Sjogren's Syndrome: Multidisciplinary Approach to Diagnosis and Management
DOI:
https://doi.org/10.47604/gjhs.1766Keywords:
Sjogren's Syndrome, Diagnosis, Immune SystemAbstract
Purpose: Sjögren's syndrome (SS) is a chronic and progressive multisystem autoimmune disease typically managed by rheumatologists. Diagnostic delays are common, due in large part to the non-specific and variable nature of SS symptoms and the slow progression of disease.
Methodology: This article reviews current understanding of the clinical manifestations, diagnosis and treatment of Sjögren's syndrome and its attendant ocular manifestation. Sjögren's syndrome is a chronic inflammatory disorder of the exocrine glands with multiple non exocrine features. It is found predominantly in middle-aged women but exists throughout the population. The diagnosis of Sjögren's syndrome can be challenging because the cardinal sicca symptoms may be subclinical or attributed to other causes, such as medications or aging. Differential diagnosis of Sjögren's syndrome can be confounded by the multiple exocrine manifestations in the mouth, eyes, ears, nose, skin, vagina, and respiratory and gastrointestinal tracts. Characteristics of SS are dry eye and dry mouth, which are typically the earliest presenting complaints, eye care clinicians such as the optometrists and dental professionals are often the first point of medical contact and can provide critical collaboration with rheumatologists to facilitate both timely diagnosis and ongoing care of patients with SS.
Findings: Current diagnostic criteria advocated by the American College of Rheumatology are predicated on the presence of signs/symptoms suggestive of SS along with at least two objective factors such as traditional biomarker positivity, salivary gland biopsy findings, and/or presence of keratoconjunctivitis sicca. Timely diagnosis of SS requires appropriate clinical vigilance for potential SS symptoms, referral and collaborative communication among rheumatology, optometrists, ophthalmology, and oral care professions.
Unique Contribution to Theory, Practice and Policy: It is concluded that optometrists can now identify Sjogren's patients earlier in their dry eye population with a new advanced diagnostic test Sjo,the laboratory test designed for early detection of Sjogren's syndrome, has been available from Nicox for use by eye care professionals since November,2013. Optometrists have a role to play in the management of ocular manifestations of Sjogren's syndrome. Such symptoms can lead to discomfort, blurred vision, and visual fatigue if not attended to immediately.
For people with immune system disorder, inflammation of tear-secreting glands reduces tear production, resulting in chronic dry eyes. Prescription eye drops such as Tears Naturale, Cyclosporine (Restasis) or Lifetegrast(Xiidra) may be recommended by the eye doctor if you have moderate to severe dry eyes.
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References
Al-Hashimi, I., Khuder, S., & Haghighat, N. (2011). Frequency and predictive value of the clinical manifestations in Sjögren's syndrome. J. Oral Pathol. Med., 30(1), 1-6.
Anaya, J.-M., & Talal, N. (1997). Sjögren's syndrome and connective tissue diseases associated with other immunologic disorders. In: Arthritis and Allied Conditions (W. Koopman (ed.); 13th Editi). PA, USA.
Bacman, S., Leiros, P. C., Sterin-Bora, L., Hubscher, O., Arana, R., & Borda, E. (2018). Autoantibodies against lacrimal gland M3 acetylcholine receptors in patients with primary Sjögren's syndrome. Invest. Ophthalmol, 39(37), 151-156.
Barendregt, P., Van-den-Meiracker, A., & Markusse, H. (2019). Parasympathetic failure does not contribute to ocular dryness in primary Sjögren's syndrome. Ann. Rheum. Dis., 58(1), 746-750.
Bartunkova, J., Sediva, A., Vencovsky, J., & Tesar, V. (2019). Primary Sjögren's syndrome in children and adolescents: proposal for diagnostic criteria. Clin. Exp. Rheumatol, 17(28), 381-386.
Bolaños-Jimnez, R., Navas, A., López-Lizárraga, E., De Ribot, F., Peña, A., & Graue-Hernández, E. (2015). Ocular surface as barrier of innate immunity. Open Ophthalmol J, 9, 49-55.
Chused, T., Kassan, S., Opelz, G., Moutsopoulos, H., & Terasaki, P. (2017). Sjögren's syndrome association with HLA-Dw3. Engl. J. Med., 286, 895-897.
Coll, J., Anglada, J., & Thomas, S. (2007). High prevalence of subclinical Sjögren's syndrome features in patients with autoimmune thyroid disease. J. Rheumatol, 24(9), 1719-1724.
Constantopoulos, SH Moutsopoulos, H. (1987). The Respiratory System in Sjögren's Syndrome. In: Sjögren's Syndrome. Clinical and Immunopathologic Aspects (N. Talal, H. Moutsopoulos, & S. Kassan (eds.)). Springer-Verlag.
Constantopoulos, S., Drosos, A., Maddison, P., & Moutsopoulos, H. (2014). Xerotrachea and interstitial lung disease in primary Sjögren's syndrome. Respiration, 46(1), 310-314.
