In 1906, alfred vogt in switzerland first described a patient with premature whitening of eyelashes of sudden onset and bilateral subacute. Uveomeningoencephalitic syndrome vogt koyanagiharanda vkh is an unusual syndrome with important neurological complications and has not, to my knowledge, been discussed in english in the neurological literature. Despite the initial treatment, the patient progressed to. Vogt koyanagi harada syndrome sharmeen akram and khabir ahmad abstract vogt koyanagi harada vkh syndrome is a rare multisystem disease of melanocyte containing organs. It is an autoimmune disease occurring in genetically susceptible individuals and clinically presents as bilateral panuveitis with serous retinal detachments and hyperemic, swollen optic discs, which are. Vogtkoyanagiharada syndrome vkh is a multisystem disease that presents with a combination of ophthalmological, neurological, and dermatological signs and symptoms. Nov 24, 2016 vogtkoyanagiharada syndrome is a cause of noninfectious panuveitis, leading to significant vision loss in many patients. Vogt koyanagi harada vkh syndrome is one such disease that requires imt for its optimal management, and failure to do so portends a poor prognosis 4,5. Oct 26, 2017 vogt koyanagi harada disease vkh disease is a disease that affects several parts of the body, including the eyes, ears, nervous system, and skin. Uveomeningoencephalitic syndrome vogtkoyanagiharada. 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 syndrome vkh is a bilateral granulomatous panuveitis associated with cutaneous, neurological and auditory manifestations 1.
Orbital mr imaging findings included bilateral choroidal and retrobulbar contrast enhancement, while brain findings included white matter abnormalities on flair and leptomeningeal enhancement. Clinical ophthalmology v ogt koyanagiharada syndrome current perspectives abeir baltmr 2 sue lightman 1 2 oren tomkinsnetzer 2 0 faculty of medicine, technion, israel institute of technology, haifa, israel 1 department of clinical ophthalmology, ucl institute of ophthalmology, london, uk 2 uveitis service, moorfields eye hospital, london, uk vogtkoyanagiharada syndrome is a cause of. Oct 26, 2017 treatment of vogt koyanagi harada vhk disease usually improves both the vision and hearing loss caused by the disease. Vogtkoyanagiharada vkh disease is one of the major visionthreatening diseases in certain populations, such as asians, native americans, hispanics and middle easterners. Revised diagnostic criteria for vogtkoyanagiharada disease fail. The most noticeable symptom is a rapid loss of vision. Vogt koyanagi harada vkh disease, also known as uveomeningitic syndrome, is a multisystem autoimmune disorder directed against any melanocytecontaining organ, including the eye, central. This study aims to make health professionals aware of the syndrome occurrences. Vogt koyanagi harada syndrome is a cause of noninfectious panuveitis, leading to significant vision loss in many patients.
However, it can present with neurological features before the ocular changes become apparent. Vogtkoyanagiharada disease vkhd is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear. Vogt koyanagi harada vkh disease is an autoimmune disorder characterized by bilateral, chronic, and diffuse granulomatous panuveitis. This article is from julyaugust 2005 and may contain outdated material. Vogtkoyanagiharada syndrome is an uncommon multisystem inflammatory disorder characterized by panuveitis with serous retinal detachments, and it is. Several authors, including the arabic doctor mohammadalghafiqi in the 12th century as well as jacobi, nettelship and tay in the 19th century, had described poliosis, neuralgias and hearing disorders. Pachymeningeal enhancement has been described previously. Vogtkoyanagiharada syndrome current perspectives opth. Vogtkoyanagiharada syndrome radiology reference article. Vogt koyanagi harada syndrome is an uncommon multisystem inflammatory disorder characterized by panuveitis with serous retinal detachments, and it is often associated with neurologic and cutaneous manifestations, including headache, hearing loss, vitiligo and poliosis.
