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1/20. Vogt-Koyanagi-Harada disease: extensive vitiligo with prodromal generalized erythroderma.

    The occurrence of vitiligo with prodromal erythema in Vogt-Koyanagi-Harada (VKH) disease is rarely observed. We describe a 68-year-old Chinese woman who developed extensive vitiligo 6 months after the onset of a generalized erythroderma and 12 months after the onset of acute bilateral uveitis. She also had a 4-year history of psoriatic plaques on the trunk which resolved completely without recurrence 15 months after the onset of erythroderma. The extensive cutaneous inflammation prior to the onset of vitiligo in our patient with VKH disease has not been reported before and strongly suggests an immunologic etiology for VKH disease.
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keywords = inflammation
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2/20. Vogt-Koyanagi-Harada syndrome after cutaneous injury.

    OBJECTIVE: To describe three patients who developed Vogt-Koyanagi-Harada syndrome (VKH) after cutaneous injury. DESIGN: Retrospective case series. PARTICIPANTS: Three patients seen in the uveitis clinic at Aravind eye Hospital and Postgraduate Institute of ophthalmology, Madurai, india, participated. MAIN OUTCOME MEASURES: The history, evaluation, and management of the three patients were summarized. RESULTS: Three patients developed VKH syndrome shortly after cutaneous injury. In each case, the affected area of skin became vitiliginous on healing and simultaneous with the onset of ocular symptoms. One patient developed additional ectopic areas of vitiligo. All three patients developed chronic, bilateral, diffuse uveitis, one associated with an exudative retinal detachment and two with Dalen-Fuchs-like nodules. Well-recognized complications of VKH syndrome that occurred in the authors' patients included geographic atrophy of the retinal pigment epithelium (3 of 3), cataract (3 of 3), and glaucoma (1 of 3). Ocular inflammation was well controlled in each patient with local or systemic corticosteroids or both. In one patient, the area of vitiligo showed increased pigmentation in response to systemic corticosteroid treatment. CONCLUSIONS: Vogt-Koyanagi-Harada syndrome may follow cutaneous injury, supporting the notion that this disorder may result from systemic sensitization to shared melanocytic antigens.
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keywords = inflammation
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3/20. Treatment of Vogt-Koyanagi- Harada's disease during pregnancy.

    BACKGROUND: Caution should be exercised in treating patients with autoimmune diseases during pregnancy. CASES: We successfully treated three cases of Vogt-Koyanagi-Harada's disease (VKH disease) during pregnancy. OBSERVATIONS: In the second trimester (14-27 weeks) of 1 patient, inflammation was mild and could be treated by topical corticosteroid. There is the possibility that her immune response had been modified by pregnancy. Systemic corticosteroid in a high dose was administered to the two cases in the second and third trimesters of pregnancy (28-41 weeks). The severity of inflammation in these 2 patients was similar to that in nonpregnant women. inflammation subsided immediately without recurrence in all cases. No abnormality was found during the deliveries or in the babies. CONCLUSIONS: Treatment for VKH disease during pregnancy should be chosen according to the severity of inflammation, the stage of pregnancy, and the maternal and fetal conditions.
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ranking = 3
keywords = inflammation
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4/20. Corticosteroid-induced central serous chorioretinopathy in patients with ocular inflammatory disorders.

