Background: Scleritis and/or uveitis sometimes accompanies patients who suffer from rheumatoid arthritis. However, few studies have reported scleritis and/or uveitis accompanying a fundus elevated lesion, such as an intraocular tumor. In this study, we report a case of rheumatoid uveitis associated with an intraocular elevated lesion. Case presentation: A 66-year-old female visited another eye clinic and was diagnosed as bilateral anterior uveitis, and was prescribed steroid eye drops for treatment. She had previously been diagnosed as rheumatoid arthritis at the age of 30 years. Due to vitreous opacity that appeared in her right eye, we increased the instillation of steroid eye drops and the amount of oral prednisolone. Although the inflammation had improved, anterior uveitis relapsed, and an intraocular whitish elevated lesion resembling an intraocular tumor at the superior nasal retina appeared. We speculated this lesion to be a granuloma complicated with rheumatoid arthritis. Thus, we increased the amount of prednisolone administration, and the lesion began to shrink and ultimately fully disappeared. Conclusions: We strongly believe that our case’s lesion was a subretinal granuloma related with rheumatoid arthritis, as it disappeared by increased corticosteroid treatment. Our findings show that we should consider rheumatoid arthritis in a differential diagnosis of such types of fundus elevated lesions. Keywords: Scleritis, Uveitis, Rheumatoid arthritis, Granuloma, Intraocular tumor Background cases. Most cases of scleritis follow good clinical courses, Rheumatoid arthritis is a collagen disease, and is one of but some cases are refractory, which cannot be cured des- the autoimmune disorders characterized by persistent pite the administration of immunosuppressants. More- synovitis, systemic inflammation, and autoantibodies . over, there are some cases that are even difficult to Ophthalmologists sometimes examine patients suffering diagnose as scleritis. For example, some studies have re- from rheumatoid arthritis combined with ocular inflam- ported cases of posterior scleritis which were diagnosed as mation, such as keratoconjunctivitis sicca, episcleritis, an intraocular tumor . However, a few studies have re- scleritis, or uveitis as an extra-articular disease [1, 2]. ported an elevated-lesion-like intraocular tumor that de- It is thought that scleritis is caused by the immune-com- veloped following scleritis and/or uveitis. In this present plex deposition, as it reportedly has been found in tissues study, we report a rare case of uveitis-associated rheuma- from vasculitis in necrotizing scleritis-associated collagen toid arthritis in which an intraocular elevated lesion oc- disease, such as rheumatoid arthritis . Thus, immuno- curred, although the uveitis had once subsided after suppressive therapies including corticosteroids are the pri- steroid therapy. mary therapeutic procedures used to treat such scleritis Case presentation * Correspondence: email@example.com The present case involved a 66-year-old female who be- Department of Ophthalmology, Osaka Medical College, 2-7 Daigaku-machi, came aware of decreased vision in her left eye. She had Takatsuki City, Osaka 569-8686, Japan Full list of author information is available at the end of the article previously visited another eye clinic, and was diagnosed © The Author(s). 2018 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. 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. Kobayashi et al. BMC Ophthalmology (2018) 18:129 Page 2 of 5 as bilateral anterior scleritis and prescribed steroid eye and intraocular pressure was 17 mmHg OD and drops for treatment. Although the inflammation had 15 mmHg OS. Slit-lamp examination revealed 1+ cells subsided and the scleral redness had disappeared, vitre- in both anterior chambers, and fine keratic precipitates ous opacity increased in her right eye around August on both corneas. The superior sclera in both eyes 2014. Thus, she was diagnosed as uveitis and referred to showed redness and thinning of tissues (Fig. 1a, b). the Department of Ophthalmology at Osaka Medical Moreover, the iris in her left eye was found to be College in September 2014 for examination. adhered to the implanted IOL (Fig. 1c, d). There was no The patient had previously been diagnosed with remarkable fundus abnormality except for moderate rheumatoid arthritis when she was 30 years of age, and vitreous opacity in her right eye (Fig. 1e). Blood tests re- she had undergone a 5 mg-per-day administration of vealed negative results for tuberculosis and syphilis. prednisolone as a maintenance dose and long-term treat- In regard to the follow-up treatment course, we ini- ment with immunosuppressants such as tacrolimus and tially increased the frequency of the instillation of steroid anti-tumor necrosis factor (anti-TNF) drugs such as eta- eye drops and added immunosuppressive eye drops. nercept by the Department of Internal Medicine at our However, those drugs were ineffective, and the vitreous university hospital. Her past medical history included pha- opacity gradually increased. Thus, we increased the coemulsification and aspiration with bilateral intraocular amount of oral prednisolone to 20 mg per day in lens (IOL) implantation surgery in January 2014. It should November 2014. Subsequently, the