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Sympathetic Ophthalmia
Ophthalmia Sympathetic

Presentation

Six of eight patients (75%) presented with anterior chamber reaction. All eight patients presented with a vitreous inflammatory response. The optic nerve was inflamed clinically or angiographically in four of eight cases (50%). [ncbi.nlm.nih.gov]

Entire Body System

  • Recurrent Inflammation

    Twelve months after the onset of inflammation, the patient’s condition was stable on a combination of oral cyclosporine and topical steroids. Sunset glow retinal changes remain, but there has been no evidence of recurrent inflammation. [globaljournals.org]

    James Folk of the University of Iowa Hospitals and Clinics, and colleagues, the implant, an approved noninfectious uveitis treatment, is a useful second-line therapy for sympathetic ophthalmia patients who have recurrent inflammation or can’t tolerate [isteroids.com]

Cardiovascular

  • Hypertension

    […] slow-release application of steroids has enabled patients to live free from diabetic treatment and immunosuppressive medication after 21 years of systemic immunosuppressive therapy with secondary Cushing disease including diabetes mellitusand arterial hypertension [ncbi.nlm.nih.gov]

    SD-OCT, FFA) Sympathetic ophthalmia 交感性眼炎, 組織学 Toxoplasmosis Tuberculosis of the Choroid Vitiliginous Chorioretinitis (Birdshot Retinochoroidopathy) Postsurgical Cases Trauma Tumors Vascular Diseases Congenital Syndromes Conjunctiva Cornea Glaucomas Hypertensive [atlasophthalmology.net]

Ears

  • Hearing Impairment

    In operated patients, another interpretation of this observation is that there might be a chronic CSF leak, as these are known to impair hearing on both sides. [dizziness-and-balance.com]

Skin

  • Alopecia

    Systemic examination was unremarkable especially for skin lesions (e.g., alopecia and vitiligo) and auditory dysfunction. Figure 4: Spectral domain optical coherence tomography (macular line scan) of left eye. [egyptretinaj.com]

    […] in TBX21, GATA3, Rorc, Foxp3 and Susceptibility to Behcet's Disease and Vogt-Koyanagi-Harada Syndrome Dan Liao, Shengping Hou, Jun Zhang, Jing Fang, Yunjia Liu, Lin Bai, Qingfeng Cao, Aize Kijlstra & Peizeng Yang Scientific Reports (2015) Reversible alopecia [nature.com]

    Cutaneous and neurologic changes (alopecia, poliosis, vitiligo, dysacousia, tinnitus, vertigo, and cells in the cerebrospinal fluid), which are classically associated with Vogt-Koyanagi-Harada syndrome, may rarely accompany SO. 12 On examination Bilateral [slideplayer.com]

    Reversible alopecia in Vogt-Koyanagi-Harada disease and sympathetic ophthalmia. J Ophthalmic Inflamm Infect 2013;3:41. [ PUBMED ] 4. Chuah SY, Lyne AJ, Dronfield MW. Vogt-Koyanagi-Harada syndrome, a rare association of Hodgkin's disease. [ojoonline.org]

    In a large retrospective study out of China, 24% of patients with SO had meningismus, 25% tinnitus, 20% dysacusis, 13% alopecia and 11% poliosis.[25] Only retinitis and retinal vasculitis are not typically seen in SO. [eyewiki.aao.org]

  • Macula

    We report a case of sympathetic ophthalmia with subretinal neovascularization of the macula. The subretinal neovascularization developed after penetrating trauma to the fellow eye. [ncbi.nlm.nih.gov]

    […] abnormalities and/or SO may be associated with the following conditions: Choroidal neovascularization (CNV): An abnormal development of blood vessels in the choroid, which if left untreated can result in permanent loss of vision Macular edema (fluid in the macula [dovemed.com]

    Bruch's membrane (corresponding to Dalen-Fuchs nodules) Absence of inflammatory involvement of the choriocapillaris and retina Phagocytosis of uveal pigment by epithelioid cells Extension of the granulomatous process into the scleral canals, optic disc, macula [webeye.ophth.uiowa.edu]

Eyes

  • Retinal Pigmentation

    SD-OCT revealed hyper-reflective lesions at the level of the retinal pigment epithelium with disruption of the inner segment/outer segment (IS/OS) junction. [ncbi.nlm.nih.gov]

