Sympathetic Ophthalmia – a Contribution to Immunology, Clinic and Current Imaging

Abstract

Background Sympathetic ophthalmia (SO) is a rare inflammation of an operated or injured eye that spreads to the fellow eye. It is typically a bilateral granulomatous panuveitis. The traumatized eye is referred to as inciting eye and the fellow eye as sympathizing eye. The pathophysiology of the disease is not entirely understood, but there is strong evidence of an autoimmune genesis.

Patients/Material and Methods A selective literature search on epidemiology, immunology, clinical features and risk factors of SO was carried out. In addition, our own experience using multimodal imaging for this clinical entity was introduced.

Results In the literature, the incidence after traumatic eye injuries is 0.1 – 3% and approximately 0.01% after intraocular surgery. Among the iatrogenic causes, vitreoretinal surgery has the highest rate of SO, presumably due to disruption of the blood-retinal barrier and involvement of retinal and choroidal tissue, which are susceptible to anterior traction, phthisis and chronic inflammation. In 90% of patients, the disease develops within a year following the eliciting event and is associated with a potentially bilateral risk of blindness. Typical symptoms include bilateral visual impairment with photophobia, dull pain and photopsia. The spectrum of clinical manifestations ranges from granulomatous anterior uveitis and vitritis, to choroiditis, serous retinal detachment and Dalen-Fuchs nodules in the context of posterior involvement. The diagnosis of SO is generally based on clinical presentation and is supported by imaging methods. These primarily comprise fluorescein and indocyanine green angiography, which are increasingly being supplemented by non-invasive methods such as optical coherence tomography. They can provide important information for assessment of severity, differential diagnosis as well as for disease monitoring. The differential diagnosis includes i. a. Vogt-Koyanagi-Harada syndrome, ocular sarcoidosis and the rare phacoanaphylactic endophthalmitis. Immediate systemic high-dose steroid therapy is used as initial treatment. The course of the disease is often relapsing to chronic progressive. Immunomodulators such as ciclosporine A, azathioprine, cyclophosphamide, mycophenolate mofetil, and biologics are increasingly being used and contribute to the significantly better prognosis of the disease. Generally, SO can be triggered by any kind of intraocular intervention.

Conclusion SO remains a threatening clinical diagnosis that poses diagnostic and therapeutic challenges. It can be triggered post-traumatic, but also any intraocular surgery. This should be taken into account when assessing the indication for intraocular eye surgery, especially in eyes with reduced visual outcome.

Key words

autoimmune disease - Dalen-Fuchs nodules - granulomatous uveitis - intraocular Inflammation - sympathetic ophthalmia

