Get a printable copy pdf file of the complete article 765k, or click on a page image below to browse page by page. Immunopathologic study of vogtkoyanagiharada syndrome. The diagnosis of vogtkoyanagiharada syndrome was considered, and a lumbar puncture was performed with normal results. Clinical records of 34 eyes of 17 vkh patients with sgf in whom laser flare photometry lfp, enhanced depth imaging optical coherence tomography edioct. Vogtkoyanagiharada with multiple peds retina image bank. Macular spectral domain optical coherence tomography sdoct images of 22 eyes of consecutive patients with vkh at the convalescent stage were compared to 17 eyes of 9 agesexrefractionmatched normal. Vogtkoyanagiharada syndrome radiology reference article. Patients with incomplete clinical files or a deficient ffa were excluded.
Abstract introduction vogtkoyanagiharada disease vkh is a wellestablished multiorgan disorder that affects pigmented structures, such as the eye, inner ear. This may be an immune response to changes brought on by ultraviolet radiation. Vogtkoyanagiharadas syndrome and its multisystem involvement. Department of dermatology, ehime university graduate school of medicine, toon, ehime, japan. People who are treated early in the course of the disease tend to recover more fully than those who are treated later. In this retrospective comparative study, the sdoct. Vogtkoyanagiharada disease vkh is a multisystem disease of presumed autoimmune cause that affects pigmented tissues, which have melanin. The first phase is the meningoencephalitis phase, with various neurological symptoms. Vogtkoyanagiharada disease genetic and rare diseases. This article is from journal of ophthalmic inflammation and infection, volume 4. Vogtkoyanagiharada 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. Some ethnic groups have a higher probability of developing the disease, including asians, indians and latin americans and females are affected more often. Both shared many ocular and systemic manifestations, including integumentary findings. Intraocular inflammation continued and vision remained poor over the next few weeks, prompting initiation of intravenous pulses and then highdose oral prednisone 2 mgkg of body mass day.
Influence of molecular genetics in vogtkoyanagiharada. We report three patients who presented with initial findings suggestive of bilateral optic neuritis requiring csf analysis and brain images. Vogtkoyanagiharada disease vkhd is a defined as a bilateral granulomatous panuveitis that affects pigmented struc tures, such as the. 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. Vogtkoyanagiharada syndrome autoimmune multiorgan disease involving eyes, ears, skin, and nervous system three phases first phase. The classic course of vkh syndrome has three phases. Vogtkoyanagiharada syndrome vkh is a severe bilateral. It is most common in german shepherds, greyhounds and siberian huskies. Mar 22, 2014 the aim of this study was to determine the clinical significance of posterior choroidal thickness and vascular changes in the convalescent stage of vogt koyanagi harada disease vkh. Jan 26, 2007 purpose to report the fluorescein fundus angiographic ffa findings in the different clinical stages of vogtkoyanagiharada vkh patients. 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. Vogtkoyanagiharada vkh disease usually presents as bilateral panuveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs.
To delineate the historical steps associated with the genesis of the name and the definition of vogtkoyanagiharada vkh disease. Vogtkoyanagiharada vkh disease is a granulomatous multisystem inflammatory disorder that classically affects the uvea, inner ear, meninges, and skin. Vogt koyanagi harada vkh disease and sympathetic ophthalmia so are both autoimmune disorders targeting melaninbearing cells, even though their etiologies are different. Vogt koyanai harada disease, difluprednate, topical corticosteroid treatment for harada disease, uveitis. Vogtkoyanagiharadas syndrome is a rare disease that affects tissues containing melanocytes, such as the eyes, central nervous system, inner ear and skin. Revised diagnostic criteria for vogtkoyanagiharada disease. Macular spectral domain optical coherence tomography sdoct images of 22 eyes of consecutive patients with vkh at the convalescent stage were compared to 17 eyes of 9 agesexrefraction. Jun 28, 2014 acute vogt koyanagi harada vkh disease and acute central serous chorioretinopathy cscr are two common disorders with serous retinal detachment caused by dysfunction of choroid. Vogtkoyanagiharada vkh disease is a tcell altered immune process that is directed at the melanocytes. Vogtkoyanagiharada vkh disease is an autoimmune disease directed against one or more antigens found on or associated with uveal melanocytes, with subsequent chorioretinal atrophy giving rise to the characteristic sunset glow fundus appearance yamaki et al. Reversible alopecia in vogtkoyanagiharada disease and.
Vogt koyanagi harada disease vkh is a multisystem autoimmune disorder principally affecting pigmented tissues in the ocular, auditory, integumentary and central nervous systems. Comparison of the optical coherence tomographic characters. Vogtkoyanaiharada disease, difluprednate, topical corticosteroid treatment for harada disease, uveitis. Vogtkoyanagiharada syndrome vkh is a bilateral, diffuse granulomatous uveitis associated with neurological, audiovestibular, and dermatological systems. Make a photocopy of the completed answer form for your own files and mail the. Vogtkoyanagiharada syndrome uveomeningoencephalitic. The clinical presentation and diagnosis of vogtkoyanagi. Vogtkoyanagiharada vkh disease and sympathetic ophthalmia so are both autoimmune disorders targeting melaninbearing cells, even though their etiologies are different. Prognostic factors for clinical outcomes in patients with.
