Coexistence of open-angle glaucoma and sarcoidosis-associated optic neuropathy

BMC Ophthalmology Jung et al. BMC Ophthalmology (2023) 23:375 https://doi.org/10.1186/s12886-023-03104-y Open Access CASE REPORT Coexistence of open-angle glaucoma and sarcoidosis-associated optic neuropathy Eun Hye Jung1†, Woonghee Kim1†, Ra Gyoung Yoon2 and Ko Eun Kim3* Abstract Background In cases with advanced glaucomatous disc changes, further changes associated with other optic neuropathies cannot be easily identified. We present a case of preexisting open-angle glaucoma and concurrent involvement of sarcoidosis-associated optic neuropathy. Case presentation A 53-year-old man presented with gradual visual loss in his left eye, which began 1 year ago and accelerated 3 months ago. The best-corrected visual acuity in the right eye was 20/20 and counting fingers in the left. Intraocular pressures (IOP) were 12 mmHg in the right eye and 34 mmHg in the left. We diagnosed him with advanced open-angle glaucoma in the left eye based on the advanced glaucomatous cupping of the left optic disc. The IOP in the left eye dropped to 10 mmHg and was well controlled with antiglaucomatous medication; however, his left optic disc developed pallor 3 months after the treatment. The patient was revealed to be diagnosed with sarcoidosis a month ago and had been treated with systemic corticosteroids thereafter by a pulmonologist. Orbital magnetic resonance imaging revealed sarcoidosis-associated optic neuropathy in the left eye. Subsequently, optic neuropathy occurred in his right eye. Conclusions In eyes with advanced glaucomatous disc change, detecting the coexistence of other optic neuropathies can be difficult. This report highlights the importance of careful ophthalmic examinations and investigation for etiologies of other optic neuropathies if non-glaucomatous changes are suspected even in eyes with advanced glaucoma. Keywords Sarcoidosis, Optic neuropathy, Neurosarcoidosis, Glaucoma † Eun Hye Jung and Woonghee Kim are co-first authors who equally contributed to this work. *Correspondence: Ko Eun Kim 1 Department of Ophthalmology, Nowon Eulji Medical Center, Eulji University, Seoul, Korea 2 Department of Radiology, Nowon Eulji Medical Center, Eulji University, Seoul, Korea 3 Department of Ophthalmology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, 05505 Seoul, Korea Background Sarcoidosis is a systemic granulomatous inflammatory disease characterized by the formation of noncaseating granulomas in the affected organs [1, 2]. Although sarcoidosis predominantly affects the pulmonary system, it can affect any organ [3, 4]. Ophthalmic complications occur in 13–79% of sarcoidosis; the most common ocular manifestation is uveitis [2, 4], and optic neuropathy is the most common neuro-ophthalmic manifestations [2, 5, 6]. Involvement of optic nerve in sarcoidosis can be explained by intrinsic granulomatous infiltration, extrinsic compression, compression or infiltration of the chiasm, or raised intracranial pressure [7]. © The Author(s) 2023. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Jung et al. BMC Ophthalmology (2023) 23:375 Ocular or neurologic involvement of sarcoidosis should be suspected in patients with systemic sarcoidosis who develop visual loss [2, 8]. However, identifying sarcoidosis-related optic nerve involvement in eyes with preexisting advanced glaucomatous disc changes would be difficult. Herein, we report a case of visual loss caused by preexisting advanced glaucoma and the concurrent involvement of sarcoidosis-associated optic neuropathy. Case presentation A 53-year-old man presented with gradually deteriorating vision in his left eye that began one year prior and accelerated over the last three months. At the time vision deterioration three months ago, the patient complained of periorbital pressure on his left upper eyelid. His bestcorrected visual acuity (BCVA) in the right eye was 20/20, and counting fingers in the left. Intraocular pressures (IOP) measured by Goldmann applanation tonometry in the right and left eyes were 12 and 34 mmHg, respectively. Anterior segment examinations showed no abnormalities other than mild nuclear cataract in both eyes, and the gonioscopic examination using four-mirror gonioscopy lens showed an open angle with clearly visible angular structures. The right optic disc had an intact neuroretinal rim (Fig. 1A and B); however, the left optic disc had advanced glaucomatous cupping with a cupto-disc ratio of 0.9 (Fig. 1C and 1D). Humphrey 30−2 visual field (HVF) showed normal results in the right eye (Fig. 1E) but complete field loss with a mean deviation of -32.35 dB in the left eye (Fig. 1F). Spectral-domain optical coherence tomography demonstrated an increased depth of lamina cribrosa in the left eye compared to that in the right eye (Fig. 2A and B), normal retinal nerve fiber layer thickness (RNFL) in the right eye (Fig. 2C) and diffuse RNFL loss in the left eye (Fig. 2D). There was a relative afferent pupillary defect (RAPD) in the left eye, without ocular pain or pain on extraocular movement. Pupillary light reflex in the right eye was normal. The patient was diagnosed with advanced open-angle glaucoma in the left eye and was treated with a combination of 2% dorzolamide and 0.5% timolol, 0.2% brimonidine, and 0.005% latanoprost, which lowered the IOP to 10 mmHg. Three months later, the IOP in the left eye was 14 mmHg. No abnormality or change was found in the anterior segment; however, pronounced optic disc pallor and thinning of the whole retinal vessels were observed in his left eye (Fig. 3A). We reexamined the patient, suspecting other concurrent optic neuropathies, and discovered that the patient had been referred to a pulmonologist due to cough and dyspnea. Approximately a month previously, he was diagnosed with sarcoidosis because he had bilateral hilar lymphadenopathy and non-caseating granulomas in the supraclavicular lymph node on radiological and histological examin (...truncated)