Background: Vogt-Koyanagi-Harada (VKH) disease is a rare, multisystem autoimmune disorder characterized by bilateral granulomatous panuveitis, with or without extraocular manifestations. Although its exact etiology an...Background: Vogt-Koyanagi-Harada (VKH) disease is a rare, multisystem autoimmune disorder characterized by bilateral granulomatous panuveitis, with or without extraocular manifestations. Although its exact etiology and pathogenesis remain unclear, it is hypothesized to involve T-cell dysregulation targeting melanocyte-containing tissues, including the CNS, eye, ear, and skin. VKH predominantly affects pigmented groups, such as Asians, Hispanics, Indians, Native Americans, and Mediterranean ethnicities, accounting for 7-22.4% of uveitis cases. Retrospective analyses indicate a higher incidence among female patients, with most cases occurring in the second and fifth decades of life. Aim: This case report discusses a patient with probable VKH who exhibited ocular, neurologic, and auditory symptoms typical of the prodromal or acute uveitic phase and responded well to prompt management. Case Presentation: A young female in her late 20s presented with low-grade fever, severe headache, neck pain, and neck stiffness. She had received symptomatic treatment at another hospital without relief. She was empirically started on intravenous antibiotics and dexamethasone for suspected pyogenic meningitis and was discharged upon symptom relief. However, she returned two days later due to symptom recurrence. Ophthalmic examination revealed decreased visual acuity bilaterally (6/24), sluggish pupil reaction, optic disc edema, and bilateral macular serous detachments. Mild vitritis with anterior chamber cells and iris pigment on the anterior lens capsule was noted in the left eye. Systemic examination was unremarkable, except for fine crepitations in the bilateral lower lung fields. Management: Considering VKH disease, the patient was started on intravenous methylprednisolone pulse therapy (1 gram/day) for 3 days, followed by oral steroids and topical steroid drops for the eyes. She was discharged with oral prednisolone and prednisolone acetate 1% eye drops. At follow-up, her vision improved, and there was resolution of papillitis and serous retinal detachments. Conclusions: VKH is a significant cause of bilateral vision loss. This case of probable VKH syndrome underscores the importance of early recognition and aggressive treatment in achieving a favorable visual prognosis.展开更多
The purpose of this review is to provide a comprehensive and updated overview of the clinical features,imaging modalities,differential diagnosis,diagnostic criteria,and treatment options for Vogt-Koyanagi-Harada(VKH)s...The purpose of this review is to provide a comprehensive and updated overview of the clinical features,imaging modalities,differential diagnosis,diagnostic criteria,and treatment options for Vogt-Koyanagi-Harada(VKH)syndrome,a rare progressive inflammatory condition characterized by bilateral granulomatous panuveitis and systemic manifestations.While the clinical features and disease course of VKH syndrome are well-characterized in the literature,its diagnosis is challenging due to a broad differential that include infectious and noninfectious causes of uveitis and rare inflammatory conditions,as well as a lack of a single diagnostic finding on exam,laboratory testing,or imaging.The evolution of the diagnostic criteria for VKH syndrome reflects the growing understanding of the disease by the ophthalmic community and advancement of imaging technology.Findings on enhanced depth imaging(EDI)optical coherence tomography(OCT)and indocyanine green angiography(ICGA)help detect subtle inflammation of the choroid and were incorporated into new diagnostic criteria developed in the last few years.There is limited research on the treatment for acute VKH,but results of studies to date support the early initiation of immunomodulatory therapy(IMT)due to a high recurrence rate and progression to chronic disease in patients treated with monotherapy with high-dose systemic corticosteroids.This review will provide an indepth summary of recent literature on advanced imaging modality and IMT to guide clinicians in their management of patients with VKH syndrome.展开更多