Recurrent unilateral vogtkoyanagiharada disease with. Purpose to report the fluorescein fundus angiographic ffa findings in the different clinical stages of vogtkoyanagiharada vkh patients. Oct 26, 2017 treatment of vogt koyanagi harada vhk disease usually improves both the vision and hearing loss caused by the disease. Vogtkoyanagiharada disease genetic and rare diseases nih. Vogtkoyanagiharada disease nord national organization.
Pdf vogtkoyanagiharada vkh disease is usually defined as a bilateral chronic granulomatous panuveitis that may be associated with cns, auditory. Melanocytes are specialized cells that produce a pigment called melanin. Development and evaluation of diagnostic criteria for vogt. Vogtkoyanagiharada syndrome vkh is a rare inflammatory disorder diagnosed clinically that presents as panuveitis with serous retinal detachments among other systemic symptoms. In this retrospective comparative study, the sdoct images of 65. Vogtkoyanagiharada disease vkh disease is a disease that affects several parts of the body, including the eyes, ears, nervous system, and skin. Pdf on aug 1, 2014, emmett t cunningham and others published vogtkoyanagiharada disease find, read and cite all the research you need on researchgate.
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 koyanagi harada vkh syndrome is a multisystemic immune disorder. Vogtkoyanagiharada vkh disease is an autoimmune disease involving pigmented tissue in eyes, auditory system, skin, and central nervous system. A case report on vogtkoyanagiharada disease seen at a. Vogtkoyanagiharada syndrome current perspectives abeir baltmr,1 sue lightman,1,2 oren tomkinsnetzer 1uveitis service, moorfields eye hospital, london, uk. Bhupinder johala, herman johala, andrew lukaris full text pdf, 182kb abstract. The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes. 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. The purpose of this study is to compare the morphological changes of these two diseases with spectral domain optical coherence tomography sdoct. The study aimed to describe the effectiveness of switching the antitnf. Phacoemulsification surgery in patients with vogt koyanagi. Resolution of subretinal fluid with systemic corticosteroid treatment in acute vogt koyanagi harada disease. Vogtkoyanagiharada syndrome vkhs is a rare multisystemic disease which probably has autoimmune etiology. All patients underwent ffa at least in one occasion.
Phacoemulsification surgery in patients with vogtkoyanagiharada syndrome 17 the timing and selection of the treatment for these complicated cataracts have been controversial10. Gross, md on oct 10, 2012 rating 2 ratings appears in vogt koyanagi harada conditionkeywords pigment epithelial detachment ped, fa mid phase. Files are available under licenses specified on their description page. Pdf to text batch convert multiple files software please purchase personal license. 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. Most of the literature focused on the ocular manifestations and related treatment. Aug, 2012 vogt koyanagi harada vkh disease is a tcell altered immune process that is directed at the melanocytes.
Indocyanine green angiography findings in patients with long. Vogtkoyanagiharada disease is a bilateral, chronic, diffuse granulomatous panuveitis typically characterized by. Some ethnic groups have a higher probability of developing the disease, including asians, indians and latin. The term vogtkoyanagiharada disease was coined by bruno and mcpherson 8 to. Vogtkoyanagiharada disease vkh is a multisystem autoimmune disorder principally affecting pigmented tissues in the ocular, auditory, integumentary and central nervous systems. Uveitis, vogtkoyanagiharada disease, antitnf abstract. Vogtkoyanagiharada vkh disease is an immunemediated disorder affecting both eyes. The pathogenesis is a result of t cell lymphocyte reaction against melanocyte component, tyrosinase and tyrosinaserelated protein. Immunomodulatory therapy, microperimetry, outcome, retinal sensitivity, vogtkoyanagiharada disease vogtkoyanagiharada vkh disease is an autoimmune disease directed against one or more antigens found on or associated with uveal melanocytes. The signs and symptoms of vkh disease are caused by chronic inflammation of melanocytes. Read rw, holland gn, rao na, tabbara kf, ohno s, arellanesgarcia l, et al.