eye redness and vit- be noted that there was no significant family history. reous opacity gradually disappeared within approxi- Upon initial ocular examination, her visual acuity (VA) mately 2 weeks, her VA slowly improved, and there was was 20/50 × S-0.25D = C-2.00D Ax90° in the right eye no remarkable fundus abnormality in the right eye at and 20/25 × S-0.50D = C-1.25DAx100° in the left eye, each examination. However, uveitis accompanied with Fig. 1 Slit-lamp and fundus photographs obtained at the initial examination of the 66-year-old female patient. a Superior sclera in her right eye. b Superior sclera in her left eye. c Frontal view of her right eye. d Frontal view of her left eye. e Vitreous opacity in her right eye. The superior sclera in both eyes showed redness and thinning of tissues (arrows). The iris in her left eye was found to be adhered to the implanted IOL (arrows) Kobayashi et al. BMC Ophthalmology (2018) 18:129 Page 3 of 5 Fig. 2 Slit-lamp photographs obtained when scleritis relapse, accompanied with an intraocular elevated lesion. a Superior sclera in her right eye, showing redness and a relatively large nodule (arrows). b Superior sclera in her left eye, showing the thinning of tissues (arrows) moderate ocular pain relapsed (Fig. 2a, b), and fundo- 1-week after, and fully disappeared 4-weeks after, initiat- scopic examination revealed an intraocular elevated ing the increased administration (Fig. 5). Her VA im- whitish lesion at the superior nasal retina of her right proved to 20/20, and there has been no recurrence of eye in November 2015 (Fig. 3a, b). We did not observe ocular inflammation up to the present time. any restricted motility accompanied with eye movement. B-scan ultrasonography was also performed, and re- Discussion vealed that the sclera was thickened and that the lesion Rheumatoid arthritis is known to be a representative dis- seemed to have high internal reflectivity (Fig. 4). order accompanying scleritis and/or uveitis. In Japan, Although the patient underwent a magnetic resonance rheumatoid arthritis occupies the first position of eti- imaging (MRI) scan for a differential diagnosis, it was ology of scleritis, except idiopathic . Scleritis is cate- difficult to distinguish whether the lesion was a granu- gorized as episcleritis, anterior scleritis, or posterior loma or a tumor. Ultrasound biomicroscopy (UBM) and scleritis, depending on location at onset. In addition, an- high frequency B scan might have been useful to distin- terior scleritis is also categorized as nodular, diffuse, and guish between a tumor and granuloma, however, those necrotizing scleritis, depending on the pathogenesis . examinations were not available at that time. In cases of rheumatoid arthritis, anterior scleritis is more Hence, we recommended to the patient that she frequent than posterior scleritis, and inflammation is should undergo fluorescein and indocyanine angiog- likely to occur in the superior sclera [7, 8]. raphy examination for differential diagnosis, however, In addition, when a case of scleritis and/or uveitis has the patient wished to receive treatment without under- possibly been caused by systemic disorders such as colla- going those examinations. Both the value of C-reactive gen disease or infectious disease, treatment of those sys- protein (0.60 mg/dL) and the blood sedimentation rate temic disorders is also essential. Even when the systemic (32 mm per hour) were increasing. In addition, the value disorders are relatively stable, ocular inflammation may of matrix metalloproteinase-3 (135.0 ng/mL), an indica- occur, such as in our present case, and the treatment of tor of the activity of rheumatoid arthritis, was also such patients must be conducted in close cooperation increasing. After consultation with her rheumatologist, with internal physicians or rheumatologists. In this we increased the administration amount of prednisolone study, we wish to emphasize that it is most important to to 30 mg per day. As a result, the lesion began to shrink distinguish whether or not the uveitis is caused by Fig. 3 Fundus photographs obtained when scleritis relapsed, accompanied with an intraocular elevated lesion. a Intraocular elevated lesion at the superior nasal retina (arrows). b Enlarged photograph of the intraocular elevated lesion (arrows) Kobayashi et al. BMC Ophthalmology (2018) 18:129 Page 4 of 5 negative results for tuberculosis and syphilis. However, it is sometimes difficult to distinguish between the two, and we usually hesitate to increase the amount of cor- ticosteroid administered in such cases. Liu et al. reported that a patient with a lesion similar to the one in our case improved with a nonsteroidal anti-inflammatory drug (NSAID) . Since it is thought that NSAIDs produce fewer side effects than corticosteroids, it might also be better to try NSAID administration in our present case. Secondly, it is vital to distinguish such an elevated lesion from an intraocular tumor, e.g., malignant melan- oma, metastatic choroidal tumor, and malignant lymph- oma, because those may occur with no relation to systemic disorders. However, it is sometimes difficult to distinguish between them. In our present case, we were unable to perform fluorescein and indocyanine angiog- Fig. 4 B-scan ultrasonography showed that the sclera was thickened