Ocular Surface Disease in Sjögren's Syndrome: Management in a Scleral Lens Clinical Practice

Main Article Content

Daddi Fadel
Melissa Barnett

Abstract

Sjögren’s syndrome is a chronic, autoimmune, systemic disease characterized by lymphocytic infiltration and malfunction of the exocrine glands, primarily the lacrimal and salivary glands, resulting in predominant symptoms of dry eye and dry mouth. Sjögren’s syndrome is a highly prevalent condition and is one of the most common systemic, rheumatic, autoimmune diseases, affecting up to 1.4% of adults in the United States, second only to rheumatoid arthritis in its prevalence in North America. Primary Sjögren’s syndrome has shown to affect patients’ health-related quality-of-life due to dryness, chronic pain, depression, anxiety,
physical and mental fatigue, and neuropsychiatric symptoms.


Scleral lenses (SLs) have shown to be significantly beneficial in relieving symptoms and improving
quality-of-life in patients with Sjögren’s syndrome and dry eye disease. SLs may be used concurrently with the other therapies including ocular lubricants, eyelid hygiene, punctal occlusion, topical prescription medications, and autologous serum.


This manuscript reviews the implication of Sjögren’s syndrome on the ocular surface and quality-of-life and describes how SLs, in combination with other treatments, may be beneficial.

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How to Cite
1.
Fadel D, Barnett M. Ocular Surface Disease in Sjögren’s Syndrome: Management in a Scleral Lens Clinical Practice. JCLRS [Internet]. 2020 Aug. 25 [cited 2024 Dec. 5];4(1):e12-e22. Available from: http://www.jclrs.org/index.php/JCLRS/article/view/36
Section
Review Article
Author Biography

Melissa Barnett, University of California, Davis

Department of Ophthalmology and Vision Science, University of California, Davis

References

Bloch K, Buchanan W, Wohl M, et al. Sjögren’s syndrome: A clinical, pathological and serological study of 62 cases. Medicine (Baltimore) 1965;44:187–31.

Kassan S, Moutsopoulos H. Clinical manifestations and early diagnosis of Sjögren’s syndrome, Arch Intern Med 2004;164:1275–84.

Dumusc A, Rao V, Bowman SJ. Sjögren’s syndrome. Medicine 2018;46:126–30.

Helmick C, Felson D, Lawrence R, et al. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Arthritis Rheum 2008;58:15–25.

Qin B, Wang J, Yang Z, et al. Epidemiology of primary Sjögren’s syndrome: a systematic review and meta-analysis. Ann Rheum Dis 2015;74:1983–9.

Absher DM, Li X, Waite LL, et al. Genome-wide dna methylation analysis of systemic lupus erythematosus reveals persistent hypomethylation of interferon genes and compositional changes to CD4+ T-cell populations. PLoS Genet 2013;9:e1003678.

Greer JM, McCombe PA. The role of epigenetic mechanisms and processes in autoimmune disorders. Biologics 2012;6:307–27.

Inoshita M, Numata S, Tajima A, et al. Sex Differences of leukocytes DNA methylation adjusted for estimated cellular proportions. Biol Sex Differ 2015;6:11.

Fish EN. The X-files in Immunity: Sex-based differences predispose immune responses. Nat Rev Immunol 2008;8:737–44.

Kovats S. Estrogen receptors regulate innate immune cells and signaling pathways. Cell Immunol 2015;294:63–9. 11. McMurray RW, May W. Sex Hormones and systemic lupus erythematosus: review and meta-analysis. Arthritis Rheum 2003;48:2100–10.

Porola P, Laine M, Virkki L, et al. The influence of sex steroids on Sjögren’s syndrome. Ann N Y Acad Sci 2007;1108:426–32.

Selmi C, Lu Q, Humble MC. Heritability versus the role of the environment in autoimmunity. J Autoimmun 2012;39:249–52.

Ramírez Sepúlveda JI, Kvarnström M, Brauner S, et al. Difference in clinical presentation between women and men in incident primary Sjögren’s syndrome. Biol Sex Diff 2017;8:16.

Vivino F. Sjögren’s syndrome : Clinical aspects. Clinical Immunology 2017;182:48–54.