It is therefore necessary for the rheumatologists to be familiar with its clinical aspects, pathogenesis and appropriate management. Vogtkoyanagiharada vkh disease is a bilateral, chronic granulomatous panuveitis associated with central nervous system, auditory, and. The patient was started on oral and topical steroids. Vogtkoyanagiharada vkh disease is a disorder affecting melanocytes of the skin, ocular, auditory and central nervous system. Vogt koyanagi syndrome an overview sciencedirect topics. Melanocytes are specialized cells that produce a pigment called melanin. Identify and treat vogtkoyanagiharada syndrome american. Vogtkoyanagiharada syndrome definition of vogtkoyanagi. Vogtkoyanagiharada syndrome nattaporn tesavibul, m. We studied 20 patients with vogt koyanagi harada syndrome. An update on inflammatory choroidal neovascularization.
It is an autoimmune disease occurring in genetically susceptible individuals and clinically presents as bilateral panuveitis with serous retinal detachments and hyperemic, swollen optic discs, which are associated with neurological and auditory manifestations. Dec 22, 2016 we report the case of a 35yearold female patient with bilateral and recurrent panuveitis. Vogt koyanagi harada vkh disease is a chronic, bilateral, granulomatous panuveitis, and exudative retinal detachment associated with poliosis. Diagnosed with vogtkoyanagiharada syndrome, the patient was treated with corticosteroids, achieving rapid improvement of functional and morphological findings. Focal neurologic signs such as cranial nerve palsies, hemiparesis, and optic neuritis are relatively uncommon. The pathogenesis is thought to be related to an aberrant t cellmediated immune response directed against selfantigens present in melanocytes. Vkh is a multisystem disorder and includes idiopathic uveitis, dysacousia. People who are treated early in the course of the disease tend to recover more fully than those who are treated later. Vogtkoyanagiharada disease vkhd, initially described as an uveomeningoencephalitic syndrome, is a systemic granulomatous autoimmune disease that targets melanocyterich tissues, such as the eye, inner ear, meninges, skin and hair. Vogtkoyanagiharada disease vkhd is a sightthreatening disease and a common cause of noninfectious panuveitis. The vogt koyanagi harada vkh syndrome is an uncommon disorder characterized by uveitis, neurological and cutaneous abnormalities tinnitus, vertigo, meningoencephalitis, vitiligo, alopecia, and poliosis.
Two cases of the vogt koyanagi harada syndrome occurring during pregnancy are reported for the first time. Despite the initial treatment, the patient progressed to total retinal detachments, and he was hospitalized for intravenous steroid treatment, which improved the condition dramatically. Vogtkoyanagiharada syndrome is an autoimmune disease of humans and dogs which consists of a uveitis of one or both eyes characterized by acute iridocyclitis, choroiditis, and retinal detachment. Vogtkoyanagiharada syndrome with more emphasis on the. The authors used a standard microlymphocyte cytotoxicity assay to type 9 patients with the vogt koyanagi harada syndrome for 25 hla a and b antigens, including bw22j. The ocular lesions may be accompanied by depigmentation of the iris and retina. Vogtkoyanagiharada disease nord national organization. Even when the symptoms of vkh disease get better with steroid therapy, many people need to slowly decrease the dose. Since then, the uveomeningoencephalitic syndrome has been known as vogt koyanagi harada syndrome. Vogtkoyanagiharada syndrome current perspectives pdf. Vogt koyanagi harada syndrome vkhs is a multisystem disorder that typically presents with bilateral uveitis, often associated with neurological andor audiological features. Inflammatory cnv icnv can occur either directly from an angiogenic stimulus mediated by local inflammation, result from a degenerative disruption in the bruchs membranerpe complex, or both 2, 6.
