Background: Scleromalacia perforans is a rare ocular manifestation of rheumatoid arthritis which can potentially lead to blindness and is a late consequence in the course of the disease. It is an unusual finding for it to be present in a patient with joint pain without any rheumatologic progression of disease. Case presentation: We describe a rare case of scleromalacia perforans and orbital inflammatory disease in a 40-year- old Pakistani woman with apparently no associated rheumatologic deformity. It is rare in the sense that we usually see scleromalacia perforans with fixed deformities of rheumatoid arthritis in the hands or progressed systemic complications but not as a starting landmark of disease. She presented to us with pronounced eye manifestation which on further inquiry and investigation was found to be associated with rheumatoid arthritis. There was perforation of left globe on presentation and the right one was preserved. She visited various physicians and ophthalmologists and was treated with topical and systemic antibiotics but ended up losing sight in her left eye. Conclusion: We conclude that ocular manifestations, however rare they are, should be foreseen, investigated, and treated in patients with suspected arthritis as the complication is grave and sight threatening. Keywords: Rheumatoid arthritis, Scleromalacia perforans, Peripheral thinning of cornea, Ocular complication, Case report Background She complained of joint pains and stiffness for the past Rheumatoid arthritis (RA) is a systemic disease that can 2 months and she had pain with blurring of vision and affect more than just the joints . It is a disorder of photophobia in her right eye for the past few days. autoimmune origin causing chronic inflammation . A She said that she had severe pain in both eyes with gritty lthough inflammation of the tissue around the joints sensation, around 8/10 on Visual Analog of Pain Scale and inflammatory arthritis are characteristic features (VAPS); she associated this pain with dryness of eyes and of RA, the disease causes inflammation not only in she stated that there had also been pus discharge from her joints but also affects other organs of the body; hence, left eye and progressive loss of vision. She visited various it is called rheumatoid disease . physicians in her local area and she was kept on antibi- Scleromalacia perforans is a rare form of anterior otics for 7 months but she had complete loss of vision by scleritis which readily presents as a blackish blue hue the time she presented to our ward. visible through a thin sclera . No significant redness She also complained of dry mouth, oral ulcers, and or pain is present but it is represented by progressive there was a history of stillbirth in eighth month of gesta- thinning of the sclera; it is a rare form of necrotizing an- tional amenorrhea 1 year back. She also complained of terior scleritis . pain in all small joints of her hands associated with morning stiffness of 30–45 minutes. She had severe pain around 7/10 on VAPS associated with joint swelling. On Case presentation examination she was vitally stable and anemic. The A 40 year-old married Pakistani woman came to our out- examination revealed acute synovitis in the joints of her patient department (OPD) on 19 July 2017 with pain and hands and feet. dryness in her left eye for 7 months, she also complained On examination she had no light perception in her left of progressive loss of vision in her left eye for 6 months. eye and her right eye was 6/6. A slit lamp examination of the cornea revealed a melted left cornea with red * Correspondence: firstname.lastname@example.org; email@example.com Department of Medicine, Jinnah Medical and Dental College, 22-23 eye and her right eye had inferior peripheral ulceration Shaheed-e-Millat Road, Karachi, Pakistan (Figs. 1 and 2). There was scleral thinning in lower Full list of author information is available at the end of the article © 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. Ghauri et al. Journal of Medical Case Reports (2018) 12:155 Page 2 of 4 Discussion We reviewed more than 25 articles for discussion. None of the articles showed that such pronounced eye compli- cations can precede joint disease. Lamba et al. discussed the different manifestations with percentages and em- phasized the need for early detection and treatment . RA is a systemic disease that can affect the eyes. The ophthalmic manifestations of RA include keratoconjunc- tivitis sicca, episcleritis, scleritis, peripheral ulcerative keratitis, and retinal vasculitis