Fuchs Uveitis Syndrome in the Developing World

Hovakimyan, Anna; Baglivo, Edoardo; Cunningham, Emmett T.

doi:10.1097/iio.0b013e3181d2cd4c

Cited by 7 publications

(8 citation statements)

References 48 publications

(73 reference statements)

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“…The typical distribution of KPs occurs in the inferior half of the cornea as a triangle with an inferior base (Arlt's triangle). By contrast, a hallmark finding in Fuch's heterochromatic iridocyclitis (FHI) is fine KPs distributed throughout the cornea [26]. Features of KPs generally do not differentiate most cases of infectious from noninfectious uveitis, although diffuse fine KPs or localized KP, not in Arlt's triangle distribution, may suggest infectious, particularly viral, etiologies.…”

Section: Clinical Examinationmentioning

confidence: 94%

“…Fuch's heterochromic iridocyclitis is typically a chronic, unilateral (>90%) AU associated with minimal pain, redness and anterior chamber cell/flare [26,55]. Some of the unique features of FHI are the small, scattered KP that are not confined to the inferior portion of the cornea and the iris atrophy that leads to a secondary, acquired heterochromia.…”

Section: Anterior Uveitismentioning

confidence: 97%

“…The conjunctiva is often inflamed in cases of AAU, but is less commonly involved in chronic and posterior uveitis. In cases of juvenile idiopathic arthritis (JIA) there are often no symptoms and no conjunctival involvement, yet there are often profound and devastating uveitis complications [17,[23][24][25][26][27]. These children must be thoroughly examined on a regular schedule in order to screen for intraocular inflammation [28].…”

Section: Clinical Examinationmentioning

confidence: 98%

“…In unilateral anterior uveitis (AU), considerations would include an underlying viral infection, FHI or idiopathic etiology [26,[53][54][55]. Viral etiologies for AU include herpes zoster, Herpes simplex and cytomegalovirus (CMV).…”

Section: Anterior Uveitismentioning

confidence: 99%

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Practical diagnostic approach to uveitis

Grillo

Levinson

Gordon³

2011

Expert Review of Ophthalmology

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Accounting for approximately 10% of blindness in the USA, uveitis refers to a group of heterogeneous diseases that share the features of intraocular inflammation but whose etiologies include idiopathic autoimmune disease, infections, rheumatologic diseases and masquerade syndromes. Correct diagnosis with timely and appropriate therapy is key to reducing diseaseassociated morbidity. The multitude of possible causes can create diagnostic challenges, but a successful approach includes a targeted history of the illness and associated symptoms, detailed ocular evaluation and specific laboratory investigations. The purpose of this article is to aid in the initial evaluation and decision-making strategy for uveitis according to the Standardization of Uveitis Nomenclature Working Group classification scheme and to provide a brief look at the anticipated future for diagnostic tools.

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Section: Clinical Examinationmentioning

confidence: 94%

Section: Anterior Uveitismentioning

confidence: 97%

Section: Clinical Examinationmentioning

confidence: 98%

Section: Anterior Uveitismentioning

confidence: 99%

See 2 more Smart Citations

Practical diagnostic approach to uveitis

Grillo

Levinson

Gordon³

2011

Expert Review of Ophthalmology

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“…It has been found that cellular and humoral immune responses towards retinal antigens in patients with RP provoke a mild and chronic inflammatory reaction thought to be responsible for some of its clinical findings: namely macular edema and vascular leakage [1]. Other studies have demonstrated the presence of B-cells against corneal, retinal and anterior segment antigens in the aqueous of patients with FHI [4, 5]. …”

Section: Introductionmentioning

confidence: 99%

The unusual association of inverse retinitis pigmentosa and Fuchs’ heterochromic iridocyclitis

et al. 2017

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BackgroundClassic retinitis pigmentosa (RP) and other syndromic variants have previously been associated to Fuchs’ heterochromic iridocyclitis (FHI). Common immunogenic and inflammatory pathways have been proposed to explain the higher incidence of this uveitic phenomenon in patients with retinal dystrophies without definitive answers. Infrequent variants of RP such as inverse RP have not been previously reported in association with FHI. We believe that finding the way these entities connect can shed some light into their complex pathogenesis and help find ways to foresee and prevent the appearance of complications such as cataract and macular edema.Case presentationWe present a 15 year old mexican male with history of nyctalopia and rapid, progressive visual loss since infancy who had profound hyper and hypopigmented retinal pigment epithelium changes in the posterior pole together with pigment clumping in the macula of both eyes and an electroretinogram pattern consistent of rod-cone dystrophy. He was diagnosed with inverse RP. Three years after his first visit he was found to have a mild asymptomatic non granulomatous anterior uveitis in the right eye with fine stellate keratic precipitates and subtle iris stromal atrophy not associated with iris synechiae and without evidence of posterior uveitis or findings consistent with infectious etiology. Findings were consistent with FHI. As the patient was normotensive, the lens was transparent and there was no clinical evidence of macular edema, the patient was kept under observation without treatment.ConclusionsPatients with RP are prone to develop chronic, low grade inflammation responses similar to the ones present in FHI. This association makes us believe that immunogenetic pathways involved in the degenerative process that leads to photoreceptor loss may become a target in the prevention and treatment of inflammatory complications in RP and disease progression. It also suggests FHI may be a triggered response predisposed by an unidentified genetic factor that may be related to genes affected in RP and thus be identified before irreversible complications such as glaucoma occur.

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