Uchino, M, Schaumberg, DA. Dry eye disease: impact on quality-of-life and vision. Curr Ophthalmol Rep 2013;1(2):51–57.

Cornec D, Devauchelle-Pensec V, Mariette X, et al. Severe health-related quality-of-life impairment inactive primary Sjögren’s syndrome is driven by patient-reported outcomes: data from a large therapeutic trial. Arthritis Care Res (Hoboken) 2017;69(4):528–35.

Lackner A, Stradner MH, Hermann J, et al. Assessing health-related quality-of-life in primary Sjögren’s syndrome-the PSS-QoL. Semin Arthritis Rheum 2018;48(1):105–10.

Jones L, Downie LE, Korb D, et al. TFOS DEWS II Management and Therapy Report. Ocul Surf 2017;15(3):575–28.

Fadel D, Kramer E. Potential contraindications to scleral lens wear. Cont Lens Anterior Eye 2019;41(1):92–103. 21. La Porta Weber S, Becco de Souza R, Gomes JÁP, Hofling-Lima AL. The use of the Esclera scleral contact lens in the treatment of moderate to severe dry eye disease. Am J Ophthalmol 2016 Mar;163:167–73.

Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren’s Syndrome: a consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis 2017;76:9–16.

Mathews PM, Hahn S, Hessen M, Kim J, et al. Ocular complications of Primary Sjögren syndrome in men. Am J Ophthalmol 2015;160(3):447–52.

Pflugfelder, S. What causes dryness in Sjögren’s syndrome patients and how can it be targeted? Exp Rev Clin Immunol 2014:10:4, 425–27.

Nguyen CQ, Peck AB. Unraveling the pathophysiology of Sjögren syndrome-associated dry eye disease. Ocul Surf 2009;7(1):11–27.

Geerling G, MacLennan S, Hartwing D. Autologus serum eye drops for ocular surface disorders. Br J Ophtalmol 2004;88(11):1467–74.

Sobrin L, Selzer MG, Lokeshwar BL, et al. Stromely-sin (MMP-3) Activates Pro-MMP-9 secreted by corneal epithelial cells. Invest Ophthalmol Vis Sci 2000;41(7):1703–9.

Chotikavanich S, de Paiva CS, Li DQ, et al. Production and activity of matrix metalloproteinase-9 on the ocular surface increase in dysfunctional tear syndrome. Invest Ophthalmol Vis Sci 2009;50(7):3203–9.

van Bijsterveld O. Diagnostic Tests in the Sicca Syndrome. Arch Ophthalmol 1969;82:10–14.

Wolffsohn J, Arita R, Chalmers R, et al. TFOS DEWS II Tear Film Report. Ocul Surf 2017;15:539–74.

Acs M, Caffery B, Barnett M, et al. Customary practices in the monitoring of dry eye disease in Sjögren’s syndrome. J Optom 2018;11(4):232–41.

Isik H, Isik M, Aynioglu O, et al. Are the women with Sjögren’s syndrome satisfied with their sexual activity? Rev Bras Reumatol Engl Ed 2017;57(3):210–16.

Mulherin DM, Sheeran TP, Kumararatne DS, et al. Sjögren’s syndrome in women presenting with chronic dyspareunia. Br J Obstet Gynaecol1997;104(9):1019–23.

Belenguer R, Ramos-Casals M, Brito-Zerón P, et al. Influence of clinical and immunological parameters on the health-related quality-of-life of patients with primary Sjögren’s syndrome. Clin Exp Rheumatol 2005;23(3):351–6.

van Nimwegen JF, Arends S, van Zuiden GS, et al. The Impact of Primary Sjögren’s Syndrome on Female Sexual Function. Rheumatology (Oxford). 2015;54(7):1286–93.

Visser E-S, Visser R, van Lier HJJ, Otten HM. Modern scleral lenses part I: Clinical features. Eye Contact Lens Sci Clin Pract 2007; 33:13–20.

Ng A, Sorbara L. Case report: Using scleral lens wear to managing severe ocular dryness secondary to an autoimmune disorder. Cont Lens Anter Eye 2018;41;S1:S50.