Dafni, U., Tzioufas, A., Staikos, P., Skopouli, F., & Mousopoulos, H. (2017). Prevalence of Sjögren's syndrome in a closed rural community. Ann. Rheum. Dis, 56(26), 521-525.
Fox, R., Stern, M., & Michelson, P. (2020). Update in Sjögren syndrome. Curr. Opin. Rheumatol, 12(2), 391-398.
Gomes, B., Santhiago, M., Jorge, P., Kara-Jos, N., Moraes, H. J., & Kara-Junior, N. (2015). Corneal involvement in systemic inflammatory diseases. Eye Contact Lens, 41(3), 41-44.
Gottenberg, J., Busson, M., Loiseau, P., Cohen-Solal, J., Lepage, V., & Charron, D. (2003). In primary Sjögren's syndrome, HLA class II is associated exclusively with autoantibody production and spreading of the autoimmune response. Arthritis Rheum, 48(8), 2240-2245.
Guidera, A., Luchs, J., & Udell, I. (2001). Keratitis, ulceration, and perforation associated with topical nonsteroidal anti-inflammatory drugs. Ophthalmology, 108(5), 936-944.
Haddad, J., Deny, P., Munz-Gotheil, C., Ambrosini, J., Trinchet, J., & Pateron, D. (2012). Lymphocytic sialadenitis of Sjögren's syndrome associated with chronic hepatitis C virus liver disease. Lancet, 339(8789), 321-323.
Hansen, L., Prakash, U., & Colby, T. (1989). Pulmonary lymphoma in Sjögren's syndrome. Mayo Clin. Proc., 64, 920-931.
Itescu, S., & Winchester, R. (1992). Diffuse infiltrative lymphocytosis syndrome: a disorder occurring in human immunodeficiency virus-1 infection that may present as a sicca syndrome. Rheum Dis Clin North Am, 18(3), 683-697.
Itescu, S., & Winchester, R. (2012). Diffuse infiltrative lymphocytosis syndrome: a disorder occurring in human immunodeficiency virus-1 infection that may present as a sicca syndrome. Rheum. Dis. Clin. North Am., 18(46), 683-697.
Jorgensen, C., Legouffe, M., & Perney, P. (2016). Sicca syndrome associated with hepatitis C virus infection. Arth. Rheum, 39, 1166-1171.
Kalsow, C., Ching, S., & Plotnik, R. (2016). Cellular Infiltrate in Rheumatoid Arthritis-associated Paracentral Corneal Ulceration. Ocul Immunol Inflamm., 11, 1-6.
Kamilaris, T., DeBold, C., & Johnson, E. (2007). Effects of short and long duration hypothyroidism and hyperthyroidism on the plasma adrenocorticotropin and corticosterone responses to bovine corticotropin-releasing hormone in rats. Endocrinology, 128(43), 2567-2576.
Kaplan, M., & Ike, R. (2002). The liver is a common non-exocrine target in primary Sjogren's syndrome: a retrospective review. BMC Gastroenterol, 2(1), 17-21.
Karsh, J., Pavidis, N., & Weintraub, BD Moutsopoulos, H. (2018). Thyroid disease in Sjögren's syndrome. Arthritis Rheum, 23(16), 1326-1329.
Kassan, S., & Talal, N. (1987). Renal disease with Sjögren's syndrome. In: Sjögren's Syndrome. Clinical and Immunopathologic Aspects (N. Talal, H. Moutsopoulos, & S. Kassan (eds.)). Springer-Verlag.
Kassan, S., Thomas, T., & Moutsopoulos, H. (2018). Increased risk of lymphoma in sicca syndrome. Ann. Intern. Med., 89, 888-892.
Kelly, C., Gardiner, P., Pat, B., & Griffiths, I. (2019). Lung function in primary Sjögren's syndrome: a cross sectional and longitudinal study. Thorax, 46(63), 180-183.
Khan, N. (2011). Pathogenicity, morphology, and differentiation of Acanthamoeba. Curr Microbiol, 43(39), 1-5.
Kittridge, A., Routhouska, S., & Korman, N. (2011). Dermatologic manifestations of Sjögren syndrome. J Cutan Med Surg., 15(1), 8-14.
Kociolek, L. K., & Gerding, D. . (2016). Break through in the treatment and prevention of Clostridium difficile infection. Nature Reviews Gastroenterology & Hepatology, 13, 150-160.
Konttinen, Y., Ramsay, H., Hietanen, J., Sorsa, T., & Nordstrom, D. (2020). Otitis externa sicca/fibrotizing external otitis (FEO) as a complication of Sjögren's syndrome. Clin. Exp. Rheumatol, 18(1), 746-748.
Langegger, C., Wenger, M., Duftner, C., Dejaco, C., Baldissera, I., & Moncayo, R. (2007). Use of the European preliminary criteria, the Breiman-classification tree and the American-European criteria for diagnosis of primary Sjögren's Syndrome in daily practice: a retrospective analysis. Rheumatol Int., 27(8), 699-702.