The diagnosis of vogt koyanagi harada syndrome was considered, and a lumbar puncture was performed with normal results. Pdf vogtkoyanagiharada syndrome is a bilateral, chronic, diffuse granulomatous panuveitis frequently associated with neurological. Article information, pdf download for vogtkoyanagiharada syndrome. There may also be neurological signs such as severe headache, vertigo, nausea, and drowsiness. Optical intensities of different compartments of subretinal. Gross, md on oct 10, 2012 rating 2 ratings appears in vogt koyanagi harada conditionkeywords. Fluorescein fundus angiographic findings in vogtkoyanagi. Vogt koyanagi harada disease vkh disease is a disease that affects several parts of the body, including the eyes, ears, nervous system, and skin. Abstract introduction vogt koyanagi harada disease vkh is a wellestablished multiorgan disorder that affects pigmented structures, such as the eye, inner ear. It is a granulomatous inflammatory disorder that affects the eyes, auditory system, meninges, skin and often presents. The most noticeable symptom is a rapid loss of vision. Here, we describe a unilateral case of vkh disease. Equally, the intraocular lens iol implantation and the choice of the iol are topics for debate6,911.
Apr 17, 2007 vogtkoyanagiharada vkh disease is a granulomatous multisystem inflammatory disorder that classically affects the uvea, inner ear, meninges, and skin. The diagnosis is essentially based on the observation of bilateral diffuse choroiditis of acute onset following flulike prodromic symptoms, and prompt treatment with systemic corticosteroid andor immunosuppressant has been recommended. Treatment of vogtkoyanagiharada vhk disease usually improves both the vision and hearing loss caused by the disease. 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. The primary pathogenesis is tcellmediated autoimmune response directed towards melanocyte or melanocyteassociated antigens causing inflammation of the choroidal layer. Diagnostic criteria for vogt koyanagi harada disease. Pdf on aug 1, 2014, emmett t cunningham and others published vogtkoyanagiharada disease find, read and cite all the research you. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for vogtkoyanagiharada. February optical intensities of different compartments of subretinal fluid in acute vogtkoyanagiharada disease dusheng lin 0 1 xiaohong luo 0 1 lingying meng 0 1 guihua zhang 0 1 weiqi chen 0 1 haoyu chen 0 1 0 1 joint shantou international eye center, shantou university and the chinese university of hong kong, shantou, guangdong, china, 2 shantou center hospital, shantou, guangdong. The clinical presentation and diagnosis of vogtkoyanagiharada syndrome cases and elective reports v ogt koyanagiharada syndrome vkh is a rare systemic disease 1.
Revised diagnostic criteria for vogt koyanagi harada disease. Acute vogtkoyanagiharada vkh disease and acute central serous chorioretinopathy cscr are two common disorders with serous retinal detachment caused by dysfunction of choroid. Melanin is the substance that gives skin, hair, and eyes their color. Vogt koyanagiharada syndrome vkh is a severe bilateral. The aim of this study was to determine the clinical significance of posterior choroidal thickness and vascular changes in the convalescent stage of vogtkoyanagiharada disease vkh. Resolution of subretinal fluid with systemic corticosteroid treatment in acute vogtkoyanagiharada disease. The cause of this condition is not known, at present. Vogt koyanagi harada syndrome is a rare multisystemic disease of presumed autoimmune etiology, affecting various melanocytecontaining organs.
Identification of underlying inflammation in vogtkoyanagi. Phacoemulsification surgery in patients with vogt koyanagi harada syndrome 17 the timing and selection of the treatment for these complicated cataracts have been controversial10. Abstractvogtkoyanagiharada vkh disease is a systemic autoimmune disorder. We divided the patients in four groups, depending on their. Vogtkoyanagiharada disease presenting as optic neuritis. To evaluate underlying subclinical ocular inflammation in vogtkoyanagiharada vkh disease with sunset glow fundus sgf by multiple analyses. To delineate the historical steps associated with the genesis of the name and the definition of vogt koyanagi harada vkh disease. Vogtkoyanagiharada disease in an 8yearold boy budi setiabudiawan, 1 feti karfiati, 2 reni ghrahani, 1 gartika sapartini, 1 and indra sahril 1 1 department of child health, medical school, padjadjaran university, dr. Vogtkoyanagiharada syndrome presenting with encephalopathy. Vogtkoyanagiharada disease during pregnancy article pdf available in british journal of ophthalmology 791. Ciks chronic immune mediated keratoconjunctivitis sicca ciks is the newer name for pannus. Methods retrospective, transversal and descriptive study. Indocyanine green angiography in vogtkoyanagiharada disease. Vogt koyanagi harada vkh disease is an autoimmune disease involving pigmented tissue in eyes, auditory system, skin, and central nervous system.
Immunopathologic study of vogtkoyanagiharada syndrome chichao chan, m. Vogtkoyanagiharada syndrome is a rare multisystemic disease of presumed autoimmune etiology, affecting various melanocytecontaining organs. Wed like to understand how you use our websites in order to improve them. Khopkar vogt koyanagi harada syndrome vkhs is an uncommon systemic syndrome characterized by intraocular inflammation with associated cutaneous, auditory and neurological abnormalitiesl4. Patients are typically 20 to 50 years old and have no history of either surgical or accidental ocular trauma. Aug 17, 2018 vogt koyanagi harada syndrome in children.
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