Vogtkoyanagiharada syndrome vkh syndrome is an uncommon multisystem disease of presumed autoimmune etiology that is characterized by chronic, bilateral, diffuse, granulomatous uveitis with accompanying dermatologic, neurologic, and auditory involvement. Vogt koyanagi harada s syndrome is a rare disease that affects tissues containing melanocytes, such as the eyes, central nervous system, inner ear and skin. There are 4 stages of vkh disease, including prodromal, acute uveitis, convalescent. We retrospectively evaluated patients diagnosed as having vkh disease with exudative retinal detachment at the osaka university hospital or the japanese community healthcare organization, osaka hospital, between 1998 and 2012. Identification of underlying inflammation in vogtkoyanagiharada disease with sunset glow fundus by multiple analyses. A tribute to syo kurokawa 19262010 the lichenologist. Theory of the optical parametric oscillator in the quadratic. Vogtkoyanagiharada disease is initially characterized by headaches, very deep pain in the eyes, dizziness vertigo, and nausea. Vogtkoyanagiharada syndrome vkh syndrome is an autoimmune disease in humans where ones own defense against infection, the tcells, attack the melaninforming cells melanocytes in the body. Vogtkoyanagiharada vkh disease is a multisystem autoimmune inflammatory disorder with ocular, auditory, skin and neurologic involvement. The symptoms of the vogt koyanagi syndrome overlap those of harada diseasesyndrome, and they may at times be indistinguishable from harada s disease. Pdf on aug 1, 2014, emmett t cunningham and others published vogtkoyanagiharada disease find, read and cite all the research you need on researchgate. Vogtkoyanagiharada syndrome is an uncommon multisystem inflammatory disorder characterized by panuveitis with serous retinal detachments, and it is.
Introduction vogt koyanagi harada vkh disease is a panuveitis of autoimmune nature where the melanocytes act both as an inducer and as a target. Vogtkoyanagiharada disease genetic and rare diseases. Seven eyes of 6 patients underwent surgical treatment. Vogtkoyanagiharadas disease is a rare syndrome that affects tissues with. Vogtkoyanagiharada disease johns hopkins university. It is a bilateral granulomatous panuveitis associated with dermatologic and neurologic manifestations, including vitiligo, alopecia, poliosis, and meningeal signs. Chapter 177 vogtkoyanagiharada disease free medical. The most noticeable symptom is a rapid loss of vision.
Vogtkoyanagiharada vkh disease is a multisystemic inflammatory autoimmune disorder that is characterized by panuveitis with iridocyclitis, serous retinal detachments, diffuse choroidal swelling and optic disc hyperemia. The signs and symptoms of vkh disease are caused by chronic inflammation of melanocytes. This may occur in both eyes at the same time or in one eye first and, a few days later, in the other. Vogtkoyanagiharada syndrome with more emphasis on the. Vogtkoyanagiharada disease vkh is a multisystem disease of presumed autoimmune cause that affects pigmented tissues, which have melanin. It is characterized by diffuse granulomatous inflammation involving various organs including eye. Vkh syndrome is named for ophthalmologists alfred vogt from switzerland and yoshizo. Vogtkoyanagiharada syndrome nederlands tijdschrift voor. Analysis of 87 cases with vogtkoyanagiharada disease. Introduction vogtkoyanagiharada vkh disease is a multisystemic disorder characterized by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations.
Vogtkoyanagiharada disease vkhd is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear. Vogtkoyanagiharadas syndrome and its multisystem involvement. Vogt koyanagi harada disease is a rare disorder of unknown origin that affects many body systems, including as the eyes, ears, skin, and the covering of the brain and spinal cord the meninges. There may also be neurological signs such as severe headache, vertigo, nausea, and drowsiness. Disease definition vogt koyanagi harada disease is a bilateral, chronic, diffuse granulomatous panuveitis typically characterized by serous retinal detachment and frequently associated with neurological meningitis, auditory, and dermatological alterations. The retina may detach and hearing loss may become apparent. The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes.
Mar 26, 2015 our aim was to determine the recurrence rate of inflammation in vogt koyanagi harada vkh disease and to describe its clinical features. Aug 17, 2018 vogtkoyanagiharada vkh disease is a multisystem autoimmune inflammatory disorder with ocular, auditory, skin and neurologic involvement. Vkh disease occurs more commonly in patients with a genetic predisposition to the disease, including those from asian, middle eastern, hispanic, and native american populations. Vogt koyanagi harada disease is an infrequent, systemic syndrome that evolves in stages and compromises organs containing melanocytes. This inflammatory syndrome is probably the result of an autoimmune mechanism, influenced by genetic factors, and appears to be directed against melanocytes. Vogtkoyanagiharadas syndrome is a rare disease that affects tissues. Vogt koyanagi harada disease, a severe bilateral granulomatous intraocular inflammation associated with serous retinal detachments, disk edema, and vitritis, with eventual development of a sunset glow fundus, is an autoimmune inflammatory condition mediated by t cells that target melanocytes in individuals susceptible to the disease. The vogtkoyanagiharada syndrome vkh is a bilateral, diffuse granulomatous uveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. An lband grapheneoxide modelocked fiber laser delivering. These symptoms are usually followed in a few weeks by eye inflammation uveitis and blurring of vision. The authors constitute the international committee on vogt koyanagi harada disease nomenclature, representing the participants of the first international workshop on vogt koyanagi harada disease. Read rw, holland gn, rao na, tabbara kf, ohno s, arellanesgarcia l, et al.
