Necrotising Anterior Scleritis and Scleroderma: A Rare Association
Authors: A. Vassila, D. Kalogeropoulos, R. Rawashdeh, N. Hall, N. Rahman, M. Fabian, S. Thulasidharan, H. Parwez
Abstract:
Necrotising scleritis is a severe form of scleritis and poses a significant threat to vision. It can manifest in various systemic autoimmune disorders, systemic vasculitis, or as a consequence of microbial infections. The objective of this study is to present a case of necrotizing scleritis associated with scleroderma, which was further complicated by a secondary Staphylococcus epidermidis infection. This is a retrospective analysis, which examines the medical records of a patient who was hospitalised in the Eye Unit at University Hospital Southampton. A 78-year-old woman presented at the eye casualty department of our unit with a two-week history of progressively worsening pain in her left eye. She received a diagnosis of necrotising scleritis and was admitted to the hospital for further treatment. It was decided to commence a three-day course of intravenous methylprednisolone followed by a tapering regimen of oral steroids. Additionally, a conjunctival swab was taken, and two days later, it revealed the presence of S. epidermidis, indicating a potential secondary infection. Given this finding, she was also prescribed topical (Ofloxacin 0.3% - four times daily) and oral (Ciprofloxacin 750 mg – twice daily) antibiotics. The inflammation and symptoms gradually improved, leading to the patient being scheduled for a scleral graft and applying an amniotic membrane to cover the area of scleral thinning. Rheumatoid arthritis and granulomatosis with polyangiitis are the most commonly identifiable systemic diseases associated with necrotising scleritis. Although association with scleroderma is extremely rare, early identification and treatment are necessary to prevent scleritis-related complications.
Keywords: Scleritis, necrotizing scleritis, scleroderma, autoimmune disease.
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[1] Daniel Diaz J, Sobol EK, Gritz DC. Treatment and management of scleral disorders. Surv Ophthalmol. 2016 Nov-Dec;61(6):702-717. doi: 10.1016/j.survophthal.2016.06.002. Epub 2016 Jun 16. PMID: 27318032.
[2] Nizam S, Johnstone A, Green M, Gough A. Necrotising scleritis and connective tissue disease--three cases and a review. Clin Rheumatol. 2009 Mar;28(3):339-41. doi: 10.1007/s10067-008-1054-2. Epub 2008 Dec 4. PMID: 19052835.
[3] Smith JR, Mackensen F, Rosenbaum JT. Therapy insight: scleritis and its relationship to systemic autoimmune disease. Nat Clin Pract Rheumatol. 2007 Apr;3(4):219-26. doi: 10.1038/ncprheum0454. PMID: 17396107.
[4] McMulen M, Kovarik G, Hodge WG. Use of topical steroid therapy in the management of nonnecrotizing anterior scleritis. Can J ophthalmol.199 Jun; 34(4):217-21. PMID: 10396658
[5] Beardsley RM, Suhler EB, Rosenbaum JT, Lin P. Pharmacotherapy of scleritis: current paradigms and future directions. Expert Opin Pharmacother. 2013 Mar;14(4):411-24. doi: 10.1517/14656566.2013.772982. Epub 2013 Feb 21. PMID: 23425055; PMCID: PMC3888795.
[6] McCluskey P, Wakefield D. Intravenous pulse methylprednisolone in scleritis. Arch Ophthalmol. 1987 Jun;105(6):793-7. doi: 10.1001/archopht.1987.01060060079037. PMID: 3579710.
[7] Sands DS, Chan SCY, Gottlieb CC. Methotrexate for the treatment of noninfectious scleritis. Can J Ophthalmol. 2018 Aug;53(4):349-353. doi: 10.1016/j.jcjo.2017.11.009. Epub 2018 Feb 13. PMID: 30119788.
[8] Mellado F, Talesnik E, Castiglione E. Azathioprine as monotherapy for scleritis in relapsing polychondritis. Ocul Immunol Inflamm. 2012 Jun;20(3):235-6. doi: 10.3109/09273948.2012.659369. Epub 2012 Mar 27. PMID: 22449393.
[9] Kolomeyer AM, Ragam A, Shah K, Jachens AW, Tu Y, Chu DS. Mycophenolate mofetil in the treatment of chronic non-infectious, non-necrotizing scleritis. Ocul Immunol Inflamm. 2012 Apr;20(2):113-8. doi: 10.3109/09273948.2012.655398. PMID: 22409564.
[10] de Fidelix TS, Vieira LA, de Freitas D, Trevisani VF. Biologic therapy for refractory scleritis: a new treatment perspective. Int Ophthalmol. 2015 Dec;35(6):903-12. doi: 10.1007/s10792-015-0124-0. Epub 2015 Aug 29. PMID: 26319144.
[11] Grennan D, Wang S. Steroid Side Effects. JAMA. 2019 Jul 16;322(3):282. doi: 10.1001/jama.2019.8506. PMID: 31310300.
[12] LiverTox: Clinical and Research Information on Drug-Induced Liver Injury (Internet). Bethesda (MD): National Institute of Diabetes and Digestive and Kidney Diseases; 2012–. Fluoroquinolones. 2020 Mar 10. PMID: 31643179.
[13] Sainz de la Maza M, Foster CS, Jabbur NS. Scleritis-associated uveitis. Ophthalmology. 1997 Jan;104(1):58-63. doi: 10.1016/s0161-6420(97)30361-3. PMID: 9022105.
[14] Smith JR, Mackensen F, Rosenbaum JT. Therapy insight: scleritis and its relationship to systemic autoimmune disease. Nat Clin Pract Rheumatol. 2007 Apr;3(4):219-26. doi: 10.1038/ncprhQeum0454. PMID: 17396107.