Corticosteroid-Resistant Sarcoid Choroidal Granuloma Presenting With Optic Disc Edema

A54-year-old man with untreated psoriatic arthritis was referred for evaluation of optic disc edema in his right eye. He was found to have an associated peripapillary choroidal granuloma, verified by enhanced depth imaging optical coherence tomography (EDI-OCT).

Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Caroline Froment, MD, PhD An-Guor Wang, MD Corticosteroid-Resistant Sarcoid Choroidal Granuloma Presenting With Optic Disc Edema Karen R. Armbrust, MD, PhD, Michael S. Lee, MD A 54-year-old man with untreated psoriatic arthritis was referred for evaluation of optic disc edema in his right eye. He was found to have an associated peripapillary choroidal granuloma, verified by enhanced depth imaging optical coherence tomography (EDI-OCT). Identification of a choroidal granuloma should trigger systemic evaluation and, in this case, led to a new diagnosis of sarcoidosis with pulmonary involvement. Serial optical coherence tomography of the optic nerve head and peripapillary choroid allowed for quantitative assessment of treatment response. Although the peripapillary choroidal granuloma was resistant to corticosteroid treatment, the patient went on to have successful ocular and systemic outcomes with adalimumab treatment. This case demonstrates the benefit of steroid-sparing immunosuppression for corticosteroidresistant ocular sarcoidosis. A 54-year-old Caucasian man with a history of untreated psoriatic arthritis was referred to neuro-ophthalmology clinic for evaluation of right optic disc edema. He reported a 2- to 3-month history of mild blurry vision in his right eye. Visual acuity was 20/20 in both eyes, and color vision was intact. There was no relative afferent pupillary defect. Slit-lamp examination was normal. Automated perimetry showed no defect in either eye. Dilated fundus examination and EDI-OCT revealed an isolated peripapillary choroidal granuloma with associated optic disc edema in the right eye (Fig. 1). QuantiFERON-TB Gold and Treponema pallidum antibody tests were negative. Complete blood count, basic metabolic panel including serum calcium, and serum angiotensin-converting enzyme (ACE) were normal. MRI of the brain and orbits was unremarkable. Chest computed tomography showed mediastinal and hilar lymphadenopathy (Fig. 2). Lymph node biopsy revealed non-necrotizing granulomas and mycobacterial and fungal stains were negative for organisms, supporting the diagnosis of sarcoidosis. FIG. 1. Fundus photograph and enhanced depth imaging optical coherence tomography (EDI-OCT) (inset) reveal a peripapillary choroidal granuloma with associated optic disc edema in the right eye. EDI-OCT confirms choroidal location of the granuloma (arrowhead). Department of Ophthalmology and Visual Neurosciences (KRA, MSL), University of Minnesota, Minneapolis, Minnesota; and Department of Ophthalmology (KRA), Veterans Affairs Health Care System, Minneapolis, Minnesota. The authors report no conflicts of interest. Address correspondence to Karen R. Armbrust, MD, PhD, Department of Ophthalmology and Visual Neurosciences, University of Minnesota, 420 Delaware Street Southeast, MMC 493, Minneapolis, MN 55455; E-mail: [e-mail] Armbrust and Lee: J Neuro-Ophthalmol 2022; 42: e335-e337 FIG. 2. Chest computed tomography shows subcarinal (arrow) and hilar (arrowhead) lymphadenopathy. e335 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence FIG. 3. Retinal nerve fiber layer (RNFL) optical coherence tomography provides quantitation of the optic disc edema at presentation (A). The optic disc edema did not improve and in fact gradually worsened by 10 months after presentation (B) despite a 2-month course of oral prednisone followed by a series of 3 periocular triamcinolone injections every other month, with imaging obtained 6 weeks after the third triamcinolone injection. The optic disc edema improved substantially (C) 6 months after the patient started adalimumab, with reduced RNFL thickness compared with initial presentation. The peripapillary choroidal thickening present in panels (A) and (B) has resolved in panel (C). Although the pulmonary lymphadenopathy regressed with oral prednisone (60 mg/day), the fundus examination and EDI-OCT