Objective To provide novel spectral domain optical coherence tomography (SD OCT) findings of Vogt-Koyanagi-Harada (VKH) disease as well as new insights into the pathogenesis of this disease. Methods Detailed SD OCT an...Objective To provide novel spectral domain optical coherence tomography (SD OCT) findings of Vogt-Koyanagi-Harada (VKH) disease as well as new insights into the pathogenesis of this disease. Methods Detailed SD OCT and fluorescein angiography (FA) findings of 18 consecutive VKH patients (11 women and 7 men) from December 2007 to April 2009 who were in acute uveitic stage at presentation were reviewed. All the patients had been followed up for at least 6 months with reevaluation(s) of SD OCT performed in 10 patients. Results Intraretinal cysts were found to be located in various layers of the outer retina. In addition to the photoreceptor layer, they could also be found between the outer plexiform layer and the outer nuclear layer, or spanning the external limiting membrane. On FA, intraretinal cysts could be hypofluorescent, normofluorescent, or hyperfluorescent. Some intraretinal cysts had a characteristic FA pattern, in which a small round hypofluorescent area was surrounded by a ring of hyperfluorescence (donut-shaped dye pooling). Subretinal fibrinoid deposit appeared in acute uveitic stage in two severe VKH patients and seemed to develop from subretinal exudates and evolved into typical subretinal fibrosis. Gradual transfiguration/migration and progressive proliferation/pigmentation of the subretinal fibrinoid deposit/subretinal fibrosis was observed in one patient. Conclusions Intraretinal cysts could form in various layers of the outer retina and may result from extension of choroidal inflammation. Subretinal fibrosis may develop from subretinal exudates in VKH patients and may cause substantial visual impairment.展开更多
AIM:To observe the melanin change of the retinal pigment epithelium(RPE)and choroid in the convalescent stage of Vogt-Koyanagi-Harada(VKH).METHODS:A retrospective study was performed on 40 eyes of 20 patients in the c...AIM:To observe the melanin change of the retinal pigment epithelium(RPE)and choroid in the convalescent stage of Vogt-Koyanagi-Harada(VKH).METHODS:A retrospective study was performed on 40 eyes of 20 patients in the convalescent stage of VKH.Fundus photography(FP),multi-spectral imaging(MSI),and optical coherence tomography(OCT)were performed.RESULTS:In the VKH convalescent stage,focal RPE melanin accumulation(FRMA)was detected in 34 eyes(85%)on MSI and in 7 eyes(17.5%)on FP.FRMA was limited to the previous retinal detachment area in all 28 eyes(FRMA was detected in 34 eyes on MSI,which were enrolled,and 6 eyes lacked data in the acute stage).Sunset-glow fundus was detected in 20 eyes(50%)on FP.The mean density of FRMA in a 1-mm-diameter circular area of the fovea was 0.04±0.07 on MSI,which was significantly correlated with sunset-glow fundus(ρ=0.467,P=0.02).CONCLUSION:In the VKH convalescent stage,FRMA is derived from the RPE melanin change,and sunset-glow fundus is derived from the choroid melanin change.A higher density of FRMA in the fovea and sunset-glow fundus represents more serious depigmentation of melanin.展开更多
Dear Editor,Vogt-Koyanagi-Harada（VKH）disease is a cell-mediated autoimmune syndrome directed against melanocytes.It is considered a multisystem disorder characterized by granulomatous panuveitis often associated wit...Dear Editor,Vogt-Koyanagi-Harada（VKH）disease is a cell-mediated autoimmune syndrome directed against melanocytes.It is considered a multisystem disorder characterized by granulomatous panuveitis often associated with neurologic and cutaneous manifestations.展开更多
AIM： To review all cases of Vogt-Koyanagi-Harada （VKH） disease in an Inflammatory Eye Disease Service in Argentina and to describe the clinical profile and outcomes of treatment.METHODS： The records from patients ...AIM： To review all cases of Vogt-Koyanagi-Harada （VKH） disease in an Inflammatory Eye Disease Service in Argentina and to describe the clinical profile and outcomes of treatment.METHODS： The records from patients with VKH disease between January 1980 and December 2008 were retrospectively analyzed for clinical profile, complications, and treatment. Patients were classified according to their initial treatment in group 1： high corticosteroid dose [≥1 mg/（kg·d）] within 2wk of illness onset; group 2： high corticosteroid dose, 2 to 4wk of onset and group 3： patients who received the high dose after 1mo of illness onset, patients who received lower oral doses than 1 mg/（kg·d） without regarding the time of beginning of the disease.RESULTS： A total of 210 eyes of 105 patients were included. The mean age at presentation was 32.6±13y （range： 10-74y）, and 86.7% were female. The mean duration of follow up was 144±96.6mo. Patients in the group 1 had significantly higher visual acuity than the other groups （P〈0.0001）, none had （loss of, or no） light perception at the end of follow up, whereas 24.7% patients in group 3 ended in light perception （P〈0.004）.CONCLUSION： Patients with early high dose corticosteroid treatment have better visual acuity and fewer complications. Proper timing in referral and treatment is critical for better visual outcome in VKH disease.展开更多
Dear Editor,I am Dr.Tian-Wei Qian,from Shanghai General Hospital,Shanghai,China.I write to present one case report of tuberculosis（TB）-associated uveitis after corticosteroid treatment for Vogt-Koyanagi-Harada（VKH...Dear Editor,I am Dr.Tian-Wei Qian,from Shanghai General Hospital,Shanghai,China.I write to present one case report of tuberculosis（TB）-associated uveitis after corticosteroid treatment for Vogt-Koyanagi-Harada（VKH）disease.展开更多