Publikationsverlauf

Eingereicht: 22. Juni 2020

Angenommen: 22. August 2020

Artikel online veröffentlicht:
23. September 2020

• References/Literatur

• 1 Albert DM, Diaz-Rohena R. A historical review of sympathetic ophthalmia and its epidemiology. Surv Ophthalmol 1989; 34: 1-14 doi:10.1016/0039-6257(89)90125-2
• 2 MacKenzie W. A practical Treatise on Diseases of the Eye. London: Longman; 1840. Available at (Accessed 05.05.2020): https://books.google.so/books?id=rhI1AQAAMAAJ&source=gbs_book_other_versions_r&cad=4
• 3 Fuchs E. Über sympathisierende Entzündung (nebst Bemerkungen über seröse traumatische Iritis). Albrecht Von Graefes Arch Ophthalmol 1905; 61: 365-456
• 4 Hakin KN, Pearson RV, Lightman SL. Sympathetic ophthalmia: visual results with modern immunosuppressive therapy. Eye (Lond) 1992; 6: 453-455 doi:10.1038/eye.1992.95
• 5 Jennings T, Tessler HH. Twenty cases of sympathetic ophthalmia. Br J Ophthalmol 1989; 73: 140-145 doi:10.1136/bjo.73.2.140
• 6 Chan CC, Roberge RG, Whitcup SM. et al. 32 cases of sympathetic ophthalmia. A retrospective study at the National Eye Institute, Bethesda, Md., from 1982 to 1992. Arch Ophthalmol 1995; 113: 597-600 doi:10.1001/archopht.1995.01100050065032
• 7 Kilmartin DJ, Dick AD, Forrester JV. Prospective surveillance of sympathetic ophthalmia in the UK and republic of Ireland. Br J Ophthalmol 2000; 84: 259-263 doi:10.1136/bjo.84.3.259
• 8 Su DH, Chee SP. Sympathetic ophthalmia in Singapore: new trends in an old disease. Graefes Arch Clin Exp Ophthalmol 2006; 244: 243-247 doi:10.1007/s00417-005-0009-4
• 9 Castiblanco CP, Adelman RA. Sympathetic ophthalmia. Graefes Arch Clin Exp Ophthalmol 2009; 247: 289-302 doi:10.1007/s00417-008-0939-8
• 10 Payal AR, Foster CS. Long-term drug-free remission and visual outcomes in sympathetic ophthalmia. Ocul Immunol Inflamm 2017; 25: 190-195 doi:10.3109/09273948.2015.1092557
• 11 Guzman-Salas PJ, Serna-Ojeda JC, Guinto-Arcos EB. et al. Characteristics of sympathetic ophthalmia in a single international center. Open Ophthalmol J 2016; 10: 154-159 doi:10.2174/1874364101610010154
• 12 Yang P, Liu S, Zhong Z. et al. Comparison of Clinical Features and Visual Outcome between Sympathetic Ophthalmia and Vogt-Koyanagi-Harada Disease in Chinese Patients. Ophthalmology 2019; 126: 1297-1305 doi:10.1016/j.ophtha.2019.03.049
• 13 Tan CL, Seen S, Majumder PD. et al. Analysis of 130 Cases of Sympathetic Ophthalmia – A retrospective multicenter Case Series. Ocul Immunol Inflamm 2019; 27: 1259-1266 doi:10.1080/09273948.2018.1517894
• 14 Kilmartin DJ, Dick AD, Forrester JV. Commentary: sympathetic ophthalmia risk following vitrectomy: should we counsel patients?. Br J Ophthalmol 2000; 84: 448-449 doi:10.1136/bjo.84.5.448
• 15 Schalken JJ, Winkens HJ, Van Vugt AH. et al. Rhodopsin-induced experimental autoimmune uveoretinitis in monkeys. Br J Ophthalmol 1989; 73: 168-172 doi:10.1136/bjo.73.3.168
• 16 Gery I, Wiggert B, Redmond TM. et al. Uveoretinitis and pinealitis induced by immunization with interphotoreceptor retinoid-binding protein. Invest Ophthalmol Vis Sci 1986; 27: 1296-1300
• 17 Gery I, Chanaud NP, Anglade E. Recoverin is highly uveitogenic in Lewis rats. Invest Ophthalmol Vis Sci 1994; 35: 3342-3345
• 18 Chaithanyaa N, Devireddy SK, Kishore Kumar RV. Sympathetic ophthalmia: A review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol 2012; 113: 172-176 doi:10.1016/j.tripleo.2011.01.036
• 19 Davis JL, Mittal KK, Freidlin V. et al. HLA associations and ancestry in Vogt-Koyanagi-Harada disease and sympathetic ophthalmia. Ophthalmology 1990; 97: 1137-1142 doi:10.1016/s0161-6420(90)32446-6
• 20 Shindo Y, Ohno S, Usui M. et al. Immunogenetic study of sympathetic ophthalmia. Tissue Antigens 1997; 49: 111-115 doi:10.1111/j.1399-0039.1997.tb02723.x
• 21 Goto H, Rao NA. Sympathetic ophthalmia and Vogt-Koyanagi-Haradaʼs syndrome. Int Ophthalmol Clin 1990; 30: 279-285 doi:10.1097/00004397-199030040-00014
• 22 Zaharia MA, Lamarche J, Laurin M. Sympathetic uveitis 66 years after injury. Can J Ophthalmol 1984; 19: 240-243
• 23 Mahajan S, Invernizzi A, Agrawal R. et al. Multimodal Imaging in Sympathetic Ophthalmia. Ocul Immunol Inflamm 2017; 25: 152-159 doi:10.1080/09273948.2016.1255339
• 24 Burkholder BM, Dunn JP. Multiple serous retinal detachments seen on wide-field imaging in a patient with sympathetic ophthalmia. JAMA Ophthalmol 2014; 132: 1220 doi:10.1001/jamaophthalmol.2014.52