of the right optic disc showed no change with oral prednisone or multiple periocular triamcinolone injections. In fact, retinal nerve fiber layer (RNFL) OCT showed interval worsening of the optic disc edema despite corticosteroid treatment (Fig. 3A, B). Corticosteroids were discontinued. After consultation with the patient’s rheumatologist, the patient started subcutaneous adalimumab to treat both the ocular disease and the sacroiliitis secondary to psoriatic arthritis. Despite the previous lack of response to corticosteroids, there was resolution of the optic disc edema (Fig. 3C) and involution of the choroidal granuloma within 6 months of initiating adalimumab, with only a residual lesion present at 1 year (Fig. 4). In this case, adalimumab benefited both the inflammatory eye disease and the inflammatory joint disease. When optic disc edema is accompanied by a choroidal granuloma, an underlying systemic illness should be suspected. Sarcoidosis, tuberculosis, and toxocariasis are the most common etiologies associated with a choroidal granuloma (1). Masquerade syndromes and other infections such as Bartonella, syphilis, and atypical e336 FIG. 4. Fundus photograph and enhanced depth imaging optical coherence tomography (inset) show regression of the peripapillary choroidal granuloma and resolution of the optic disc edema after 1 year of adalimumab treatment. Armbrust and Lee: J Neuro-Ophthalmol 2022; 42: e335-e337 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence mycobacterium (2) are less common causes. Although cases of idiopathic isolated “choroidal” granuloma have been described in the literature, re-evaluation with EDI-OCT imaging showed that the location of these lesions was scleral, not choroidal (3). Identification of a choroidal granuloma, with location verified by EDIOCT, necessitates a systemic evaluation. In our case, this systemic evaluation led to a new diagnosis of sarcoidosis. We used OCT technology to monitor the treatment response in this minimally symptomatic patient without visual field defects. Serial EDI-OCT scans are helpful for monitoring choroidal granulomas (4,5), and we recommend serial RNFL OCT scans as well to monitor the optic disc edema in cases of peripapillary choroidal granuloma with associated optic disc edema. The OCT findings in this case provided clear evidence for a lack of response to corticosteroids, although sarcoid choroidal granulomas typically regress, at least partially, with corticosteroid treatment (4–6). Although off-label treatment with an antimetabolite such as methotrexate or mycophenolate mofetil is more typical first-line immunosuppression for ocular sarcoidosis (7), here, treatment with a tumor necrosis factor inhibitor was indicated for psoriatic arthritis. Thus, this case provides a reminder that ocular sarcoidosis may be resistant to corticosteroids and require steroid-sparing immunosuppression, and concomitant systemic disease Armbrust and Lee: J Neuro-Ophthalmol 2022; 42: e335-e337 should be considered when choosing an immunosuppressive agent. STATEMENT OF AUTHORSHIP Category 1: a. conception and design: K. R. Armbrust and M. S. Lee; b. acquisition of data: K. R. Armbrust and M. S. Lee; c. analysis and interpretation of data: K. R. Armbrust and M. S. Lee. Category 2: a. drafting the manuscript: K. R. Armbrust; b. revising it for intellectual content: K. R. Armbrust and M. S. Lee. Category 3: a. final approval of the completed manuscript: K. R. Armbrust and M. S. Lee. REFERENCES 1. Nussenblatt RB, Whitcup SM. Uveitis: Fundamentals and Clinical Practice, 4th edition. Maryland Heights, MI: Mosby, 2010. 2. Moorthy RS, Valluri S, Rao NA. Nontuberculous mycobacterial ocular and adnexal infections. Surv Ophthalmol. 2012;57:202–235. 3. Fung AT, Waldstein SM, Gal-Or O, Pellegrini M, Preziosa C, Shields JA, Welch RJ, Dolz-Marco R, Sarraf D, Nagiel A, Lalane R, Jung JJ, Ghazi NG, Ramtohul P, Arnold JJ, Sakurada Y, Choudhry N, Balaratnasingam C, Freund KB, Shields CL. Focal scleral nodule: a new name for solitary idiopathic choroiditis and unifocal helioid choroiditis. Ophthalmology. 2020;127:1567–1577. 4. Modi YS, Epstein A, Bhaleeya S, Harbour JW, Albini T. Multimodal imaging of sarcoid choroidal granulomas. 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