Vogtkoyanagiharada disease is a bilateral, chronic, diffuse granulomatous panuveitis typically characterized by. Visual outcomes were good, with final visual acuity of better than 2030 in 29 66% of 44 eyes and of worse than 20400 in only three 7% of. Management of vogt koyanagi harada vkh disease may involve various specialists including dermatologists, ophthalmologists, and neurologists. In both, the disease appeared during the second half of pregnancy with complete remission. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for vogtkoyanagiharada. We describe a 28yearold man with presumed vkh syndrome, whose presenting symptoms were bilateral impaired vision and headaches. 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 vkh disease is a multisystem autoimmune inflammatory disorder with ocular, auditory, skin and neurologic involvement. Vogtkoyanagiharada syndrome current perspectives abeir baltmr,1 sue lightman,1,2 oren tomkinsnetzer 1uveitis service, moorfields eye hospital, london, uk. Later similar cases were reported by koyanagi in 1929,3 after which the entity was identified as vogt koyanagi syndrome. Vogtkoyanagiharada syndrome current perspectives ncbi nih.
Although rare its incorrect diagnosis and therapy can lead to severe complications. Research suggests it may be due to an autoimmune reaction in which the body attacks its own healthy cells that contain the pigment melanin. The main defining clinical finding of vkh is severe bilateral granulomatous panuveitis inflammation throughout the uveal tract in the. Vogtkoyanagi syndrome definition of vogtkoyanagi syndrome. 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. Postgraduate medicaljournal1989 65, 587588thevogt koyanagi haradasyndrome. Neurologic manifestations of vkh typically include aseptic meningitis and headache. Babel in 1932 combined the disorders described by vogt, koyanagi and harada and suggested that these symptoms were manifestations of the same disease process. Vogtkoyanagiharada disease is a rare, multisystem, autoimmune disorder with numerous clinical manifestations, mediated through a thelper 1 response against melanocytes in the eye, inner ear, central nervous system, hair and skin. 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 vogt koyanagiharada vkh syndrome is a multisystemic immune disorder. Vogtkoyanagiharada vkh disease is defined as a bilateral granulomatous panuveitis with or without extraocular manifestations affecting.
Vogt koyanagi harada syndrome vkh syndrome has neurological, eye, ear, and skin symptoms. Vogt koyanagi harada syndrome vkh is a disease distinguished by a triad of uveitis, dermatologic findings, and cns involvement. Vogtkoyanagiharada syndrome is a cause of noninfectious panuveitis, leading to significant vision loss in many patients. Vogtkoyanagiharada syndrome an overview sciencedirect.
Vogtkoyanagiharada disease genetic and rare diseases nih. Melanin is the substance that gives skin, hair, and eyes their color. 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. Vogt koyagani harada syndrome a condition affecting humans and dogs, attributed to a tcell autoimmune reaction to melanocytes and characterised by uveitis, meningoencephalitis, choroiditis, retinal detachment, loss of hearing. Full text vogtkoyanagiharada syndrome current perspectives. Experts agree that successful therapy for vkh disease involves early and aggressive treatment with systemic corticosteroids steroids. Vogt koyanagi harada disease vkhd, initially described as an uveomeningoencephalitic syndrome, is a systemic granulomatous autoimmune disease that targets melanocyterich tissues, such as the eye, inner ear, meninges, skin and hair. Vkh syndrome is named for ophthalmologists alfred vogt from switzerland and yoshizo koyanagi and einosuke harada from japan. 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. It is characterized by diffuse granulomatous inflammation involving various organs including eye.
Vogtkoyanagiharada disease genetic and rare diseases. The signs and symptoms of vkh disease are caused by chronic inflammation of melanocytes. It is an autoimmune disease occurring in genetically susceptible individuals and clinically presents as bilateral. American indian ancestry appears to be the link to oriental racial type. Vogt koyanagi harada syndrome vkh is a multisystemic granulomatous autoimmune disease affecting organs with high melanocyte concentrations including the eye, cns, inner ear, and skin. We describe a 20yearold britishhonduran man with recent worsening headache and photophobia, vomiting and visual blurring. Treatment of vogtkoyanagiharada syndrome with intravenous.