raphy when the lesion appeared. Sin et al. reported that and that the lesion seemed to have high internal reflectivity (arrows) ocular pain is a useful symptom for differentiating nodu- lar posterior scleritis from other forms of choroidal infectious inflammation. Some studies have reported masses , and our patient also reported ocular pain cases of infectious inflammation, such as tuberculosis or when the lesion appeared. syphilis, caused by nodular infectious uveitis [9, 10]. And thirdly, it is vital to understand that some types Biswas et al. reported a case of tuberculous uveitis of uveitis may occur with a giant elevated lesion, as associated with rheumatoid arthritis that resulted in Sridharan et al. reported in a 53-year-old female case of enucleation of the eye . It is now possible for the life posterior scleritis . In that study, the lesion was ini- prognosis of the patients who suffer from rheumatoid tially thought to be an amelanotic melanoma. However, arthritis to improve thanks to medical advancements, so after various further examinations, she was diagnosed as the opportunities for ophthalmologists to examine such posterior scleritis with a lesion mimicking malignant patients who have taken immunosuppressants for a few melanoma. She was treated with prednisolone, and the decades will increase in the future. lesion completely regressed. When treating scleritis and /or uveitis accompanying such an elevated lesion, it is vital to first distinguish Conclusion non-infectious scleritis/or uveitis from infectious dis- In this present case, we strongly believe that the elevated eases. In the present case, blood examinations revealed lesion was granuloma caused by recurrence of uveitis. Our findings show that we should consider rheumatoid arthritis in the differential diagnosis of such types of fun- dus elevated lesions. Abbreviations IOL: Intraocular lens; NSAID: Nonsteroidal anti-inflammatory drug; TNF: Tumor necrosis factor; VA: Visual acuity Acknowledgements The authors wish to thank John Bush for editing the manuscript. Availability of data and materials The datasets during the current study are available from the corresponding author on reasonable request. Authors’ contributions TKo and TI drafted this manuscript, collected the data, and reviewed the literature. TKi reviewed the literature. NT, RT, HS, TO, ST, KM, TO, and SM interpreted the data and critically reviewed the manuscript. TKo, TKi, and TI critically reviewed the final version of the manuscript. All authors have read and approved the final manuscript. Ethics approval and consent to participate Fig. 5 Fundus photograph obtained after disappearance of the This case study was approved by the Ethics Committee of the Osaka Medical intraocular elevated lesion (arrows) College. Kobayashi et al. BMC Ophthalmology (2018) 18:129 Page 5 of 5 Consent for publication Written informed consent for publication was obtained from the patient. Competing interests The authors declare that they have no competing interests. Publisher’sNote Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Author details Department of Ophthalmology, Osaka Medical College, 2-7 Daigaku-machi, Takatsuki City, Osaka 569-8686, Japan. Tada Eye Clinic, Ikeda City, Japan. Department of Internal Medicine, Osaka Medical College, Takatsuki City, Japan. Received: 26 January 2018 Accepted: 23 May 2018 References 1. Scott DL, Wolfe F, Huizinga TW. Rheumatoid arthritis. Lancet. 2010; 376(9746):1094–108. 2. Artifoni M, Rothschild PR, Brézin A, Guillevin L, Puéchal X. Ocular inflammatory diseases associated with rheumatoid arthritis. Nat Rev Rheumatol. 2014;10(2):108–16. 3. Fong LP, Sainz de la Maza M, Rice BA, Kupferman AE, Foster CS. Immunopathology of scleritis. Ophthalmology. 1991;98(4):472–9. 4. Finger PT, Perry HD, Packer S, Erdey RA, Weisman GD, Sibony PA. Posterior scleritis as an intraocular tumour. Br J Ophthalmol. 1990;74(2):121–2. 5. Keino H, Watanabe T, Taki W, Nakashima C, Okada AA. Clinical features and visual outcomes of Japanese patients with scleritis. Br J Ophthalmol. 2010; 94(11):1459–63. 6. Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol. 1976;60(3): 163–91. 7. Murray PI, Rauz S. The eye and inflammatory rheumatic diseases: the eye and rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis. Best Pract Res Clin Rheumatol. 2016;30(5):802–25. 8. Jayson MI, Jones DE. Scleritis and rheumatoid arthritis. Ann Rheum Dis. 1971;30(4):343–7. 9. Biswas J, Aparna AC, Annamalai R, Vaijayanthi K, Bagyalakshmi R. Tuberculous scleritis in a patient with rheumatoid arthritis. Ocul Immunol Inflamm. 2012;20(1):49–52. 10. Shaikh SI, Biswas J, Rishi P. Nodular syphilitic scleritis masquerading as an ocular tumor. J Ophthalmic Inflamm Infect. 2015;5:8. https://doi.org/10.1186/ s12348-015-0040-5. 11. Liu AT, Luk FO, Chan CK. A case of giant nodular posterior scleritis mimicking choroidal malignancy. Indian J Ophthalmol. 2015;63(12):919–21. 12. Sin PY, Liu DT, Young AL. Nodular posterior scleritis mimicking choroidal tumor in a patient with systemic lupus erythematous. a case report and literature review. Asia Pac J Ophthalmol (Phila). 2016;5(5):324–9. 13. Sridharan S, Juneja R, Hussain A, Biswas J. Giant nodular posterior scleritis mimicking choroidal tumor. Retin Cases Brief Rep. 2007;1(2):65–7.
– Springer Journals
Published: May 30, 2018