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
AIM: To assess helper T(Th) lymphocyte subset balance in patients with Vogt-Koyanagi-Harada(VKH) disease. METHODS: Sixty-eight active VKH patients and seventytwo inactive VKH patients were included in this study. One ...AIM: To assess helper T(Th) lymphocyte subset balance in patients with Vogt-Koyanagi-Harada(VKH) disease. METHODS: Sixty-eight active VKH patients and seventytwo inactive VKH patients were included in this study. One hundred healthy individuals served as controls. Peripheral blood was obtained from VKH patients and healthy controls. Th lymphocyte subsets were analyzed by flow cytometry. Plasma concentration of interleukin(IL)-17, IL-10, transforming growth factor(TGF)-β, IL-23 and IL-6 was examined by enzyme-linked immunosorbent assay(ELISA). RESULTS: VKH patients with active uveitis had significantly higher percentages of both Th1 and Th17 cells and lower percentages of regulatory T(Treg) cells as compared with inactive VKH patients and healthy controls. Th1/Th2 and Th17/Treg ratios were also significantly elevated in active VKH patients. The percentages of Th1, Th17 and Treg cells and the Th1/Th2, Th17/Treg ratio did not differ between inactive VKH patients and healthy controls. There was no difference concerning the percentage of Th2 cells among all the groups. VKH patients with active uveitis showed an elevated level of peripheral Th17 related cytokines levels(TGF-β, IL-6, IL-23, and IL-17) and a decreased level of Treg related cytokines(IL-10) compared with inactive VKH patients and healthy controls. Inactive VKH patients showed no differences in peripheral Th17 related cytokines(TGF-β, IL-6, IL-23, and IL-17) and Treg related cytokines(IL-10) levels compared with healthy controls. CONCLUSION: Th1 and Th17 cells are significantly increased and Treg cells significantly decreased in active VKH compared with inactive VKH or healthy controls. Therefore, Th lymphocyte subset analysis may serve as a disease biomarker for VKH.展开更多
Vogt-Koyanagi-Harada syndrome(VKH)is a bilateral granulomatous panuveitis associated with serous retinal detachments and vitritis,and can be associated with extraocular manifestations of meningismus,poliosis,vitiligo,...Vogt-Koyanagi-Harada syndrome(VKH)is a bilateral granulomatous panuveitis associated with serous retinal detachments and vitritis,and can be associated with extraocular manifestations of meningismus,poliosis,vitiligo,hearing loss,and headaches.It is mediated by CD4+T cells that target melanocytes in the eye,ear,meninges,and skin.It classically presents in 4 different phases:prodromal,uveitic,convalescent,and recurrent.There have been considerable advances in our understanding of the disease in recent years,and options for treatment have also expanded beyond systemic corticosteroids though these remain the mainstay of therapy in patients with VKH.This brief review will focus on updates in the diagnosis and treatment of VKH,specifically advances in imaging techniques including the use of optical coherence tomography angiography(OCTA)and enhanced depth imaging(EDI)optical coherence tomography(OCT).OCT parameters that are diagnostically predictive of acute VKH compared to other exudative maculopathies include the presence of subretinal membranous structures,a high retinal detachment,subretinal hyperreflective dots,and RPE folds.Evaluations of choroidal thickness using EDI-OCT demonstrate predominant involvement of the outer choroid in the acute inflammatory phase of VKH,consistent with histopathological analysis.OCTA may emerge as an alternative to fluorescein angiography(FA)and indocyanine angiography(ICGA)but is limited at this time due to its small field of view.While the mainstay of treatment of acute VKH continues to be systemic corticosteroids,biological response modifiers(BRMs)such as adalimumab and infliximab have been shown to be effective in the management of adult and pediatric VKH with one benefit being a faster onset of action compared to conventional immunosuppression.Literature Search:A literature search was done in PubMed using the words“Vogt Koyanagi Harada”“imaging”“diagnosis”“treatment”“therapy“posterior uveitis”.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
Thirty patients with Vogt-Koyanagi-Haradas disease were typed for HLA-A and HLA-B antigenic determinants by a microlymphocytotoxicity technique. HLA-B22 antigen showed an increased frequency of 43.3% in the patient gr...Thirty patients with Vogt-Koyanagi-Haradas disease were typed for HLA-A and HLA-B antigenic determinants by a microlymphocytotoxicity technique. HLA-B22 antigen showed an increased frequency of 43.3% in the patient group(relative risk=8.69; exact P<0.0001; corrected P<0.0025) compared with normal control group(frequency=7.69%). This association suggests that immunogenetic factor may play an important role in the pathogenesis of Vogt-Koyanagi-Harada's disease.展开更多
