Vogt Koyanagi Harada Syndrome

HIDAYATI11109084Vogt Koyanagi Harada Syndrome

Introduction Vogt-Koyanagi-Harada (VKH) syndrome is a systemic autoimmune disease in which the main target is melanin-containing-cells present in the eye,meninges, ear and skinPathogenesis

Pathology and pathogenesisdiffuse thickening of the uveal tract -> non-necrotizing granulomatous inflammationInflammatory cell infiltration, consisting of lymphocytes and focal aggregates of epithelioid histiocytes and multinucleated giant cells, spares the inner layer of the choroid (choriocapillaris) and the overlying retinaThe focal collection of lymphocytes, pigment-laden macrophages, epithelioid cells and proliferated retinal pigment epithelium (RPE) cells -> Daln-Fuchs nodules.retinal detachment with collection of subretinal fluid most likely results from alterations in the RPE as upstream effect of choroidal compromise.The inflammatory cell infiltration may often extend to ciliary body and iris stroma.The convalescent phase of the disease is characterized by a mild to moderate non-granulomatous inflammation with uveal infiltration of lymphocytes, few plasma cells, and occasional macrophages.The choroid is depigmented -> damage of the choroidal melanocytes -> sunset-glow fundusnumerous peripheral choroidal depigmented small atrophic lesions involving the overlying choriocapillaris, the RPE. These atrophic lesions correspond to focal RPE loss with chorioretinal adhesionsIn the chronic recurrent stage, a diffuse uveal infiltration consisting of a granulomatous process, less prominent than in the acute stage. Chorioretinal adhesions, with atrophy and/or proliferation of RPE are common.The RPE proliferation has the clinical appearance of hyperpigmented changes on ophthalmoscopic examination and formation of subretinal neovascular membranes.hyperplastic RPE, devoid of pigmentation, may be clinically reorganized as subretinal fibrosis. In association with the RPE changes, photoreceptor degeneration and gliosis may be observed in the overlying neural retina.Clinical stageThe clinical course of VKH disease follows four stages or phases:prodromic, uveitic, chronic and recurrentProdromal stageThe prodrome typically lasts for a few daysFever, Headache, Meningismus, Nausea, Vertigo, Orbital pain, Tinnitus, CSF pleocytosis, Photophobia, and optic neuritis.

Uveitic stageacute bilateral blurring of visionbilateral posterior uveitis with retinal edema, optic disc hyperemia or edema, and, serous retinal detachments. anterior uveitis characterized by mutton-fat keratic precipitates and iris nodules. The intraocular pressure may be elevated.

Chronic stageocular and dermatologic manifestations Occular Depigmentation of the choroid, Dalen-Fuchs nodulesDermatologicvitiligo and poliosis of the lashes, eyebrows, and hair.

Recurrent stagerecurrent or chronic anterior uveitis, low-grade choroidal inflammation,Recurrent posterior uveitis with serous retinal detachment.Retinal detachment and disc edem

Mutton fat keratitic presipitate

Iris nodule

polyosis

Sunset glow fundusComplicationscataract (1042%) Glaucoma (645%)subretinal fibrosis (840%)neovascular membranes (914%)Complementary ophthalmic examinationsFluorescein angiography (FA) ocular ultrasoundoptic coherence tomography (OCT)Fluorescein AngiographyAcute VKH diseaseMultiple pinpoint areas of leakage at the level of the retinal pigment epithelium (RPE) overlying areas of choroiditis are visible during the arteriovenous phase.Peripapillary pinpoint hyperfluorescence, radial folds of the choroid may be visible as alternating dark and light bands of fluorescence.Multiple serous retinal detachments with pooling of dye. Other, less common findings include retinal vascular leakage and optic disc staining.

Pinpoint area

Pooling of dyeRecovery phase of VKH disease (after treatment with systemic corticosteroids)Most of the acute phase abnormalities, including exudative retinal detachment and disc edema, resolve during this period. Fluorescein angiography may show persistent pinpoint areas of leakage and disc staining. Some patients may exhibit window defects and areas of mottled background hyperfluorescence.Chronic VKH diseasedepigmentation of the choroid, RPE atrophy are visible, such as a moth-eaten appearance, multiple window defects, and areas of alternating hyperfluorescence and hypofluorescence.Additional findings include choroidal neovascularization, retinochoroidal and arteriovenous anastomoses, and neovascularization of the disc. Macular edema is rare in this disorder but may be seen in the chronic phase

FAMidphase is shown on the left, with multiple areas of hyperfluorescence at the level of the retinal pigment epithelium (RPE). Late phase on the same angiogram (right) reveals multiple placoid areas of hyperfluorescence at the level of the RPE and pooling of dye in the areas of serous detachment.

UltrasonographyThe most characteristic ultrasonographic finding is diffuse, low to medium reflective thickening of the posterior choroid. Additional findings include serous retinal detachments, mild thickening of the sclera and/or episclera adjacent to areas of choroidal thickening, and mild vitreous opacities. These ultrasonographic features may be useful in monitoring the patients response to therapy.Patient with progressive dysacusis and recent onset of visual loss. Fundus photo shows a large, multifocal serous detachment of the right eye. B-scan ultrasonography reveals posterior choroidal thickening with an overlying retinal detachment

OCT scanningSerous retinal detachments with subretinal septa may be visible, especially early in the disease. Optical coherence tomography (OCT) scanning may be useful for monitoring serous detachments and response to therapy. Enhanced depth imaging OCT scanning has revealed a markedly thickened choroid in patients with active VKHoptic coherence tomography (OCT) with elevated retinal detachment and subretinal membranes

Diagnosis Complete disease (1 to 5 must be present)No history of penetrarting ocular trauma or surgery preceeding the initial onset of uveitisNo clinical or laboratory evidence suggestive of other ocular disease entities

3. Bilateral ocular involvement (a or b must be met)Early manifestations Diffuse choroiditis (focal areas of subretinal fluid, bullous serous retinal detachments )OR, charateristics fluorescein angiography findings (focal delayed choroidal perfusion, pinpoint leakage, pooling within subretinal fluid, AND echography evidence of diffuse chroidal thickeningLate manifestationsHistory suggestive of prior uveitis with the above described characteristics AND ocular depigmentation (sunset of glow fundus, sugiura sign)AND other ocular signs (numular chorioretinal depigmented scars, retinal pigment epitelium clumping and/or migration, or recurrent or chronic anterior uveitis)

4. Neurological/auditory findingsMeningismus, OR tinnitus, OR cerebrospinal fluid pleocytosis5. Integumentary findingsAlopecia, or poliosis or vitiligoIncomplete disease (1 to 3 and either 4 or 5 must be present)Probable disease (isolated ocular disease; 1 to 3 must be present)

Treatment Corticosteroids should initially be given in high doses, i.e. 12 mg/kg/day of oral prednisone or 1 g/day of intravenous methylprednisolone for 3 days, followed by oral prednisone (1.0 mg/kg/day) with slow tapering along 6 months or more.Steroid is given for 6-9 months prior to tappering offTappering off done by lowering the dose of prednisone 10 mg/day weekly until the dose reached 40 mg/day,followed by reduced 5 mg/day dose weekly to 20 mg/daythen 2.5 mg/day weekly until the dose reached 10 mg/dayNext tappering off done by lowering the dose of 2.5 mg/day per month. Therapy was performed at least as long as six months

Cyclosporine AThe recommended dosage of cyclosporine is 3 to 5 mg/kg/daySustained control of inflammation is observed in approximately 52% of patients within 12 months

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