Wang Y, Kornberg DL, St Clair RM, et al. Corneal nerve structure and function after long-term wear of fluid-filled scleral lens. Cornea 2015;34(4):427–32.

Situ P, Simpson TL, Fonn D, et al. Conjunctival and corneal pneumatic sensitivity is associated with signs and symptoms of ocular dryness. Invest Ophthalmol Vis Sci 2008; 49:2971–76.

Labbé A, Alalwani H, Van Went C, et al. The relationship between subbasal nerve morphology and corneal sensation in ocular surface disease. Invest Ophthalmol Vis Sci 2012; 53:4926–31.

Labbé A, Liang Q, Wang Z, et al. Corneal nerve struc-ture and function in patients with non-Sjogren dry eye: Clinical correlations. Invest Ophthalmol Vis Sci 2013; 54:5144–50.

Villani E, Galimberti D, Viola F, et al. The cornea in Sjogren’s syndrome: an in vivo confocal study. Invest Ophthalmol Vis Sci 2007; 48:2017–22.

Tuisku IS, Konttinen YT, Konttinen LM, et al. Alterations in corneal sensitivity and nerve morphology in patients with primary Sjögren’s syndrome. Exp Eye Res 2008; 86:879–85.

Ormerod LD, Fong LP, Foster CS. Corneal infection in mucosal scarring disorders and Sjogren’s syndrome. Am J Ophthalmol 1988;105(5):512–8.

Rosenthal P, Cotter JM, Baum J. Treatment of persis-tent corneal epithelial defect with extended wear of a fluid-ventilated gas-permeable scleral contact lens. Am J Ophthalmol 2000;130:33–41.

Kalwerisky K, Davies B, Mihora L, et al. Demartelaere S. Use of the Boston Ocular Surface Prosthesis in the management of severe periorbital thermal injuries: a case series of 10 patients. Ophthalmology 2012;119:516–21.

Zimmerman AB, Marks A. Microbial Keratitis sec-ondary to unintended poor compliance with scleral gas-permeable contact lenses. Eye Contact Lens Sci Clin Pract 2014;40:e1–4.

Severinsky B, Behrman S, Frucht-Pery J, Solomon A. Scleral contact lenses for visual rehabilitation after penetrating keratoplasty: long term outcomes. Contact Lens Anterior Eye 2014;37:196–02.

Farhat B, Sutphin JE. Deep anterior lamellar keratoplasty for acanthamoeba keratitis complicating the use of Boston scleral lens. Eye Contact Lens 2014;40:e5–7.

Fadel D, Toabe M. Compliance using scleral lenses. J Cont Lens Res Sci 2018 April;2(1):e22–29.

Fernandes M, Sharma S. Polymicrobial and microsporidial keratitis in a patient using Boston scleral contact lens for Sjogren’s syndrome and ocular cicatricial pemphigoid. Contact Lens Anterior Eye 2013;36(2):95–7.

Sharma S, Das S, Joseph J, Vemuganti GK,et al. Mi-crosporidial keratitis: need for increased awareness. Survey Ophthalmol 2011;56:1–22.

Fadel D. Scleral lens issues and complications related to a non-optimal fitting relationship between the lens and ocular surface. Eye Contact Lens 2019;45(3):152–63.

Foulks GN, Forstot SL, Donshik PC, et al. Clinical guidelines for management of dry eye associated with Sjögren Disease. Ocul Surf;2015:13(2):118–32.

Choi JH, Kim JH, Li Z, et al. Efficacy of the mineral oil and hyaluronic acid mixture eye drops in murine dry eye. Korean J Ophthalmol 2015;29(2):131–7.

Lanzini M, Curcio C, Colabelli-Gisoldi RA, et al. In vivo and impression cytology study on the effect of compatible solutes eye drops on the ocular surface epithelial cell quality in dry-eye patients. Mediators Inflamm 2015;2015:351424.

Martin E, Oliver KM, Pearce EI, Tomlinson A, et al. Effect of tear supplements on signs, symptoms and inflammatory markers in dry eye. Cytokine 2018 May;105:37–44.

