Clinical spectrum and management options in vogtkoyanagiharada disease sikander ak lodhi, jm lokabhi reddy, venkataratnam perum department of ophthalmology, osmania medical collegesarojini devi eye hospital, hyderabad, telangana, india purpose. Vogtkoyanagiharada vkh syndrome is one such disease that requires imt for its optimal management, and failure to do so portends a poor prognosis 4,5. The patient was started on oral and topical steroids. Vogt koyanagi harada syndrome vkhs is a rare autoimmune multisystem syndrome. The absence of ocular trauma or previous intraocular surgery sets vkhd appart from. The relation between vk syndrome and sympathetic ophthalmia. A similar disorder with uveitis and depigmentation of the nose, lips, eyelids, footpads and anus occurs in dogs. Vogtkoyanagiharada vkh disease is a bilateral granulomatous uveitis often associated with exudative retinal detachment and with extraocular manifestations, such as pleocytosis in the cerebrospinal fluid and, in some cases, vitiligo, poliosis, alopecia, and dysacusis.
Cases have most often been reported from japan, north and south america and europe with a seasonal peak during spring and autumn. Vkh disease occurs more commonly in patients with a genetic predisposition to the disease, including those from asian, middle eastern. Although frequently unrecognised,vkh may affect children. Increased peripheral blood cd4plus cd8plus tcell ratios have been seen in vkh, suggesting an immunemediated disease. Vogtkoyanagiharada disease genetic and rare diseases. Vogtkoyanagiharada syndrome current perspectives abeir baltmr,1 sue lightman,1,2 oren tomkinsnetzer 1uveitis service, moorfields eye hospital, london, uk.
After cowpers work in 1951 vogtkoyanagi and haradas sequenz is considered the same entity. The signs and symptoms of vkh disease are caused by chronic inflammation of melanocytes. Identify and treat vogtkoyanagiharada syndrome american. A group of symptoms that collectively indicate or characterize a disease, disorder, or other condition considered abnormal. Association between vogtkoyanagiharada syndrome and hladr1 and dr4 in hispanic patients living in southern california. Vogtkoyanagiharada syndrome description, causes and risk factors. Vogtkoyanagiharada syndrome in dogs symptoms, causes. Vogtkoyanagiharada syndrome article about vogtkoyanagi. The vogtkoyanagiharada syndrome vkh is characterized by bilateral uveitis with serous detachments and signs of systemic disease, including skin disorders alopecia, poliosis, hearing problems loss of hearing, dizziness and signs of meningeal irritation 1,2. Complicated cataract, glaucoma, panuveitis, vogtkoyanagiharada disease.
Vogt koyanagi harada disease is a rare disorder with a multiorgan clinical course, characterized by the presence of ophthalmological, auditory, cutaneous and neurological disorders national organization for rare disorders, 2016. Once confirmed, although there is no known cure for vogtkoyanagiharada syndrome in dogs, the disease can be kept under control with longterm immunosuppressive medication. Sensorineural hearing loss in vogtkoyanagiharada syndrome. Vkh syndrome is an uncommon multisystem disease of presumed autoimmune etiology that is characterized by chronic, bilateral, diffuse, granulomatous panuveitis with accompanying integumentary, neurologic, and auditory involvement. Despite the initial treatment, the patient progressed to. Vogtkoyanagiharada syndrome current perspectives ncbi nih. Incredibles wannabe sidekick turned bad guy, from the incredibles 2004.
The vogtkoyanagiharada syndrome vkh is a bilateral, diffuse granulomatous uveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. Vogtkoyanagiharada vkh syndrome is a systemic condition characterized by ocular inflammatory disease as well as skin, ear, and meningeal manifestations. Vogtkoyanagiharada vkh disease is defined as a bilateral granulomatous panuveitis with or without extraocular manifestations affecting. Background vogt koyanagi harada syndrome vkh is a severe, bilateral panuveitis associated with serous retinal detachments and signs of meningeal irritation, with or without auditory disturbances and integumentary changes. Vogt koyanagi harada disease vkh disease is a disease that affects several parts of the body, including the eyes, ears, nervous system, and skin. Before vogtkoyanagiharada syndrome begins, individuals usually experience symptoms such as headache, vertigo, nausea, stiff neck, vomiting, and lowgrade fever for several days. Vogt koyanagi harada syndrome is a rare multisystemic disease of presumed autoimmune etiology, affecting various melanocytecontaining organs. The etiology is unknown, but it may be an inflammatory autoimmune condition. The classic course of vkh syndrome has three phases. The vogtkoyanagiharada vkh syndrome is a rare systemic disorder of uveitis, dysacousia, vitiligo, premature graying of the hair, eyebrows and eyelashes, and meningoencephalitis. San francisco, california t h e vogtkoyanagiharada vkh syndrome represents a spectrum o f inflammatory disease that affects the uvea, retina, m e n i n g e s, and skin. The condition has several extraocular manifestations, and is a common uveitic entity in japanese, relatively less com. 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. The first phase is the meningoencephalitis phase, with various neurological symptoms.