Purpose: Vogt-Koyanagi-Harada (VKH) syndrome is usually defined as an uveo-meningitis who may be associated with auditory and cutaneous signs. Association of VKH syndrome and autoimmune thyroiditis is uncommon. Observ...Purpose: Vogt-Koyanagi-Harada (VKH) syndrome is usually defined as an uveo-meningitis who may be associated with auditory and cutaneous signs. Association of VKH syndrome and autoimmune thyroiditis is uncommon. Observation: A 28-year-old man was admitted with thoracic pain due to myocarditis. Two years ago he presented VKH syndrome with specific ocular manifestation and deafness, treated by corticosteroids and immunosuppressive. Etiologic investigation of myocarditis concluded to deep hypothyroidism related to Hashimoto thyroiditis. The patient improved after substitutive treatment by thyroid hormones. Conclusion: Thyroid function should be systematically investigated in case of VKH syndrome and particularly when associated to dysthyroidism symptoms.展开更多
文摘Background: Vogt-Koyanagi-Harada (VKH) disease is a rare, multisystem autoimmune disorder characterized by bilateral granulomatous panuveitis, with or without extraocular manifestations. Although its exact etiology and pathogenesis remain unclear, it is hypothesized to involve T-cell dysregulation targeting melanocyte-containing tissues, including the CNS, eye, ear, and skin. VKH predominantly affects pigmented groups, such as Asians, Hispanics, Indians, Native Americans, and Mediterranean ethnicities, accounting for 7-22.4% of uveitis cases. Retrospective analyses indicate a higher incidence among female patients, with most cases occurring in the second and fifth decades of life. Aim: This case report discusses a patient with probable VKH who exhibited ocular, neurologic, and auditory symptoms typical of the prodromal or acute uveitic phase and responded well to prompt management. Case Presentation: A young female in her late 20s presented with low-grade fever, severe headache, neck pain, and neck stiffness. She had received symptomatic treatment at another hospital without relief. She was empirically started on intravenous antibiotics and dexamethasone for suspected pyogenic meningitis and was discharged upon symptom relief. However, she returned two days later due to symptom recurrence. Ophthalmic examination revealed decreased visual acuity bilaterally (6/24), sluggish pupil reaction, optic disc edema, and bilateral macular serous detachments. Mild vitritis with anterior chamber cells and iris pigment on the anterior lens capsule was noted in the left eye. Systemic examination was unremarkable, except for fine crepitations in the bilateral lower lung fields. Management: Considering VKH disease, the patient was started on intravenous methylprednisolone pulse therapy (1 gram/day) for 3 days, followed by oral steroids and topical steroid drops for the eyes. She was discharged with oral prednisolone and prednisolone acetate 1% eye drops. At follow-up, her vision improved, and there was resolution of papillitis and serous retinal detachments. Conclusions: VKH is a significant cause of bilateral vision loss. This case of probable VKH syndrome underscores the importance of early recognition and aggressive treatment in achieving a favorable visual prognosis.
文摘The purpose of this review is to provide a comprehensive and updated overview of the clinical features,imaging modalities,differential diagnosis,diagnostic criteria,and treatment options for Vogt-Koyanagi-Harada(VKH)syndrome,a rare progressive inflammatory condition characterized by bilateral granulomatous panuveitis and systemic manifestations.While the clinical features and disease course of VKH syndrome are well-characterized in the literature,its diagnosis is challenging due to a broad differential that include infectious and noninfectious causes of uveitis and rare inflammatory conditions,as well as a lack of a single diagnostic finding on exam,laboratory testing,or imaging.The evolution of the diagnostic criteria for VKH syndrome reflects the growing understanding of the disease by the ophthalmic community and advancement of imaging technology.Findings on enhanced depth imaging(EDI)optical coherence tomography(OCT)and indocyanine green angiography(ICGA)help detect subtle inflammation of the choroid and were incorporated into new diagnostic criteria developed in the last few years.There is limited research on the treatment for acute VKH,but results of studies to date support the early initiation of immunomodulatory therapy(IMT)due to a high recurrence rate and progression to chronic disease in patients treated with monotherapy with high-dose systemic corticosteroids.This review will provide an indepth summary of recent literature on advanced imaging modality and IMT to guide clinicians in their management of patients with VKH syndrome.
