020-01531-0

Abstract

PurposeTo describe the ocular manifestations in a cohort of patients with systemic sarcoidosis (SS). Recent advances in the pathophysiology, diagnosis, and therapy of SS are also discussed.MethodsData from 115 Italian patients diagnosed between 2005 and 2016 were retrospectively reviewed. All but the first 17 patients underwent a comprehensive ophthalmologic examination. The diagnosis was based on clinical features, the demonstration of non-caseating granulomas in biopsies from involved organs, and multiple imaging techniques. Data on broncho-alveolar lavage fluid analysis, calcemia, calciuria, serum angiotensin-converting enzyme levels and soluble interleukin-2 receptor levels were retrieved when available.ResultsOcular involvement, detected in 33 patients (28.7%), was bilateral in 29 (87.9%) and the presenting feature in 13 (39.4%). Anterior uveitis was diagnosed in 12 patients (36.4%), Löfgren syndrome and uveoparotid fever in one patient each (3%), intermediate uveitis in 3 patients (9.1%), posterior uveitis in 7 (21.2%), and panuveitis in 9 (27.3%). First-line therapy consisted of corticosteroids, administered as eyedrops (10 patients), sub-Tenon’s injections (1 patient), intravitreal implants (9 patients), or systemically (23 patients). Second-line therapy consisted of steroid-sparing immunosuppressants, including methotrexate (10 patients) and azathioprine (10 patients). Based on pathogenetic indications that tumor necrosis factor (TNF)-α is a central mediator of granuloma formation, adalimumab, targeting TNF-α, was employed in 6 patients as a third-line agent for severe/refractory chronic sarcoidosis.ConclusionUveitis of protean type, onset, duration, and course remains the most frequent ocular manifestation of SS. Diagnostic and therapeutic advancements have remarkably improved the overall visual prognosis. An ophthalmologist should be a constant component in the multidisciplinary approach to the treatment of this often challenging but intriguing disease.

Highlights

Sarcoidosis is a chronic, multisystem, non-caseating granulomatous disease of unknown etiology
Based on pathogenetic indications that tumor necrosis factor (TNF)-a is a central mediator of granuloma formation, adalimumab, targeting TNF
All segments of the eye and its adnexa can be involved, the most common sarcoidosis-related ocular disease is uveitis that may result in permanent visual impairment if not timely diagnosed and properly treated [14,15,16]

Summary

Introduction

Sarcoidosis is a chronic, multisystem, non-caseating granulomatous disease of unknown etiology. Sarcoidosis differs in patients of different ethnicities in terms of its clinical variability and visual outcome [1, 2], among those in whom clinical expression is characterized by acute onset with erythema nodosum and/or asymptomatic bilateral hilar lymphadenopathy the course is usually self-limiting and spontaneous resolution, mostly within the first 2–3 years after the diagnosis, occurs in one-third to half of the patients [3, 4] Those with an insidious onset and slow progression will eventually develop a multi-systemic disorder that includes lung involvement and multiple extra-pulmonary lesions [5]. Ocular manifestations may be the presenting sign of sarcoidosis in 11–30% of patients [10, 11] who will later develop extra-ocular disease, but ocular findings may be recognized at any time during the course of SS [12, 13]

Objectives

Methods

Results

Discussion

Conclusion