Vogt–Koyanagi–Harada (VKH) syndrome: A new perspective for healthcare professionals

Patil, Yojana; Garg, Ruchira; Rajguru, Jagadish Prasad; Sirsalmath, Manjunath; Bevinakatti, Varsha A; Kumar, Manish; Sharma, Sonika

doi:10.4103/jfmpc.jfmpc_787_19

Cited by 23 publications

(11 citation statements)

References 19 publications

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“…As patients may have persisting inflammation for several months to years and given the risks of long-term steroid use, early use of immunomodulatory therapy should be strongly considered in these patients. [ 1 2 ]…”

Section: Discussionmentioning

confidence: 99%

“…Vogt-Koyanagi-Harada (VKH) syndrome is a granulomatous disorder which affects melanocyte-rich tissues like eye, inner ear, skin, and the meninges. [ 1 2 ] This syndrome has been recognized for more than a hundred years but continues to pose a diagnostic challenge because of its rarity and fragmentary manifestations. The nervous system is commonly involved early in the disease course, mainly in the form of aseptic meningitis although other neurological manifestations, like cranial neuropathies,[ 3 ] encephalopathy,[ 4 ] and myelitis[ 5 ] can also occur.…”

Section: Introductionmentioning

confidence: 99%

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Vogt-koyanagi-harada syndrome - A neurologist's perspective

Shivaram

Nagappa

Seshagiri

et al. 2021

Ann Indian Acad Neurol

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Vogt-Koyanagi-Harada (VKH) syndrome is an immune-mediated granulomatous disease which affects melanin-rich organs like eyes, skin, nervous system, and ears. Neurological and auditory manifestations usually precede the involvement of other sites. Patients may manifest with “complete” or “incomplete” syndrome. We report two patients who presented with acute headache and impaired vision. Fundus examination revealed optic disc hyperemia and exudative retinal detachment which provided a clue for the diagnosis at the bedside. Fundus fluorescein angiogram (FFA) revealed abnormal dye leakage, whereas B scan showed choroid thickening. Cerebrospinal fluid (CSF) pleocytosis contrasted with unremarkable brain magnetic resonance imaging and lack of meningeal signs. Melanophagocytosis was evidenced by melanin-laden macrophages in CSF and skin biopsy. This finding is specific for VKH syndrome and helps to clinch the diagnosis even when the complete syndrome is not present cross-sectionally. VKH syndrome should be suspected in patients with aseptic meningitis if tests for common infectious and immune-mediated diseases are negative.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Vogt-koyanagi-harada syndrome - A neurologist's perspective

Shivaram

Nagappa

Seshagiri

et al. 2021

Ann Indian Acad Neurol

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“…Vitiligo is characterized by loss of melanocytes in the skin and mucosa (1). VKH is more extensive and includes the skin, mucosa, eyes, ears, and meninges, resulting in chronic uveitis, alopecia, vitiligo, poliosis, and irritation of the meninges (10). The current hypothesis in the field is that VKH represents an exacerbated reaction by melanocytes and their precursors as compared to vitiligo.…”

Section: Introductionmentioning

confidence: 99%

“…3. Probable VKH-these patients have similar ocular symptoms as patients with VKH, and they tend to have neurologic and auditory manifestations or dermatologic signs, but not both (10).…”

Section: Introductionmentioning

confidence: 99%

“…Several cytokines and chemokines contribute to the development of vitiligo and VKH, including type 1 responses (IFNg, CXCL9/10/11, IL-12, TNF) type 17 responses (IL-17, CCL20, IL-23) and IL-2, which supports T cell growth and survival (13)(14)(15)(16)(17)(18)(19). Some studies have demonstrated that patients with VKH that have vitiligo have a predominance of CD4+ T-cell lymphocytes and an imbalanced ratio of CD4+/CD8+ T-cells (10), though others have demonstrated a CD8+ cytotoxic T cell preponderance (15). These CD8+ T cells react to melanocyte antigens and exhibit markers of skin resident memory T cells (20,21).…”