文摘Objective To provide novel spectral domain optical coherence tomography (SD OCT) findings of Vogt-Koyanagi-Harada (VKH) disease as well as new insights into the pathogenesis of this disease. Methods Detailed SD OCT and fluorescein angiography (FA) findings of 18 consecutive VKH patients (11 women and 7 men) from December 2007 to April 2009 who were in acute uveitic stage at presentation were reviewed. All the patients had been followed up for at least 6 months with reevaluation(s) of SD OCT performed in 10 patients. Results Intraretinal cysts were found to be located in various layers of the outer retina. In addition to the photoreceptor layer, they could also be found between the outer plexiform layer and the outer nuclear layer, or spanning the external limiting membrane. On FA, intraretinal cysts could be hypofluorescent, normofluorescent, or hyperfluorescent. Some intraretinal cysts had a characteristic FA pattern, in which a small round hypofluorescent area was surrounded by a ring of hyperfluorescence (donut-shaped dye pooling). Subretinal fibrinoid deposit appeared in acute uveitic stage in two severe VKH patients and seemed to develop from subretinal exudates and evolved into typical subretinal fibrosis. Gradual transfiguration/migration and progressive proliferation/pigmentation of the subretinal fibrinoid deposit/subretinal fibrosis was observed in one patient. Conclusions Intraretinal cysts could form in various layers of the outer retina and may result from extension of choroidal inflammation. Subretinal fibrosis may develop from subretinal exudates in VKH patients and may cause substantial visual impairment.
文摘AIM:To observe the melanin change of the retinal pigment epithelium(RPE)and choroid in the convalescent stage of Vogt-Koyanagi-Harada(VKH).METHODS:A retrospective study was performed on 40 eyes of 20 patients in the convalescent stage of VKH.Fundus photography(FP),multi-spectral imaging(MSI),and optical coherence tomography(OCT)were performed.RESULTS:In the VKH convalescent stage,focal RPE melanin accumulation(FRMA)was detected in 34 eyes(85%)on MSI and in 7 eyes(17.5%)on FP.FRMA was limited to the previous retinal detachment area in all 28 eyes(FRMA was detected in 34 eyes on MSI,which were enrolled,and 6 eyes lacked data in the acute stage).Sunset-glow fundus was detected in 20 eyes(50%)on FP.The mean density of FRMA in a 1-mm-diameter circular area of the fovea was 0.04±0.07 on MSI,which was significantly correlated with sunset-glow fundus(ρ=0.467,P=0.02).CONCLUSION:In the VKH convalescent stage,FRMA is derived from the RPE melanin change,and sunset-glow fundus is derived from the choroid melanin change.A higher density of FRMA in the fovea and sunset-glow fundus represents more serious depigmentation of melanin.
文摘Dear Editor,Vogt-Koyanagi-Harada（VKH）disease is a cell-mediated autoimmune syndrome directed against melanocytes.It is considered a multisystem disorder characterized by granulomatous panuveitis often associated with neurologic and cutaneous manifestations.
文摘AIM： To review all cases of Vogt-Koyanagi-Harada （VKH） disease in an Inflammatory Eye Disease Service in Argentina and to describe the clinical profile and outcomes of treatment.METHODS： The records from patients with VKH disease between January 1980 and December 2008 were retrospectively analyzed for clinical profile, complications, and treatment. Patients were classified according to their initial treatment in group 1： high corticosteroid dose [≥1 mg/（kg·d）] within 2wk of illness onset; group 2： high corticosteroid dose, 2 to 4wk of onset and group 3： patients who received the high dose after 1mo of illness onset, patients who received lower oral doses than 1 mg/（kg·d） without regarding the time of beginning of the disease.RESULTS： A total of 210 eyes of 105 patients were included. The mean age at presentation was 32.6±13y （range： 10-74y）, and 86.7% were female. The mean duration of follow up was 144±96.6mo. Patients in the group 1 had significantly higher visual acuity than the other groups （P〈0.0001）, none had （loss of, or no） light perception at the end of follow up, whereas 24.7% patients in group 3 ended in light perception （P〈0.004）.CONCLUSION： Patients with early high dose corticosteroid treatment have better visual acuity and fewer complications. Proper timing in referral and treatment is critical for better visual outcome in VKH disease.
文摘Dear Editor,I am Dr.Tian-Wei Qian,from Shanghai General Hospital,Shanghai,China.I write to present one case report of tuberculosis（TB）-associated uveitis after corticosteroid treatment for Vogt-Koyanagi-Harada（VKH）disease.
文摘Purpose: Vogt-Koyanagi-Harada (VKH) syndrome is usually defined as an uveo-meningitis who may be associated with auditory and cutaneous signs. Association of VKH syndrome and autoimmune thyroiditis is uncommon. Observation: A 28-year-old man was admitted with thoracic pain due to myocarditis. Two years ago he presented VKH syndrome with specific ocular manifestation and deafness, treated by corticosteroids and immunosuppressive. Etiologic investigation of myocarditis concluded to deep hypothyroidism related to Hashimoto thyroiditis. The patient improved after substitutive treatment by thyroid hormones. Conclusion: Thyroid function should be systematically investigated in case of VKH syndrome and particularly when associated to dysthyroidism symptoms.