which are described in the literature . Wu et al. discussed the presence of scleromalacia perfor- ans in a patient with a history of joint pains for approxi- mately 10 years . This is in contrast to our case who developed ocular complications preceding joint disease. Watson stated that scleritis is a severe inflammatory Fig. 1 Left eye shows matted lashes, conjunctival injection, and condition that is characterized by edema and inflamma- opaque cornea with thinning at inferior half tory cell infiltration of the sclera often presenting as pain and redness . It has a peak incidence in the fifth dec- ade but is most common in the fourth to sixth decades temporal quadrant with visible uveal tissue. There was . McCluskey and Wakefield stated that in 40% of the peripheral corneal thinning in lower temporal quadrant. cases it is bilateral . As in our case, the patient lost On investigation she was anemic with hemoglobin of her eye quickly; the goal of treatment in a patient with 10.2 mg/dl and a mean corpuscular volume (MCV) of scleritis is to identify a potentially life-threatening 66. She had an erythrocyte sedimentation rate (ESR) of systemic etiology, control ocular and systemic inflam- 15. She was found to have a strongly positive RA factor mation, make the patient comfortable, and prevent a value of 191.9 IU/ml with a positive C-reactive protein scleral melt. (CRP) and anti-citrullinated cyclic polypeptide (CCP) of Watson and Hayreh divided scleritis into anterior and 32 IU/ml. posterior types based on the anatomic distribution of She was started on methylprednisolone at a dose of disease . Anterior scleritis is the most common type 1 g intravenously administered once a day in her right ; it includes necrotizing without inflammation (scler- eye for 3 days, methotrexate 10 mg/week, analgesics, omalacia perforans) and necrotizing with inflammation, proton pump inhibitor, and folic acid supplementation with diffuse and nodular as its types . Zlatanović et to which she responded really well. The dose of steroid al. stated that 27.2% of patients with RA presented with was tapered to 5 mg after 3 months and hydroxychloro- ocular complications . quine 400 mg/day was added. She was asked to attend In eyes with scleritis, a potentially blinding disease, the follow-up. inflammatory process may extend to adjacent structures . Okhravi et al. stated that anterior scleritis can be associated with multiple ocular manifestations [11, 13– 15]. RA is associated with many extra-articular manifes- tations, which include ocular diseases. The ocular manifestations must be addressed because of the high potential for permanent damage and blind- ness if they are allowed to run their course without intervention. Common complications of anterior scleritis often comprise peripheral corneal thinning, stromal keratitis, and peripheral ulcerative keratitis . Poster- ior scleritis complications comprise exudative retinal de- tachment, optic disk edema, cystoid macular edema, and choroidal folds [11, 14]. In our case, our patient was treated initially as an infective etiology and lost one eye quickly during the course of the disease. Other common Fig. 2 Right eye shows limbal injection at 3 o’clock to 7 o’clock complications include scleral thinning and globe rupture corneal thinning at inferior half with exposure of uveal tissue with minor trauma . Ghauri et al. Journal of Medical Case Reports (2018) 12:155 Page 3 of 4 Galor and Thorne stated that scleritis may be idio- Conclusions pathic or associated with local or systemic disease. RA is associated with many extra-articular manifestations, Autoimmune conditions are found in approximately which include ocular diseases such as keratoconjunctivitis 40% of patients and infections in approximately 7% sicca, episcleritis, scleritis, peripheral ulcerative keratitis, . Our patient was treated with antibiotics by and retinal vasculitis. some ophthalmologists for 7 months. These concomitant ocular manifestations are of Scleromalacia perforans, a form of autoimmune an- utmost concern and must be addressed because of terior scleritis, is a potential blinding disease which the high potential for permanent damage and blind- appears as a black area of scleral thinning surrounded ness if they are allowed to run their course without by inflammatory tissue . intervention. Collaborative efforts between the oph- Although scleritis may be the initial sign of rheumatoid thalmologists and rheumatologists involved in the disease, it usually presents more than 10 years after the evaluation and treatment of patients