Retinal and optic disc vasculitis in Susac's syndrome

Sánchez-Vicente, J.L.; Molina-Socola, F.E.; Medina-Tapia, A.; Moruno-Rodríguez, A.; Gálvez-Carvajal, S.; López-Herrero, F.

doi:10.1016/j.oftale.2018.01.003

Cited by 2 publications

(1 citation statement)

References 9 publications

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“…[95] Optik disk anjiyopatisi de vakaların az bir kısmında Susac sendromu ile ilişkili olarak tanımlanmıştır. [99] Yapılan bir çalışmada, immünosupresif ve immünomodülasyon tedavileri altında sistemik oküler Susac sendromu olan hastalıklarla ilgili kapsamlı bir vaka serisi sunmaktadır. Bu çalışma da, daha önce bildirilmeyen gözlerin çoğunda retinal mikroanevrizmalarının varlığı oküler bulgu olarak gösterilmiştir.…”

Section: Susac Sendromuunclassified

Autoimmune Diseases and Retina

2023

Güncel Retina (Current Retina)

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Autoimmune diseases are a group of diseases that can result in organ-specific and/or systemic (systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis,...) anatomical deformation and loss of function as a result of the body's immune system attacking its own antigens under the influence of genetic and environmental factors. The eye is a potential target for complications related to autoimmunity, and depending on the severity of the immune response, vision-threatening problems can be observed. Immune complexes accumulate in and around the vessel walls as a result of humoral immunity, in which the autoantibody-related complement system is activated, and cellular immune response can lead to retinal vasculitis, vaso-occlusion, retinal hemorrhage, retinal ischemia, ischemic optic neuropathy, and inflammation of periorbital tissues. Therefore, the role of ophthalmologists in disease management is important in diagnosing, investigating, and treating ocular symptoms in a timely manner. Early diagnosis and treatment can play a critical role to decrease the risk of irreversible immunological damage to the retinal tissue. A multi-disciplinary approach for complete management and evaluation is needed in this disease group. This study aimed to provide a detailed review of the current clinical perspective for the diagnosis, workup, and treatment of autoimmune diseases with retinal manifestations. Systemic lupus erythematosus, Polyarteritis Nodosa, Dermatomyositis, Scleroderma, Wegener's Granulomatosis, Churg-Strauss Syndrome, Takayasu Arteritis, Susac Syndrome, and Nodular Panniculitis are discussed under this heading.

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Section: Susac Sendromuunclassified

Autoimmune Diseases and Retina

2023

Güncel Retina (Current Retina)

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Susac’s syndrome: an update

Sauma¹,

Rivera

et al. 2020

Br J Ophthalmol

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Susac’s syndrome (SS) is a relatively rare cause of multiple recurrent branch retinal arterial occlusions (BRAO). SS is frequently misdiagnosed and probably underdiagnosed. Ophthalmic manifestations may be the sole presenting sign of SS. Comprehensive literature review The typical triad of encephalopathy, sensorineural hearing loss and multiple recurrent BRAO is seldom seen at presentation in SS. The characteristic ophthalmological feature in SS is the presence of recurrent multiple BRAO in the absence of intraocular inflammation. Yellow to yellow-white, non-refractile or refractile retinal arterial wall plaques (Gass plaques) found at midarteriolar segments not associated to bifurcations are commonly found in SS. Because of its ability to capture more peripheral retinal area, ultrawide field fluorescein angiography (FA) has definite advantages over conventional FA and its use should be encouraged in patients suspected of having SS. Optical coherence tomography (OCT), particularly spectral domain OCT complements FA. Patients with BRAO and hearing loss that do not develop encephalopathy during the initial 2 years will most likely not develop encephalopathy. However, these patients will be prone to recurrent BRAO and hearing loss. Imunosuppression is the cornerstone of treatment but the best regimen still needs to be defined. A minority of patients with SS present with the typical triad. A high index of suspicion is needed to make the diagnosis promptly. Early diagnosis and treatment are important to delay disease progression and prevent blindness, deafness and dementia.

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Retinal and optic disc vasculitis in Susac's syndrome

Cited by 2 publications

References 9 publications

Autoimmune Diseases and Retina

Autoimmune Diseases and Retina

Susac’s syndrome: an update

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