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
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
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.