Section: Introductionmentioning

confidence: 99%

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Case Series: Gene Expression Analysis in Canine Vogt-Koyanagi-Harada/Uveodermatologic Syndrome and Vitiligo Reveals Conserved Immunopathogenesis Pathways Between Dog and Human Autoimmune Pigmentary Disorders

et al. 2020

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Vogt-Koyanagi-Harada syndrome (VKH) and vitiligo are autoimmune diseases that target melanocytes. VKH affects several organs such as the skin, hair follicle, eyes, ears, and meninges, whereas vitiligo is often limited to the skin and mucosa. Many studies have identified immune genes, pathways and cells that drive the pathogeneses of VKH and vitiligo, including interleukins, chemokines, cytotoxic T-cells, and other leukocytes. Here, we present case studies of 2 canines with VKH and 1 with vitiligo, which occurred spontaneously in client-owned companion dogs. We performed comparative transcriptomics and immunohistochemistry studies on lesional skin biopsies from these cases in order to determine if the immunopathogenesis of autoimmune responses against melanocytes are conserved. In dogs, we found enrichment of T cell gene signatures, with upregulation of IFNG, TNF, PRF1, IL15, CTSW, CXCL10, and CCL5 in both VKH and vitiligo in dogs compared to healthy controls. Similar findings were reported in humans, suggesting that these genes play a role in the pathogenesis of spontaneous VKH and vitiligo. T cell-associated genes, including FOXP3 and TBX21, were enriched, while IGFBP5, FOXO1, and PECAM1 were decreased compared to healthy controls. Further, we identified TGFB3, SFRP2, and CXCL7 as additional potential drivers of autoimmune pigmentary disorders. Future studies exploring the immunopathogenesis of spontaneous autoimmunity will expand our understanding of these disorders, and will be useful in developing targeted therapies, repurposing drugs for veterinary and human medicine, and predicting disease prognosis and treatment response.

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Vogt–Koyanagi–Harada syndrome in the setting of COVID‐19 infection

Eatz

2023

Clinical Case Reports

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Vogt-Koyanagi-Harada (VKH) disease is a T-cellmediated multisystemic autoimmune inflammatory disorder characterized by skin, ocular, auditory, and neurologic involvement. The T-cell-mediated granulomatous intraocular inflammation is responsible for vitritis, disk edema, serous retinal detachments, and eventual sunset glow fundus. 1 T cells target melanocytes, with an ensuing cascade leading to four distinct disease phases as follows: prodromal, uveitis, convalescent, and recurrent. 2 VKH symptoms include headache, meningismus, hearing loss, poliosis, alopecia, and vitiligo. 3 It is most prevalent in Asians, Native Americans, Hispanics, and Middle Easterners. If promptly and adequately treated, patients can experience good outcomes and avoid complications including sunset glow fundus, cataracts, glaucoma, subretinal fibrosis, and choroidal neovascularization. Some studies have reported the link between some viral infections such as cytomegalovirus (CMV) and Epstein-Barr virus (EBV) and the development of VKH. It has been postulated that similarity between peptides on melanocytes and some exogenous viral peptides lead to adverse T cells attacks on melanocytes-containing tissues and lead to the symptoms seen in VKH. 4 We report a case of a VKH syndrome in a patient diagnosed with COVID-19. | CASE REPORTA 27-year-old male patient with no significant past medical history presented with a two-week history of intermittent unilateral headache, bilateral eye pain with photophobia, and phonophobia, followed by tinnitus and blurry vision, which ultimately progressed to total bilateral vision loss. His initial physical examination was unremarkable including vitals, except for total bilateral visual loss with preserved light perception. An ophthalmologic examination revealed normal intraocular pressure and panuveitis with serous retinal detachments bilaterally.The patient was admitted for further evaluation. Infectious work-up revealed positive COVID-19 PCR. Other infectious and metabolic panels were unremarkable. Cerebrospinal fluid studies (CSF), venereal disease research laboratory test (VDRL), fluorescent treponemal antibody absorption test (FTA), QuantiFERON gold, flow cytometry, cytology, and cultures were all negative, except for moderate pleocytosis with lymphocytic predominance

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Vogt–Koyanagi–Harada (VKH) syndrome: A new perspective for healthcare professionals

Cited by 23 publications

References 19 publications

Vogt-koyanagi-harada syndrome - A neurologist's perspective

Vogt-koyanagi-harada syndrome - A neurologist's perspective

Case Series: Gene Expression Analysis in Canine Vogt-Koyanagi-Harada/Uveodermatologic Syndrome and Vitiligo Reveals Conserved Immunopathogenesis Pathways Between Dog and Human Autoimmune Pigmentary Disorders

Vogt–Koyanagi–Harada syndrome in the setting of COVID‐19 infection

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