Caffery B, Harthan J, Srinivasan S, et al. sjögren’s syndrome in optometric practices in North America. Contact Lens Anterior Eye 2018;41(6):518–26.

Benitez-Del-Castillo JM. How to promote and preserve eyelid health. Clin Ophthalmol 2012;6:1689–98.

Maher TN. The use of tea tree oil in treating blepharitis and meibomian gland dysfunction. Oman J Ophthalmol 2018;11(1):11–15.

Romanowski EG, Stella NA Yates KA, et al. In vitro evaluation of a hypochlorous acid hygiene solution on established biofilms. Eye Contact Lens 2018;44 Suppl 2:S187–91.

Murphy O, O’Dwyer V, Lloyd-McKernan A. The efficacy of tea tree face wash, 1, 2-Octanediol and micro-blepharoexfoliation in treating demodex folliculorum blepharitis. Contact Lens Anter Eye 2018;41(1):77–82.

Alghamdi YA, Camp A, Feuer W, et al. Compliance and subjective patient responses to eyelid hygiene. Eye Contact Lens 2017;43(4):213–17.

Qiu W, Liu Z, Ao M, et al. Punctal plugs versus artificial tears for treating primary Sjögren’s syndrome with keratoconjunctivitis sicca: a comparative observation of their effects on visual function. Rheumatol Int 2013;33:2543–8.

Marsh P, Pflugfelder SC. Topical Nonpreserved methylprednisolone therapy for keratoconjunctivitis sicca in Sjögren syndrome. Ophthalmology 1999;106:811–6.

Hong S, Kim T, Chung SH, et al. Recurrence after topical non-preserved methylprednisolone therapy for keratoconjunctivitis sicca in Sjögren’s syndrome. J Ocul Pharmacol Ther 2007;23:78–82.

Kunert KS, Tisdale AS, Gipson IK. Goblet cell numbers and epithelial proliferation in the conjunctiva of patients with dry eye syndrome treated with cyclosporine. Arch Ophthalmol 2002;120:330–37.

Gündüz K, Ozdemir O. Topical cyclosporin treatment of keratoconjunctivitis sicca in secondary Sjögren’s syndrome. Acta Ophthalmol 1994;72:438–42.

Sall K, Stevenson OD, Mundorf TK, Reis BL. Two multicenter, randomized studies of the efficacy and safety of cyclosporine ophthalmic emulsion in moderate to severe dry eye disease. CsA Phase 3 Study Group. Ophthalmology 2000;107:631–39.

Stevenson D, Tauber J, Reis BL. Efficacy and safety of cyclosporine a ophthalmic emulsion in the treatment of moderate-to-severe dry eye disease: a dose-ranging, randomized trial. The Cyclosporin A Phase 2 Study Group. Ophthalmology 2000;107:967–74.

Perez VL, Pflugfelder SC, Zhang S, et al. Lifitegrast, a novel integrin antagonist for treatment of dry eye disease. Ocul Surf 2016;14:207-15.

Stern ME, Gao J, Schwalb TA, et al. Conjunctival T-cell populations in Sjögren’s and non-Sjögren’s patients with dry eye. Invest Ophthalmol Vis Sci 2002;43:2609–14.

Tauber J, Karpecki P, Latkany R, et al. Lifitegrast ophthalmic solution 5.0% versus placebo for treatment of dry eye disease: Results of The Randomized Phase III OPUS-2 Study. Ophthalmology 2015;122:2423–31.

Holland EJ, Luchs J, Karpecki PM, Nichols KK, et al. Lifitegrast for the treatment of dry eye disease: Results of a Phase III, Randomized, Double-Masked, Placebo-Controlled Trial (OPUS-3). Ophthalmology 2017 Jan;124(1):53-60.

Tsubota K, Goto E, Fujita H, et al. Treatment of dry eye by autologous serum application in Sjögren’s syndrome. Br J Ophthalmol 1999;83(4):390–5.

Hwang J, Chung SH, Jeon S, Kwok SK, et al. Comparison of clinical efficacies of autologous serum eye drops in patients with primary and secondary Sjögren syndrome. Cornea 2014;33(7):663–7.