    BACKGROUND: Development of central serous chorioretinopathy (CSC) following the administration of corticosteroids by diverse routes is a well-known fact. We report acute visual loss after the use of systemic corticosteroids in three patients with long-standing ocular inflammatory disorders in whom CSC could initially be misinterpreted as a worsening of the primary inflammatory condition. methods: We analyzed the clinical findings and the fluorescein and indocyanine green (ICG) angiographic signs in those three patients. RESULTS: The first patient had birdshot chorioretinopathy with minimal functional impairment for several years without treatment. When visual acuity and fields deteriorated, systemic corticosteroids were administered resulting in improved inflammatory and functional parameters during the first 2 months. Subsequently, the visual acuity of his left eye decreased due to CSC. The second patient had Vogt-Koyanagi-Harada disease with five episodes of acute inflammation. She was treated each time with systemic corticosteroids, but thrice her visual acuity deteriorated, caused by CSC. The third patient presented with scleritis of his right eye related to relapsing polychondritis. Massive oral corticosteroids were given, soon followed by the development of CSC in the right eye. Fluorescein and ICG angiographic signs were typical for CSC in all three patients. cyclosporine was introduced in the two first patients and cyclophosphamide in the third patient, in parallel with tapering of oral corticosteroids. Progressive regression of CSC occurred in all three patients. CONCLUSION: The potentially deleterious effects of corticosteroids, favoring CSC, are well-known. They should be borne in mind when an unexpected clinical and angiographic evolution compatible with CSC develops in an uveitis patient treated with corticosteroids.
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keywords = inflammation
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5/20. Early manifestation of vogt-koyanagi-harada disease as unilateral posterior scleritis.

    PURPOSE: To describe a patient with an initial diagnosis of unilateral posterior scleritis who developed bilateral Vogt-Koyanagi-Harada (VKH) disease 12 months later.CASE: A 38-year-old woman was diagnosed with posterior scleritis in her right eye at successive examinations. The left eye showed no signs of ocular inflammation.OBSERVATIONS: Her right eye developed a second attack of posterior scleritis 3 months after the initial diagnosis. Twelve months after the first incident, granulomatous uveitis and sunset glow fundus were observed in both eyes. At the same time, lymphocytosis was found in the cerebrospinal fluid, and she was HLA DR-4-positive. In addition, poliosis was noted.CONCLUSIONS: The final diagnosis was primarily VKH disease with presenting signs and symptoms of unilateral posterior scleritis. We therefore recommend that when following a patient with posterior scleritis include VKH disease in the differential diagnosis.
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keywords = inflammation
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6/20. Delayed regeneration of foveal cone photopigments in Vogt-Koyanagi-Harada disease at the convalescent stage.

    PURPOSE: To evaluate the physiological characteristics of the macula in patients with Vogt-Koyanagi-Harada disease during the convalescent stage with specific reference to the kinetics of foveal cone photopigment regeneration. methods: Six eyes of three patients at the convalescent stage of Vogt-Koyanagi-Harada disease were studied. All the eyes had best corrected visual acuity of 1.0 or better and had had no recurrence of inflammation for at least 12 months after the last episode. Foveal cone densitometry (FCD), focal macular electroretinograms, color vision tests, two-color perimetry, and optical coherence tomography (OCT) were performed. RESULTS: No regeneration of cone photopigments was detected within the 7-minute testing time by FCD in all eyes at the first examination after the last episode. However, the other functional tests were normal, and the OCT-determined macular morphology was also normal. The regeneration kinetics of the foveal cone photopigment improved in three of six eyes at 36, 37, and 19 months after the last episode, whereas the other three remained delayed at 18, 18, and 49 months. CONCLUSIONS: These findings suggest that a disorder of the foveal cone photopigment regeneration, and its recovery, requires a significantly longer time than that of other macular functions in some patients with Vogt-Koyanagi-Harada disease.
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ranking = 1
keywords = inflammation
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7/20. Annular choroidal detachment in a patient with Vogt-Koyanagi-Harada disease.

    BACKGROUND: Vogt-Koyanagi-Harada (VKH) disease is one of the diseases that show choroidal detachment. However, there are few reports about VKH disease associated with annular choroidal detachment. In this study, we describe an unusual case of VKH disease with an annular choroidal detachment and absence of a serous retinal detachment. methods: The study was an interventional case report. A 59-year-old Japanese woman presented with bilateral blurred vision with meningismus. Laboratory and clinical investigations were performed, including fluorescein angiography, B-scan echography, computed tomography scans, magnetic resonance imaging, cerebrospinal fluid examination, and blood examination including HLA typing. After a diagnosis of VKH disease was made, the patient was treated with systemic corticosteroids. RESULTS: The patient had iridocyclitis, optic disc swelling, choroidal thickening, and annular choroidal detachment in both eyes. Ophthalmoscopic examination did not reveal an obvious serous retinal detachment. fluorescein angiography showed prominent leakage of dye from the optic disc and mild dye pooling in the subretinal space. The sclera was not thickened, and the axial lengths were relatively short. The findings were bilateral and symmetrical. cerebrospinal fluid pleocytosis and HLA typing were consistent with VKH disease. After systemic corticosteroid treatment, the abnormal findings were resolved. Neither sunset glow fundus nor cutaneous lesions were observed, and no recurrences were observed during 4 years of follow-up. CONCLUSION: The annular choroidal detachment was probably caused by severe choroidal inflammation and resistance to scleral outflow because of the scleral abnormality due to inflammation and short axial length. We believe that intact and functioning retinal pigment epithelium prevented serous retinal detachment. This case reports annular choroidal detachment without retinal detachment associated with VKH diseases.
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ranking = 2
keywords = inflammation
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8/20. magnetic resonance imaging of choroidal inflammation in Vogt-Koyanagi-Harada disease.