Superior oblique palsy is the most common isolated cranial nerve palsy and is commonly associated with exotropia. Modified Harada-Ito procedure can be used to correct excyclotorsion in symptomatic patients. We report ...Superior oblique palsy is the most common isolated cranial nerve palsy and is commonly associated with exotropia. Modified Harada-Ito procedure can be used to correct excyclotorsion in symptomatic patients. We report a case of acquired superior oblique palsy with exotropia undergoing modified Harada-Ito procedure. A 33 years old man presented with torsional diplopia. On examination there was 10 degrees of left excyclotorsion on double Maddox rod with 25-diopter of exotropia on primary gaze and 20-diopter on lateral gaze suggestive of lateral incomitancy. He underwent a Fells modification of Harada-Ito procedure with bilateral rectus recession. Three months postoperative, he was orthophoric. Modified Harada-Ito procedure is a successful procedure in overcoming torsional diplopia. It is important to identify lateral incomitancy to prevent overcorrection of the horizontal deviation.展开更多
Purpose: To discuss the relationship of Bovine Melanin-Associated Antigen (BMAA) to Sympathetic Ophthalmia (SO) and Vogt-Kayanagi-Harada (VKH) syndrome.Methods:BMAA was isolated and purified from bovine iris and cilia...Purpose: To discuss the relationship of Bovine Melanin-Associated Antigen (BMAA) to Sympathetic Ophthalmia (SO) and Vogt-Kayanagi-Harada (VKH) syndrome.Methods:BMAA was isolated and purified from bovine iris and ciliary body and MTT method was used to measure the peripheral lymphocyte reactions of SO and VKH syndrome patients to BMAA. 15 cases of SO were included in the SO group with 11 males and 4 females. The average age was 37.5, ranging from 23 to 56 years; 15 patients with VKH syndrome were included in the VHK group with 7 males and 8 females. Their average age was 37.2 years, ranging from 24 to 69 years; 20 normal individuals without ocular and systemic autoimmune diseases were included in the control group.These included 9 males and 11 females, aging from 14 to 28 with an average of 23.8 years.Results: The OD values of each group were as follows: SO controls 0.327+0.032; SO+ BMAA 0.490+0.758; SO+PHA 0.5310+0.918; VKH controls 0.328+0.503; VKH+BMAA 0.430 +0.530; VKH +PHA 0.328 +0.484;Normal controls 0.304 +0.267; Normal +BMAA 0.343+0.326; Normal+PHA 0.477+0.598. The average OD values of peripheral lymphocytes to BMAA stimulation in SO patients and normal controls were 0.490 and 0.343 respectively. The difference was statistically significant, P＜ 0.001; The average OD values of peripheral lymphocytes to BMAA stimulation in SO and VKH syndrome patients were 0.470 and 0.430 respectively. The difference was not statistically significant,P ＞ 0.05.Conclusions:BMAA effectively stimulate the proliferative reaction of peripheral lymphocytes in SO patients and BMAA may act as an antigen and contribute in the pathological process of SO.No difference in proliferative reaction of peripheral lymphocytes was found between SO and VKH syndrome patients after BMAA stimulations and BMAA may be involved in the development of both SO and VKH yndrome.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.
基金Supported by National Natural Science Foundation of China(No.30772013)Basic Research Program of Chongqing(No.cstc2015jcyjA10112)
文摘AIM: To assess helper T(Th) lymphocyte subset balance in patients with Vogt-Koyanagi-Harada(VKH) disease. METHODS: Sixty-eight active VKH patients and seventytwo inactive VKH patients were included in this study. One hundred healthy individuals served as controls. Peripheral blood was obtained from VKH patients and healthy controls. Th lymphocyte subsets were analyzed by flow cytometry. Plasma concentration of interleukin(IL)-17, IL-10, transforming growth factor(TGF)-β, IL-23 and IL-6 was examined by enzyme-linked immunosorbent assay(ELISA). RESULTS: VKH patients with active uveitis had significantly higher percentages of both Th1 and Th17 cells and lower percentages of regulatory T(Treg) cells as compared with inactive VKH patients and healthy controls. Th1/Th2 and Th17/Treg ratios were also significantly elevated in active VKH patients. The percentages of Th1, Th17 and Treg cells and the Th1/Th2, Th17/Treg ratio did not differ between inactive VKH patients and healthy controls. There was no difference concerning the percentage of Th2 cells among all the groups. VKH patients with active uveitis showed an elevated level of peripheral Th17 related cytokines levels(TGF-β, IL-6, IL-23, and IL-17) and a decreased level of Treg related cytokines(IL-10) compared with inactive VKH patients and healthy controls. Inactive VKH patients showed no differences in peripheral Th17 related cytokines(TGF-β, IL-6, IL-23, and IL-17) and Treg related cytokines(IL-10) levels compared with healthy controls. CONCLUSION: Th1 and Th17 cells are significantly increased and Treg cells significantly decreased in active VKH compared with inactive VKH or healthy controls. Therefore, Th lymphocyte subset analysis may serve as a disease biomarker for VKH.
