There is a genetic bridge between Asia and the Native American Indian related to the migration of peoples across the Bering Strait. Russell W. Read, MD, a fellow in uveitis and ophthalmic pathology at the Doheny Eye Institute in Los Angeles, served as program secretary for the workshop. Read said. At least one U. Read is hopeful that current diagnostic guidelines can be revised to account for both the acute onset and chronic stage of the disease. It also affects the central nervous system [CNS], the inner ear and the cutaneous system.
Nonetheless, Howard H. Tessler, MD, a professor of ophthalmology at the University of Illinois in Chicago, said that in most cases VKH is not a difficult disease to diagnose.
Patients can have neurological symptoms, specifically meningitis-type symptoms. VKH often becomes bilateral, with patients experiencing blurred vision. Tessler said. Patients also usually develop exudative retinal detachments. In fact, Dr. Tessler believes that exudative retinal detachment is probably the most prominent characteristic of the disease.
In addition, there are at least four distinct phases of VKH syndrome: 1 prodromal stage: neurologic symptoms only headache, tinnitus, meningismus ; 2 uveitis stage: exudative retinal detachment, vitritis, disk hyperemia; 3 chronic stage: retinal pigmentary changes, sunset glow fundus, Dalen-Fuchs nodules, Sugiura sign; 4 recurrent stage: predominately anterior uveitis.
This frequently occurs months after the disease initially begins. Tessler said that it is important that VKH be accurately diagnosed early on.
Fluorescein angiography is one of the laboratory investigations that should be routinely conducted in clinically suspected cases of VKH syndrome. The four stages of VKH are the prodromal stage , uveitic stage , chronic stage and chronic recurrent stage.
The prodromal stage symptoms will resemble a viral illness. Headaches, fever, orbital pain, nausea, dizziness and light sensitivity are present.
The symptoms will last around days. Within the first couple of days the patient will begin to complain about blurred vision, photophobia, hyperemia of the conjunctiva, and ocular pain. Eventually, the inflammation becomes more diffuse affecting the anterior chamber, presenting itself as a panuveitis. The chronic or convalescent stage will take place weeks after the uveitic stage. It is characterized by the development of vitiligo, poliosis and depigmentation of the choroid.
The recurrent stage consists of a panuveitis with acute exacerbations of anterior uveitis. Recurrent posterior uveitis with exudative retinal detachment is uncommon. Iris nodules may appear in this stage. Read et al evaluated the existing criteria and concluded that it was inadequate for the diagnosis of VKH. Incomplete Vogt-Koyanagi-Harada disease criteria 1 to 3 and either 4 or 5 must be present.
Probable Vogt-Koyanagi-Harada disease isolated ocular disease; criteria 1 to 3 must be present. In the majority of cases the diagnosis of VKH will be based on clinical findings.
The following testing modalities are utilized to assist in the diagnosis and to follow the response to treatment. During the acute stage of the disease we find an early irregular focal or patchy fluorescence of the choroidal circulation. There exist various pinpoint areas of leakage at the level of the retinal pigment epithelium. In the later phases the localized hyperfluorescent spots increase in size, coalesce, and expand into the subretinal space in areas of serous detachment leading to a large area of leakage.
The optic disc can demonstrate blurred fluorescent margins accompanied by late leakage. Soon-Phaik Chee et al report findings in their retrospective study that indicate the importance of early pinpoint peripapillary hyperfluorescence as a prognostic factor in VKH.
The absence of this sign suggests that the disease is no longer in its early stages; indicating the need of more aggressive and prolonged treatment to prevent future recurrences.
ICG demonstrates an early choroidal stromal vessel hyperfluorescence and hypofluorescent dark spots during the early and midphase, distributed mainly posteriorly, and in excess of those seen clinically on FFA. The late phase will vary according to the current stage of the disease. During the active stage, the hypofluorescent spots fade and are replaced by hyperfluorescent ones representing focal sites of active choroidal inflammation.
In the chronic stage, the hypofluorescent dark spots are seen during all the phases of ICG, but they are unapparent during clinical or FFA evaluation. In the active stage of the disease, FAF will show hyperautofluorescence in the macula with hypoautofluorescence in the areas of the serous detachment; returning to normal at 6 months after treatment is initiated.
During the chronic stage many different patterns can occur: decreased autofluorescence due to peripapillary atrophy and multiple atrophic and pigmented scars , increased autofluorescence patches or irregular areas of pigmentation and cystoid macular edema and normal autofluorescence.
An OCT will demonstrate the presence of subretinal fluid. Typically the inner retina inward to the external limiting membrane is normal. There is increased choroidal thickness in acute stage. During the acute stages of the disease; the ultrasound will present diffuse choroidal thickening with low to medium reflectivity, serous retinal detachments, vitreous opacities without posterior vitreous detachments and scleral or episcleral thickening. Full-field electroretinography analysis demonstrated diffusely diminished amplitudes in both scotopic and photopic phases in patients in the chronic stages.
Spinal fluid examination reveals evidence of pleocytosis which may persist for up to 8 weeks [11] and elevation of protein levels in the early stages. Scalp hair, thinning. Sparse, thin scalp hair. Blotchy loss of skin color. Clouding of the lens of the eye. Cloudy lens. Detached retina. Decreased body height.
Small stature. Impaired vision. Loss of eyesight. Poor vision. Do you have more information about symptoms of this disease? We want to hear from you. Cause Cause. It is thought to be due to an abnormal immune response in which the body attacks its own pigment cells melanocytes. In addition, genetic factors may play a role. Diagnosis Diagnosis. Vogt-Koyanagi-Harada VKH disease is diagnosed based on the symptoms, clinical exam, eye exam, and imaging studies.
In addition, other more common conditions may need to be excluded before VKH disease can be diagnosed. Treatment Treatment. Treatment usually involves early and aggressive treatment with systemic corticosteroids steroids. Other medications may be used as well. Statistics Statistics. The incidence may be higher in other countries. Do you have updated information on this disease? Research Research. Clinical Research Resources ClinicalTrials. Click on the link to go to ClinicalTrials.
Please note: Studies listed on the ClinicalTrials. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study. Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition. Organizations Organizations. Organizations Supporting this Disease.
Organizations Providing General Support. Do you know of an organization? Learn More Learn More. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them. In-Depth Information Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health.
Visit the website to explore the biology of this condition. Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge. PubMed is a searchable database of medical literature and lists journal articles that discuss Vogt-Koyanagi-Harada disease.
0コメント