The vogtkoyanagiharada syndrome vkhs is rare in the netherlands. 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. Vogtkoyanagiharada vkh syndrome is a rare multisystem disease of melanocyte containing organs. 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 characterized by. The ocular lesions may be accompanied by depigmentation of the iris and retina. Vogtkoyanagiharada disease vkh and birdshot retino choroiditis brc.
Aug 17, 2018 the key to successful therapy for vogtkoyanagiharada vkh disease is early and aggressive treatment with systemic corticosteroids. The most noticeable symptom is a rapid loss of vision. Vogtkoyanagiharada syndrome is a rare multisystemic disease of presumed autoimmune etiology, affecting various melanocytecontaining organs. Vogt koyanagi harada syndrome vkh is a disease distinguished by a triad of uveitis, dermatologic findings, and cns involvement. Vogtkoyanagiharada disease vkh is a systematic autoimmune disorder that affects tissues. Vogtkoyanagiharada syndrome definition of vogtkoyanagi. 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. American journal of ophthalmology, chicago, 1957, 43.
The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes. Disease definition vogtkoyanagiharada disease is a bilateral, chronic, diffuse granulomatous panuveitis typically characterized by serous retinal detachment and frequently associated with neurological meningitis, auditory, and dermatological alterations. Vogtkoyanagiharada syndrome vkh american journal of. 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, initially described as an uveomeningoencephalitic syndrome, is a systemic granulomatous autoimmune. Vogtkoyanagiharada disease vkhd is a sightthreatening disease and a common cause of noninfectious panuveitis.
This condition is common in the third and fourth decade, rarely. This disease is mainly a th1 lymphocyte mediated aggression to melanocytes after a viral trigger in the presence of hladrb10405 allele. It is characterized by diffuse granulomatous inflammation involving various organs including eye. Vogtkoyanagiharada syndrome presenting with encephalopathy. Syndrome pop culture when innocent heroworship goes unrequited, the consequences can be dire for both the admirer and the admired. Vogtkoyanagiharada vkh disease clinical presentation.
Vogt koyanagi harada syndrome go to academy store learn more and purchase. Vogt koyanagi harada disease is a chronic,bilateral, granulomatous uveitis with extraocular manifestations in the central nervous system such as cerebrospinal fluid pleocytosiscsf, dysacousia, tinnitus, vertigo, and in some cases, integumentary system vitiligo,poliosis,and. However, in asia it is one of the most common causes of panuveitis. The syndrome is characterised by a combination of panuveitis, pleocytosis in cerebral spinal fluid with or without neurological signs. Vogtkoyanagiharada disease nord national organization. S i n c e the initial description b y v o g t in 1 9 0 6, and the amplification o f this description by koya n a g i in 1 9 2 9, a n u m b e r o f patients have had. Jun 10, 2015 symptoms, risk factors and treatments of vogtkoyanagiharada syndrome medical condition vogtakoyanagiaharada syndrome is an uncommon multisystem disease of presumed autoimmune. The second phase is the ophthalmic auditory phase, presenting. Vogt koyanagi harada disease vkhd is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin and hair. Introduction vogtkoyanagiharada vkh disease is a panuveitis of autoimmune nature where the melanocytes act both as an inducer and as a target. Case presentation a 20 year old asian male was referred with the complaint of decreased vision in both eyes for a year. 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. These symptoms are not specific to vkh syndrome and may be diagnosed as a viral infection or influenza. Vogtkoyanagiharada syndrome is a bilateral, chronic, diffuse granulomatous panuveitis frequently associated with neurological, auditory, and.
Vogtkoyanagiharada disease vkh is a wellestablished multiorgan disorder that affects pigmented structures, such as the eye, inner ear, meninges, and skin 1. Vkh syndrome is named for ophthalmologists alfred vogt from switzerland and yoshizo koyanagi and einosuke harada from japan. The aetiology of the disease is still not known although recent findings point to an autoimmune reaction against. Vogtkoyanagiharada syndrome nattaporn tesavibul, m. 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. Vogtkoyanagiharada syndrome synonyms, vogtkoyanagiharada syndrome pronunciation, vogtkoyanagiharada syndrome translation, english dictionary definition of vogtkoyanagiharada syndrome.
Mortimer house, 37 41 mortimer street, london w1t 3jh, uk seminars in ophthalmology publication. This article is from julyaugust 2005 and may contain outdated material. Khopkar vogt koyanagi harada syndrome vkhs is an uncommon systemic syndrome characterized by intraocular inflammation with associated cutaneous, auditory and neurological abnormalitiesl4. Pdf editor,vogtkoyanagiharada vkh syndrome is a bilateral, diffuse, granulomatous uveitis associated with exudative retinal detachment and. The peak age of presentation is the third and fourth decade with equal sex incidence. Vogtkoyanagiharada syndrome current perspectives opth. Vogtkoyanagiharada disease johns hopkins university. Vogtkoyanagiharada syndrome nederlands tijdschrift. Vogt koyanagi harada syndrome vkhs is a rare multisystemic disease which probably has autoimmune etiology and which affects tissues that contain melanin.