Age and sex distribution of vogtkoyanagiharada disease in indian clinic. Revised diagnostic criteria for vogt koyanagi harada disease rajendram r, evans m, rao a n. No history of penetrating ocular trauma or surgery preceding the initial onset of uveitis 2. Melanin is what gives color, or pigment, to our hair, skin and parts of our eyes. Vogtkoyanagiharada syndrome article about vogtkoyanagi. The disease is characterised by bilateral uveitis associated with a varying constellation of auditory, neurological and cutaneous manifestations. Vogt koyanagi harada s disease is a rare syndrome that affects tissues with melanocytes like eyes, central nervous system, skin and inner ear. These manifestations are variable and race dependent. Vogt koyanagi harada syndrome vkhs is a rare multisystemic autoimmune disease that affects tissues containing melanin, including the eye, inner ear, meninges, and skin. The syndrome is characterised by a combination of panuveitis, pleocytosis in cerebral spinal fluid with or without neurological signs, alopecia and depigmentation of body hair. Note the exudative detachment of the neural retina due to inflammation in the choroid. Despite the initial treatment, the patient progressed to. Vogtkoyanagiharada vkh disease is defined as a bilateral granulomatous panuveitis with or without extraocular manifestations affecting.
If an exudative choroiditis, causing retinal detachment, is present the condition is called. Vogt koyanagi harada syndrome is a multisystem disorder characterized by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations. Vogtkoyanagiharadalike syndrome in dogs dog breed info. Vogtkoyanagiharada syndrome radiology reference article. It may be associated with neurologic and cutaneous manifestations. Some ethnic groups have a higher probability of developing the disease, including asians, indians and latin americans and females are affected more often. Oct 30, 2015 vogt koyanagi harada vkh disease is a multisystemic inflammatory autoimmune disorder that is characterized by panuveitis with iridocyclitis, serous retinal detachments, diffuse choroidal swelling and optic disc hyperemia. Pdf a u g u s t 3 1, 2 0 1 0 v o l u m e 9 n u m b e r 1 6 contemporary vogtkoyanagiharada vkh syndrome is a multisystemic immune disorder. Aug 17, 2018 vogt koyanagi harada syndrome in children. Complicated cataract, glaucoma, panuveitis, vogt koyanagi harada disease. The condition has several extraocular manifestations, and is a common uveitic entity in japanese, relatively less com. Vogt koyanagi harada syndrome vkh is a systemic inflammatory disorder that predominantly affects individuals with darker skin pigmentation and shows a slight female predilection. Jan 21, 2011 figure 1771 fundus in the uveitic phase of vogt koyanagi harada disease.
The vogt koyanagi harada syndrome vkhs is rare in the netherlands. Beim vogtkoyanagiharadasyndrom handelt es sich um eine multisystemische erkrankung, deren primares symptom eine beidseitige, chronische uveitis ist. Available formats pdf please select a format to send. Revised diagnostic criteria for vogt koyanagi harada disease.
New criteria, taking into account the multisystem nature of vogt koyanagi harada disease, with allowance for the different ocular findings. Typically, patients experience four different phases. Childhood unilateral vogt koyanagi harada syndrome. Identify and treat vogtkoyanagiharada syndrome american. Vkh pode tambem afetar a orelha interna, reduzindo a audicao, e afetar a pele e as meninges do sistema nervoso. Vogtkoyanagiharada disease nord national organization. Vogtkoyanagiharada syndrome vkhs is a disease entity consisting of an inflammatory reac tion of the uvea, the retinal pigment epithelium and the meninges, variably accompanied by in volvement of brain substance, pathology of the cranial nerves, hair and skin changes in the form. It affects primarily asians, indians and latin americans and also women. The conspicuous feature of the vogt koyanagi syndrome is the severe, prolonged bilateral uveitis. Vogt koyanagi harada syndrome, which is characterized by bilateral granulomatous uveitis, exudative retinal detachment, meningeal symptoms, hearing loss and pigment loss has been reported to regress and in some cases complete resolve during pregnancy and the postpartum period. However, in asia it is one of the most common causes of panuveitis. Vkh may variably also involve the inner ear, with effects on hearing, the skin and the meninges of the central nervous system. The vogt koyanagi harada syndrome vkh is a bilateral, diffuse granulomatous uveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs.
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