    Recent experimental studies suggest that this preservation of choriocapillaris could be the result of anti-inflammatory products secreted by the retinal pigment epithelium, including transforming growth factor-beta and a novel protein called retinal pigment [nature.com]

  • Anterior Uveitis

    The commonest presenting signs were anterior uveitis and exudative detachment. All patients were treated with systemic steroids, while 7 patients received additional immunosuppressive therapy. Eight patients had a follow-up of more than 6 months. [ncbi.nlm.nih.gov]

    Clinical presentation is an insidious or acute anterior uveitis with mutton-fat keratic precipitates. The posterior segment manifests moderate to severe vitritis, usually accompanied by multiple yellowish-white choroidal lesions. [doi.org]

  • Photophobia

    Six weeks after the injury he complained of photophobia and redness in his left eye. Visual acuity without correction was: right eye (RE), no light perception; and left eye (LE), 1.0. [ncbi.nlm.nih.gov]

    Symptoms include photophobia, redness, and blurred vision; in some cases, there are also "floaters" and possibly pain. [tsbvi.edu]

    There is often a history of blurry, distorted vision and photophobia in the normal eye (or sympathetic eye, hence the name of this condition), as previously all these features can develop gradually. [duttongregory.co.uk]

    Clinical description Patients present with pain, photophobia, paresis of accommodation, metamorphopsia and mild to significant visual loss. [orpha.net]

  • Blurred Vision

    Five days after the onset of hearing loss, she presented with sudden bilateral blurred vision. In Case 2, a 32-year-old female noticed acute bilateral hearing loss and also experienced acute bilateral blurred vision the subsequent day. [ncbi.nlm.nih.gov]

    Symptoms include photophobia, redness, and blurred vision; in some cases, there are also "floaters" and possibly pain. [tsbvi.edu]

    The signs and symptoms can range from reduced vision to permanent vision loss and may include: Small specks, called floaters, that may be seen moving through one's field of vision Blurred vision Reduced vision or vision loss is observed in a majority [dovemed.com]

    There is usually no pain when this condition first sets in, but the individual suffering from SO will begin to experience blurred vision as the eye loses its ability to focus properly. [eyehealthweb.com]

  • Conjunctival Injection

    Symptoms Patients usually present with symptoms of uveitis: blurred vision, ocular discomfort, conjunctival injection. [eyewiki.aao.org]

    Classically this is accompanied by conjunctival injection and a granulomatous anterior chamber reaction with mutton-fat keratic precipitates (KPs) on the corneal endothelium. In the posterior segment, the extent of inflammation can vary. [scienceopen.com]

Psychiatrical

  • Suggestibility

    These pathologic findings suggest a possible mechanism by which local antigen processing by subconjunctival melanophages may play a role in the initiation of the complex cell-mediated response seen in SO. [ncbi.nlm.nih.gov]

Neurologic

  • Headache

    One month after the second vitrectomy for a recurred retinal detachment, she presented with decreased visual acuity and metamorphopsia in both eyes, headache, tinnitus, and facial swelling. [ncbi.nlm.nih.gov]

    Extraocular symptoms include headache, meningitis or cerebrospinal fluid pleocytosis, hearing loss, poliosis and vitiligo. Etiology The etiology of SO is not completely understood. [orpha.net]

  • Papilledema

    […] sympathizing eye is relatively advanced, enucleation of the injured eye is not recommended; visual function is likely to be severely impaired in both eyes, but the injured eye may turn out to be the better of the two eyes, since optic nerve damage (due to papilledema [tsbvi.edu]

    Swelling of the optic disc (papilledema), secondary glaucoma, vitiligo, and poliosis of the eyelashes may accompany SO. [en.wikipedia.org]

    Papilledema, secondary glaucoma, vitiligo, and poliosis of the eyelashes may accompany SO. In approximately 80% of cases, the uveitis appears within 2-12 weeks after injury, and 90% occur within 1 year from the time of injury. [wikidoc.org]

Workup

Steroids should be initiated as soon as the diagnosis is made in the absence of other contraindications and once infectious workup is negative. [eyewiki.aao.org]

If lymphoma is suspected, careful systemic workup, including neurological evaluation, should be performed. If necessary, a vitreous sample must be obtained for diagnostic purposes. [ncbi.nlm.nih.gov]