文摘Vogt-Koyanagi-Harada syndrome(VKH)is a bilateral granulomatous panuveitis associated with serous retinal detachments and vitritis,and can be associated with extraocular manifestations of meningismus,poliosis,vitiligo,hearing loss,and headaches.It is mediated by CD4+T cells that target melanocytes in the eye,ear,meninges,and skin.It classically presents in 4 different phases:prodromal,uveitic,convalescent,and recurrent.There have been considerable advances in our understanding of the disease in recent years,and options for treatment have also expanded beyond systemic corticosteroids though these remain the mainstay of therapy in patients with VKH.This brief review will focus on updates in the diagnosis and treatment of VKH,specifically advances in imaging techniques including the use of optical coherence tomography angiography(OCTA)and enhanced depth imaging(EDI)optical coherence tomography(OCT).OCT parameters that are diagnostically predictive of acute VKH compared to other exudative maculopathies include the presence of subretinal membranous structures,a high retinal detachment,subretinal hyperreflective dots,and RPE folds.Evaluations of choroidal thickness using EDI-OCT demonstrate predominant involvement of the outer choroid in the acute inflammatory phase of VKH,consistent with histopathological analysis.OCTA may emerge as an alternative to fluorescein angiography(FA)and indocyanine angiography(ICGA)but is limited at this time due to its small field of view.While the mainstay of treatment of acute VKH continues to be systemic corticosteroids,biological response modifiers(BRMs)such as adalimumab and infliximab have been shown to be effective in the management of adult and pediatric VKH with one benefit being a faster onset of action compared to conventional immunosuppression.Literature Search:A literature search was done in PubMed using the words“Vogt Koyanagi Harada”“imaging”“diagnosis”“treatment”“therapy“posterior uveitis”.
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Thirty patients with Vogt-Koyanagi-Haradas disease were typed for HLA-A and HLA-B antigenic determinants by a microlymphocytotoxicity technique. HLA-B22 antigen showed an increased frequency of 43.3% in the patient group(relative risk=8.69; exact P<0.0001; corrected P<0.0025) compared with normal control group(frequency=7.69%). This association suggests that immunogenetic factor may play an important role in the pathogenesis of Vogt-Koyanagi-Harada's disease.
文摘Superior oblique palsy is the most common isolated cranial nerve palsy and is commonly associated with exotropia. Modified Harada-Ito procedure can be used to correct excyclotorsion in symptomatic patients. We report a case of acquired superior oblique palsy with exotropia undergoing modified Harada-Ito procedure. A 33 years old man presented with torsional diplopia. On examination there was 10 degrees of left excyclotorsion on double Maddox rod with 25-diopter of exotropia on primary gaze and 20-diopter on lateral gaze suggestive of lateral incomitancy. He underwent a Fells modification of Harada-Ito procedure with bilateral rectus recession. Three months postoperative, he was orthophoric. Modified Harada-Ito procedure is a successful procedure in overcoming torsional diplopia. It is important to identify lateral incomitancy to prevent overcorrection of the horizontal deviation.
基金This research has been supported by Guangzhou Scientific Research Fund and Sun Yat-sen University Scientific Research Fund
文摘Purpose: To discuss the relationship of Bovine Melanin-Associated Antigen (BMAA) to Sympathetic Ophthalmia (SO) and Vogt-Kayanagi-Harada (VKH) syndrome.Methods:BMAA was isolated and purified from bovine iris and ciliary body and MTT method was used to measure the peripheral lymphocyte reactions of SO and VKH syndrome patients to BMAA. 15 cases of SO were included in the SO group with 11 males and 4 females. The average age was 37.5, ranging from 23 to 56 years; 15 patients with VKH syndrome were included in the VHK group with 7 males and 8 females. Their average age was 37.2 years, ranging from 24 to 69 years; 20 normal individuals without ocular and systemic autoimmune diseases were included in the control group.These included 9 males and 11 females, aging from 14 to 28 with an average of 23.8 years.Results: The OD values of each group were as follows: SO controls 0.327+0.032; SO+ BMAA 0.490+0.758; SO+PHA 0.5310+0.918; VKH controls 0.328+0.503; VKH+BMAA 0.430 +0.530; VKH +PHA 0.328 +0.484;Normal controls 0.304 +0.267; Normal +BMAA 0.343+0.326; Normal+PHA 0.477+0.598. The average OD values of peripheral lymphocytes to BMAA stimulation in SO patients and normal controls were 0.490 and 0.343 respectively. The difference was statistically significant, P＜ 0.001; The average OD values of peripheral lymphocytes to BMAA stimulation in SO and VKH syndrome patients were 0.470 and 0.430 respectively. The difference was not statistically significant,P ＞ 0.05.Conclusions:BMAA effectively stimulate the proliferative reaction of peripheral lymphocytes in SO patients and BMAA may act as an antigen and contribute in the pathological process of SO.No difference in proliferative reaction of peripheral lymphocytes was found between SO and VKH syndrome patients after BMAA stimulations and BMAA may be involved in the development of both SO and VKH yndrome.