• 25 Gupta V, Gupta A, Dogra MR. Posterior sympathetic ophthalmia: a single centre long-term study of 40 patients. Eye (Lond) 2008; 22: 1459-1464 doi:10.1038/sj.eye.6702927
• 26 Bernasconi O, Auer C, Zografos L. et al. Indocyanine green angiographic findings in sympathetic ophthalmia. Graefes Arch Clin Exp Ophthalmol 1998; 236: 635-638 doi:10.1007/s004170050134
• 27 Saatci AO, Paşa E, Söylev MF. et al. Sympathetic ophthalmia and indocyanine green angiography. Arch Ophthalmol 2004; 122: 1568-1569 doi:10.1001/archopht.122.10.1568
• 28 Casella AM, Farah ME, Martins MC. et al. Sympathetic ophthalmia – histopathological correlation with fluorescein and indocyanine green and indocyanine green angiography: case report. Arq Bras Oftalmol 2008; 71: 886-889 doi:10.1590/s0004-27492008000600025
• 29 Fleischman D, Say EA, Wright JD. et al. Multimodality diagnostic imaging in a case of sympathetic ophthalmia. Ocul Immunol Inflamm 2012; 20: 300-302 doi:10.3109/09273948.2012.682637
• 30 Mrejen S, Spaide RF. Optical coherence tomography: imaging of the choroid and beyond. Surv Ophthalmol 2013; 58: 387-429 doi:10.1016/j.survophthal.2012.12.001
• 31 Correnti AJ, Read RW, Kimble JA. et al. Imaging of Dalen-Fuchs nodules in a likely case of sympathetic ophthalmia by fluorescein angiography and OCT. Ophthalmic Surg Lasers Imaging 2010;
• 32 Jap A, Chee SP. The role of enhanced depth imaging optical coherence tomography in chronic Vogt-Koyanagi-Harada disease. Br J Ophthalmol 2017; 101: 186-189 doi:10.1136/bjophthalmol-2015-308091
• 33 Khatri A, Timalsena S, Khatri BK. et al. A rare entity: Sympathetic ophthalmia presumably after blunt trauma to the phthisical eye and optical coherence tomography angiography metrics to monitor response to treatment. Clin Case Rep 2019; 8: 149-154 doi:10.1002/ccr3.2597
• 34 Aggarwal K, Agarwal A, Mahajan S. et al. OCTA Study Group. The role of optical coherence tomography angiography in the diagnosis and management of acute Vogt-Koyanagi-Harada disease. Ocul Immunol Inflamm 2018; 26: 142-153 doi:10.1080/09273948.2016.1195001
• 35 Palexas GN, Sussman G, Welsh NH. Ocular and systemic determination of IL-1 beta and tumour necrosis factor in a patient with ocular inflammation. Scand J Immunol Suppl 1992; 11: 173-175 doi:10.1111/j.1365-3083.1992.tb01645.x
• 36 Santos LM, Marcos MC, Gallardo JM. et al. Aqueous humor and serum tumor necrosis factor-alpha in clinical uveitis. Ophthalmic Res 2001; 33: 251-255 doi:10.1159/000055677
• 37 Kase S, Shinohara T, Kase M. Adalimumab for pediatric sympathetic ophthalmia. JAMA Ophthalmol 2014; 132: 1022-1024 doi:10.1001/jamaophthalmol.2014.426
• 38 Soheilian M, Jabbarpourbonyadi J, Soheilian R. Bilateral uveitis after phakic intraocular lens implantation and management with adalimumab. J Cataract Refract Surg 2012; 38: 1094-1096 doi:10.1016/j.jcrs.2012.02.026
• 39 Hiyama T, Harada Y, Kiuchi Y. Effective treatment of refractory sympathetic ophthalmia with glaucoma using adalimumab. Am J Ophthalmol Case Rep 2019; 14: 1-4 doi:10.1016/j.ajoc.2019.01.009
• 40 Suhler EB, Adan A, Brezin AP. et al. Safety and efficacy of adalimumab in patients with noninfectious uveitis in an ongoing open-label study: VISUAL III. Ophthalmology 2018; 125: 1075-1087 doi:10.1016/j.ophtha.2017.12.039
• 41 Chan RV, Seiff BD, Lincoff HA. et al. Rapid recovery of sympathetic ophthalmia with treatment augmented by intravitreal steroids. Retina 2006; 26: 243-247 doi:10.1097/00006982-200602000-00029
• 42 Jonas JB, Rensch F. Intravitreal steroid slow-release device replacing repeated intravitreal triamcinolone injections for sympathetic ophthalmia. Eur J Ophthalmol 2008; 18: 834-836 doi:10.1177/112067210801800531
• 43 Mahajan VB, Gehrs KM, Goldstein DA. et al. Management of sympathetic ophthalmia with the fluocinolone acetonide implant. Ophthalmology 2009; 116: 552-557 doi:10.1016/j.ophtha.2008.10.024
• 44 Bilyk JR. Enucleation, evisceration, and sympathetic ophthalmia. Curr Opin Ophthalmol 2000; 11: 372-386 doi:10.1097/00055735-200010000-00015
• 45 Manandhar A. Sympathetic ophthalmia: Enucleation or evisceration?. Nepal J Ophthalmol 2011; 3: 181-187 doi:10.3126/nepjoph.v3i2.5274
• 46 Lubin JR, Albert DM, Weinstein M. Sixty-five years of sympathetic ophthalmia. A clinicopathologic review of 105 cases (1913–1978). Ophthalmology 1980; 87: 109-121 doi:10.1016/s0161-6420(80)35270-6