with RA are onset of arthritis but this was a contrasting feature in our essential to effectively manage any ocular complica- case because she presented with advanced eye manifest- tions that may arise. ation and no joint deformities. Multiple studies have Abbreviations found that patients with scleritis have more advanced joint CCP: Citrullinated cyclic polypeptide; CRP: C-reactive protein; DMARDS: Disease- disease and more extra-articular manifestations than do modifying antirheumatic drugs; ESR: Erythrocyte sedimentation rate; MCV: Mean patients with RA without scleritis [15, 17–20]. corpuscular volume; OPD: Out-patient department; RA: Rheumatoid arthritis; VAPS: Visual Analog of Pain Scale Some studies showed an association of scleritis with other systemic manifestations and complications. Sub- Acknowledgements cutaneous nodules appeared in 20–30% of patients We would like to thank the Department of Medicine for taking care of the with RA, their presence increased to approximately patient and providing useful information. 50% in patients with scleritis . It is often seen that Authors’ contributions pulmonary disorders are more common in patients All persons who meet authorship criteria are listed as authors, and all authors with RA with scleritis than in patients who do not have certify that they have participated sufficiently in the work to take public scleritis. In addition, cardiac manifestations are more responsibility for this manuscript. Furthermore, each author certifies that this material or similar material has not been and will not be submitted to or common in patients with RA who have a history of published in any other publication before its appearance in the Journal. All scleritis [17, 19, 21, 22]. Exacerbation of scleritis often authors read and approved the final manuscript. occurs during a flare of RA [17, 18, 20, 23]. Fitzgerald discussed the development of osteoporosis Ethics approval and consent to participate The Ethics Review Committee (ERC) of Jinnah Medical and Dental College in a patient with diagnosed RA and her response to a gave ethical review approval dated 15 October 2017. multidisciplinary approach . Reddy et al. described bilateral corneal thinning in a patient with Wegener’s Consent for publication granulomatosis, which is a similar finding to ours as our Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written patient has a bilateral disease . consent is available for review by the Editor-in-Chief of this journal. Patients with scleritis have a higher morbidity and mortality rate [19, 21]. If left untreated with systemic Competing interests medications, 36–45% of patients with scleritis and RA The authors declare that they have no competing interests. will die within 3 years of the onset of scleritis. This compares to a 3-year mortality rate of 18% in patients Publisher’sNote with RA without scleritis. Death is usually secondary Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. to extra-articular vasculitis. Necrotizing scleritis is as- sociated with a higher mortality than the other forms Author details [17, 20, 23]. Department of Medicine, Jinnah Medical and Dental College, 22-23 Shaheed-e-Millat Road, Karachi, Pakistan. Department of Ophthalmology, The most effective treatment of scleritis is aggressive and Jinnah Medical and Dental College, 22-23 Shaheed-e-Millat Road, Karachi, systemic. The use of non-steroidal anti-inflammatory 3 Pakistan. Department of Ophthalmology, Karachi Medical and Dental drugs, corticosteroids, or immunomodulatory drugs is usu- College, Block M North Nazimabad, Karachi, Pakistan. ally necessary in the treatment of scleritis . Kahlenberg Received: 1 January 2018 Accepted: 11 April 2018 and Fox discussed the role of biological disease-modifying antirheumatic drugs (DMARDS) stating that they are a revolution in the treatment of RA . Luwayi and References 1. Rheumatoid arthritis. http://www.mayoclinic.org/diseases-conditions/ Gurbaxani reported two cases of scleromalacia per- rheumatoid-arthritis/home/ovc-20197388. Accessed 22 Sept 2017. forans treated with adalimumab with satisfactory re- 2. Rheumatoid Arthritis (RA). http://www.medicinenet.com/rheumatoid_ sults . arthritis/article.html. Accessed 22 Sept 2017. Ghauri et al. Journal of Medical Case Reports (2018) 12:155 Page 4 of 4 3. Tong L, Thumboo J, Tan YK, Wong TY, Albani S. The eye: a window of opportunity in rheumatoid arthritis? 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Journal of Medical Case Reports
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Published: Jun 5, 2018