Bilateral Iris Depigmentation and Ocular Hypotony as End-Stage Manifestations of Untreated Vogt–Koyanagi–Harada Disease

Cuevas, Miguel; de-la-Torre, Alejandra; Córdoba, Andrea

doi:10.1080/09273948.2017.1320411

Cited by 6 publications

(4 citation statements)

References 16 publications

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“…Very few cases have been reported in the literature of patients with VKH disease presenting with bilateral iris depigmentation and hypotony. [ 3 4 5 ] All these reported cases were inadequately treated for the disease in the acute phase. The case we reported was also not diagnosed as VKH previously and was never treated with oral steroids and immunosuppressants.…”

Section: Discussionmentioning

confidence: 99%

Bilateral Iris depigmentation, transillumination defects and hypotony in Vogt–Koyanagi–Harada disease

Garg

Das²,

Bhattacharjee³

et al. 2022

Indian Journal of Ophthalmology

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Section: Discussionmentioning

confidence: 99%

Bilateral Iris depigmentation, transillumination defects and hypotony in Vogt–Koyanagi–Harada disease

Garg

Das²,

Bhattacharjee³

et al. 2022

Indian Journal of Ophthalmology

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“…Crossing knowledge boundaries, which are often not clearly defined in such disorders, is essential to reach the goal of early diagnosis and treatment which, in turn, significantly affects prognosis, avoiding long-term complications such as severe iris depigmentation and persistent ocular hypotony, which have been described in South American patients [14]. VKH is a rare disease in Europe.…”

Section: Discussionmentioning

confidence: 99%

Differentiating Vogt-Koyanagi-Harada syndrome from recurrent optic neuritis: a case report and review of the literature concerning Hispanic patients

Scarioni

Pietroboni

Invernizzi

et al. 2017

Mult Scler Demyelinating Disord

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Background: First recognized at the beginning of twentieth century and named after three authors who independently described some affected patients, Vogt-Koyanagi-Harada syndrome is a rare multisystemic autoimmune disease targeting melanin-containing tissues of the eye, meninges, inner ear and skin. It predominantly affects Asian people, but also people with darker skin pigmentation such as Native Americans and Hispanics (Mestizos), whose ancestors moved from Asia across the Bering strait to North America and further down to Central and South America. Heterogenous presentation is observed, especially among different ethnic groups. Here we describe the case of an Hispanic South American patient presenting with multiple visual relapses and thus mimicking recurrent optic neuritis; we provide insights into the differential diagnosis and a brief review of the literature concerning the epidemiology of Vogt-Koyanagi-Harada syndrome in Hispanic patients compared with other ethnic groups. Case presentation: A 34-year-old Ecuadorian woman presented over years with multiple relapses involving the visual system. She was investigated in both neurologic and ophthalmic clinical settings. Brain Magnetic Resonance Imaging, cerebrospinal fluid examination, Spectral Domain Optical Coherence Tomography and Fluorescein Angiography were performed. She was misdiagnosed first as an optic neuritis pointing to a demyelinating disorder, then as a posterior scleritis. Due to the protean manifestations of Vogt-Koyanagi-Harada syndrome and the incomplete clinical presentation at the beginning, the right diagnosis was made only at a later disease stage using retrospective criteria. Conclusions: Hispanic patients often present without extraocular symptoms in early phases of the disease and they have globally lower rates of intertegumentary signs compared to Asian patients. The diagnosis of a multisystemic disease such as Vogt-Koyanagi-Harada syndrome is a challenge involving specialists operating in different medical fields; especially in urban multiethnic populations, rare etiologies of common symptoms have to be taken into account when performing a differential diagnosis.

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“…Exudative retinal detachment is the hallmark of acute disease while the sunset glow fundus is a unique feature of chronic disease. [1][2][3][4] The treatment of choice for VKH disease is systemic high-dose corticosteroids in combination with immunosuppressive agents followed by a slow tapering of the drugs over at least 3-6 months to suppress intraocular in ammation and prevent further recurrence or chronicity. While appropriate corticosteroid therapy may adequately control the condition in some cases, recurrent and chronic disease is common.…”

Section: Introductionmentioning

confidence: 99%

Long-Term Efficacy of Dexamethasone Intravitreal Implant in the Treatment of Vogt-Koyanagi-Harada (VKH) Disease Relapsing Posterior Uveitis

Elhamaky¹

2021

Preprint

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Purpose: To investigate the efficacy and safety of dexamethasone intravitreal implant in the treatment of relapsing posterior uveitis in patients with chronic recurrent VKH disease.Methods: This is a prospective study of 29 eyes of 16 patients with posterior uveitis in chronic recurrent VKH disease. All patients received previous systemic steroid and immunosuppressive regimens. All patients underwent a comprehensive ophthalmic examination, including BCVA (log MAR), IOP, FFA, and SD-OCT. All patients underwent intravitreal injection with sustained-release dexamethasone 0.7 mg implant (Ozurdex®). Primary outcome measures included mean change in best corrected visual acuity (BCVA) and central foveal thickness (CFT) at 24 months follow-up compared to baseline. Results: At 24 months follow-up, the mean BCVA improved from 0.82 ± 0.13 to 0.38 ± 0.06logMAR (P <0.0001). The mean CFT reduced from 505 ± 29 to 244 ± 23 um (P <0.0001). The mean IOP changed from 15.1±2.2 to16.9±3.1 mmHg with no significant value. Twenty-one eyes (72.4%) received one injection, while eight eyes (27.6%) required two injections. The mean number of injections was 1.2± 0.60. The mean follow-up time was 24.75± 0.9months. No serious ocular or systemic adverse events were noted during the follow-up period. Ocular hypertension was recorded in there (10.3%) eyes and controlled by IOP lowering medications. Cataract progression occurred in 11 (37.9%) eyes.Conclusions: Our cohort highlights the beneficial effects of the Dexamethasone implant 0.7 mg in the treatment of VKH disease relapsing posterior uveitis improving visual acuity, reducing macular edema, and minimizing the burden of systemic steroid in this sample study.

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Bilateral Iris Depigmentation and Ocular Hypotony as End-Stage Manifestations of Untreated Vogt–Koyanagi–Harada Disease

Abstract: Early diagnosis and treatment of inflammation are crucial factors in the clinical outcome of Vogt-Koyanagi-Harada disease. When left undiagnosed and untreated from early stages, severe iris depigmentation and ocular hypotony, uncommon manifestations of this disease, can develop.

Cited by 6 publications

References 16 publications

Bilateral Iris depigmentation, transillumination defects and hypotony in Vogt–Koyanagi–Harada disease

Bilateral Iris depigmentation, transillumination defects and hypotony in Vogt–Koyanagi–Harada disease

Differentiating Vogt-Koyanagi-Harada syndrome from recurrent optic neuritis: a case report and review of the literature concerning Hispanic patients

Long-Term Efficacy of Dexamethasone Intravitreal Implant in the Treatment of Vogt-Koyanagi-Harada (VKH) Disease Relapsing Posterior Uveitis

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