Vogtkoyanagiharada syndrome medical condition youtube. There may also be neurological signs such as severe headache, vertigo, nausea, and drowsiness. 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. In this creative challenge, surrealism and onepoint perspective combine to. Melanin is the substance that gives skin, hair, and eyes their color. Vogtkoyanagiharadalike syndrome in dogs dog breed info. Vogt koyanagi harada syndrome vkh syndrome has neurological, eye, ear, and skin symptoms. Vogtkoyanagiharadalike syndrome definition of vogt. Vkh syndrome is an uncommon multisystem disease of presumed autoimmune etiology that is characterized by chronic, bilateral, diffuse, granulomatous panuveitis with accompanying. Those patients who are treated later in the course of the disorder have a more guarded prognosis for recovery of visual acuity and probably have a greater risk for chronic inflammation.
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. Vogt koyanagi harada vkh syndrome is a systemic condition characterized by ocular inflammatory disease as well as skin, ear, and meningeal manifestations. Vogtkoyanagiharada disease vkh is a multisystem disease of presumed autoimmune cause that affects pigmented tissues, which have melanin. 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. Regarding immunopathogenesis of the vogt koyanagi harada syndrome vkhs, peripheral blood lymphocytes of a female patient with this disease were tested for sensitization against several antigens such as myelinic basic protein bp and uveoretinal, brain and spleen tissue homogenates. 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 koyanagi harada vkh syndrome is a multisystemic immune disorder characterized by. 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 disease is a chronic,bilateral, granulomatous uveitis with extraocular manifestations in the central nervous system such as cerebrospinal fluid pleocytosiscsf, dysacousia, tinnitus, vertigo, and in some cases, integumentary system vitiligo,poliosis,and. Clinical spectrum and management options in vogtkoyanagi. Vogt koyanagi harada syndrome vkhs is an uncommon systemic syndrome characterized by intraocular inflammation with associated cutaneous, auditory and neurological abnormalitiesl4. Vkh may variably also involve the inner ear, with effects on hearing, the skin and the meninges of the central nervous system. Einosuke harada, harada einosuke, february 25, 1892 december 20, 1946 was a japanese ophthalmologist who reported a condition now known as vogtkoyanagiharada disease career. The aim of this study was to describe the clinical features, treatment options, and visual outcome of vogtkoyanagiharada vkh disease. Vogtkoyanagiharada disease definition of vogtkoyanagi.
Vogtkoyanagiharada syndrome vkhs is a rare multisystemic disease which probably has autoimmune etiology and which affects tissues that contain melanin. 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. He was born in goryo, amakusa district, kumamoto prefecture in 1892 and grew up in taragi, kuma district, and kumamoto, kumamoto prefecture. Vogtkoyanagiharada syndrome go to academy store learn more and purchase. Vogtkoyanagiharada syndrome medigoo health medical. His past medical and surgical histories were unremarkable. It is important to follow a veterinarians treatment plan.
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. Research suggests it may be due to an autoimmune reaction in which the body attacks its own healthy cells that contain the pigment melanin. We report a case of a 10 year old girl who presented with. Vogtkoyanagiharada syndrome is an uncommon multisystem inflammatory disorder characterized by panuveitis with serous retinal detachments, and it is. Melanin is what gives color, or pigment, to our hair, skin and parts of our eyes. It is therefore necessary for the rheumatologists to be familiar with its clinical aspects, pathogenesis and appropriate management. Vogtkoyanagiharada syndrome an overview sciencedirect. Also known as uveomeningoencephalitic syndrome, the disease is characterized by chronic bilateral panuveitis associated with a varying constellation of neurological, auditory and cuta.
Vogt koyanagi harada syndrome synonyms, vogt koyanagi harada syndrome pronunciation, vogt koyanagi harada syndrome translation, english dictionary definition of vogt koyanagi harada syndrome. Vogt koyanagi harada syndrome aalia r sufi, sumera m zargar, tejit singh introduction vogt koyanagi harada syndrome vkh is a bilateral granulomatous panuveitis associated with cutaneous, neurological and auditory manifestations 1. His family history revealed thyroid disease in his mother. What distinguishes vkh syndrome from the flu is the start of eye symptoms such as sudden blurry. Vogtkoyanagiharada syndrome radiology reference article. Melanocytes are specialized cells that produce a pigment called melanin. 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. Vogt koyanagi harada syndrome nattaporn tesavibul, m. Aug 17, 2018 vogtkoyanagiharada vkh disease is a multisystemic disorder characterized by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations. Analysis of 87 cases with vogtkoyanagiharada disease. It is considered to be a granulomatous inflammatory syndrome, probably as a result of an autoimmune mechanism, influenced by genetic factors, and appears to be directed.
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