HLA Type

  • HLA-A2

    A2, HLA-B51, and HLA-D4. [ojoonline.org]

Treatment

Management and treatment Prompt and careful closure of all wounds decreases the risk of SO. [orpha.net]

RESULTS: Six patients were diagnosed with sympathetic ophthalmia following treatment with diode laser CPC, an incidence of 0.001% during the study period. [ncbi.nlm.nih.gov]

Prognosis

Prognosis Without treatment, the visual prognosis is poor with SO leading to bilateral blindness. However, prompt wound closure and efficient medical treatment improve the visual prognosis, which may then be relatively good. [orpha.net]

Immunosuppressive and immunomodulatory medications, when initiated promptly as treatment, are effective in controlling the inflammation and improving the prognosis. [doi.org]

Modern immunosuppressive therapy with systemic steroids and steroid-sparing agents such as cyclosporin A and azathioprine have improved the prognosis. [ncbi.nlm.nih.gov]

Etiology

Etiology The etiology of SO is not completely understood. The inflammation is caused by a cell-mediated immune mechanism and autoimmune inflammatory response directed against ocular self-antigens released after the initial injury. [orpha.net]

Extensive work-up was undertaken to rule out another etiology of the inflammation. [ncbi.nlm.nih.gov]

Etiology The etiology is not well understood but is thought to be secondary to the development of an autoimmune reaction to ocular antigens that are exposed during the traumatic or surgical event. The primary mediators are thought to be T cells. [eyewiki.aao.org]

Epidemiology

A historical review of sympathetic ophthalmia and its epidemiology. Survey Ophthalmology 1989; 34: 1–14. Kilmartin D, Dick A, Forrester J. Prospective surveillance of sympathetic ophthalmia in the UK and Republic of Ireland. [slideplayer.com]

A review of epidemiologic data from the 19th and 20th centuries reveals that acceptable statistical studies have yet to be carried out, but suggests that the disease has always been of extremely low incidence. [ncbi.nlm.nih.gov]

A historical review of sympathetic ophthalmia and its epidemiology. Surv Ophthalmol 1989;34:1–14. 2. Mackenzie W. In: A practical treatise on the diseases of the eye. 3rd ed. London: Longmans; 1840. 3. Makley TA Jr, Azar A. Sympathetic ophthalmia. [synapse.koreamed.org]

Epidemiology Sympathetic ophthalmia is rare, affecting 0.2% to 0.5% of non-surgical eye wounds, and less than 0.1% of surgical penetrating eye wounds. There are no gender or racial differences in incidence of SO. [wikidoc.org]

Summary Epidemiology SO is reported to affect between 1/1000 and 1/1650 individuals with a history of a penetrating eye wound. [orpha.net]

Pathophysiology

The pathophysiology is not clearly understood, but it appears that the disrupted integrity of the inciting eye leads to an autoimmune hypersensitivity reaction against the exposed ocular antigens in the injured eye as well as in the sympathizing eye. [ncbi.nlm.nih.gov]

Fuchs later described the histopathological findings of SO. 1 Although the pathophysiology of the disease is not clearly understood, an autoimmune process incited by the initial penetrating injury or by surgery appears to play a significant role. [doi.org]

Comprehensively updated to reflect everything you need to know regarding retinal diagnosis, treatment, development, structure, function, and pathophysiology, this monumental ophthalmology reference work equips you with expert answers to virtually any [books.google.de]

Prevention

The current anti-inflammatory therapy combined with agents that could prevent oxidative stress may prevent photoreceptor damage in SO and may preserve vision. [ncbi.nlm.nih.gov]

Onset For 65%, within 2-8 weeks; for 90% < 1yr Many delayed presentations as well Presentation Granulomatous panuveitis Any clinical uveitis Inciting Eye Enucleation within 2 weeks of trauma for prevention of SO Enucleation solely for the prevention of [slideplayer.com]

Wednesday 02, Jun 2010 Total blindness preventable with new surgical implant A new device can prove effective for preventing complete loss of vision besides eliminating dependence on systemic immunosuppression for a rare but potentially devastating eye [isteroids.com]

Many surgeons recommend enucleation within two weeks of trauma to prevent this condition. [doi.org]

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