    Acute binocular visual loss, photophobia, headache, and pulsatile tinnitus developed in a 51-year-old woman. Ophthalmologic examination showed bilateral optic disc edema with peripapillary nerve fiber layer hemorrhages. Lumbar puncture disclosed a monocytic pleocytosis. A diagnosis of Vogt-Koyanagi-Harada disease was made. magnetic resonance imaging showed striking enhancement and thickening of the posterior ocular wall. A macular star figure appeared several days after prednisone treatment was begun. Laboratory evaluation was entirely negative. Within weeks, the clinical manifestations had resolved except for retinal striae. This is the third report of the magnetic resonance imaging visualization of choroidal inflammation in Vogt-Koyanagi-Harada disease and shows the imaging abnormalities in finer detail than earlier reports.
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ranking = 5
keywords = inflammation
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9/20. Vogt-Koyanagi-Harada associated with diabetes mellitus and celiac disease in a 3-year-old girl.

    PURPOSE: To present a case of Vogt-Koyanagi-Harada (VKH) associated with type I diabetes mellitus and celiac disease in a 3 year old female. methods: We studied a three-year old female who presented with clinical manifestation of VKH and type I diabetes mellitus and celiac disease. RESULTS: Patient was found to have hyperglycemia with type I diabetes mellitus. Duodenal mucosal biopsy specimen confirmed the diagnosis of celiac disease. Patient's ocular inflammation was treated by topical and systemic corticosteroid and immune-suppressive therapy. Her diabetes mellitus was controlled by insulin and her celiac disease was controlled by gluten-free diet. CONCLUSIONS: The association of VKH with two autoimmune diseases (celiac disease and type I diabetes mellitus) is rare. This case is, to our knowledge, the youngest patient reported with VKH.
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ranking = 1
keywords = inflammation
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10/20. Favorable response to high-dose infliximab for refractory childhood uveitis.

    OBJECTIVE: uveitis in children most commonly is associated with juvenile idiopathic arthritis. In addition to topical glucocorticoids, treatment may include systemic immunosuppressive agents. Tumor necrosis factor alpha (TNF-alpha) has been implicated in the pathogenesis of uveitis; therefore, TNF-alpha blockade seems to be a reasonable therapeutic option to investigate. We report successful treatment of children with uveitis using infliximab. STUDY DESIGN: A retrospective study of our complete experience using infliximab for the treatment of childhood uveitis was conducted. PARTICIPANTS: Seventeen children (14 females, 3 males) with chronic uveitis were administered high-dose infliximab (10-20 mg/kg/dose). MAIN OUTCOME MEASURES: Our main outcome measure was the ability to eliminate all signs of intraocular inflammation. RESULTS: All 17 patients demonstrated a dramatic, rapid response, with no observed inflammation in 13 patients after the second infusion, and 4 patients requiring 3 to 7 infusions to achieve disease quiescence. Additional immunosuppressives and topical glucocorticoids were tapered when patients achieved no intraocular inflammation. CONCLUSIONS: In this series, high-dose infliximab was a rapidly effective, well-tolerated therapeutic agent for the treatment of chronic, medically refractory, noninfectious uveitis.
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ranking = 3
keywords = inflammation
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