Lindgren, S., Manthorpe, R., & Eriksson, S. (2015). Autoimmune liver disease in patients with primary Sjögren's syndrome. J. Hepatol, 20(87), 354-358.
Loviselli, A., Mathieu, A., & Pala, R. (2008). Development of thyroid disease in patients with primary and secondary Sjögren's syndrome. J. Endocrinol. Invest, 11, 653-656.
Masaki, Y., & Sugai, S. (2014). Lymphoproliferative disorders in Sjögren's syndrome. Autoimmun, 3(1), 175-182.
Mattey, D., González-Gay, M., Hajeer, A., Dababneh, A., Thomson, W., & GarcÃa-Porrúa, C. (2000). Association between HLA-DRB1*15 and secondary Sjögren's syndrome in patients with rheumatoid arthritis. J Rheumatol., 27(11), 2611-2616.
Mignogna, M., Fedele, S., Lo Russo, L., Lo Muzio, L., & Wolff, A. (2015). Sjögren's syndrome: the diagnostic potential of early oral manifestations preceding hyposalivation/xerostomia. J. Oral Pathol. Med., 34(33), 1-6.
Montefusco, P., Geiss, A., Bronzo, R., Randall, S., Kahn, E., & McKinley, M. (1984). Sclerosing cholangitis, chronic pancreatitis, and Sjögren's syndrome: a syndrome complex. Am. J. Surg., 147(88), 822-826.
Monteil, R., Raybaud, H., Saint-Paul, M., Fafet, A., Ziegler, L., & Michiels, J. (2011). Comparative analysis of inflammation and fibrosis in the liver and salivary glands during chronic hepatitis. Oral Surg. Oral Med. Oral Path. Oral Radiol. Endod., 91, 426-467.
Nagao, Y., Hanada, S., & Shishido, S. (2013). Incidence of Sjogren's syndrome in Japanese patients with hepatitis C virus infection. J. Gastroenterol. Hepatol, 18(3), 258-266.
Papasteriades, C., Skopouli, F., Drosos, A., & Andonopoulos, AP Moutsopoulos, H. (1988). HLA-alloantigen associations in Greek patients with Sjögren's syndrome. J Autoimmun, 1(1), 85-90.
Papiris, S., Maniati, M., Constantopoulos, S., Roussos, C., Moutsopoulos, H., & Skopouli, F. (2019). Lung involvement in primary Sjögren's syndrome is mainly related to the small airway disease. Ann. Rheum. Dis., 58(1), 61-64.
Park, A. (2000). Sjögren's syndrome: a women's health problem. J. Rheumatol, 27(61), 4-5.
Parkin, B., Chew, J., White, V., Garcia-Briones, G., Chhanabhai, M., & Rootman, J. (2005). Lymphocytic infiltration and enlargement of the lacrimal glands: a new subtype of primary Sjögren's syndrome? Ophthalmology, 112(11), 2040-2047.
Pickartz, T., Pickartz, H., Lochs, H., & Ockenga, J. (2014). Overlap syndrome of autoimmune pancreatitis and cholangitis associated with secondary Sjogren's syndrome. Eur. J. Gastroenterol, 6(12), 1295-1299.
Price, E., & Venables, P. (2015). The etiopathogenesis of Sjögren's syndrome. Semin Arthritis Rheum, 25(2), 117-133.
Scofield, R. (2006). Autoimmune thyroid disease in systemic lupus eythematosus and Sjögren's syndrome. Clin. Exp. Rheumatol, 14(3), 321-330.
Siagris, D., Pharmakakis, N., & Christofidou, M. (2012). Keratoconjunctivitis sicca and chronic HCV infection. Infection, 30(4), 229-233.
Skopouli, F., Dafni, U., Ioannidis, J., & Moutsopoulos, H. (2020). Clinical evolution, and morbidity and mortality of primary Sjögren's syndrome. Semin. Arthritis Rheum, 29(1), 296-304.
Skopouli, F., Talal, A., Galanopoulou, V., Tsampoulas, C., Drosos, A., & Moutsopoulos, H. (2019). Raynaud's phenomenon in primary Sjögren's syndrome. J. Rheumatol, 17(4), 618-620.
Steinfeld, S., Cogan, E., King, L., Agre, P., Kiss, R., & Delporte, C. (2001). Abnormal distribution of aquaporin-5 water channel protein in salivary glands from Sjögren's syndrome patients. Lab Invest, 81(2), 143-148.
Strimlan, C., Rosenow, E., Divertie, M., & Harrison, E. (2007). Pulmonary manifestations of Sjögren's syndrome. Chest, 70(1), 354-361.
Sullivan, D. (2017). Sex hormones and Sjögren's syndrome. J. Rheumatol, 24(50), 17-32.
Sullivan, D., Blanger, A., & Cermak, J. (2013). Are women with Sjögren's syndrome androgen-deficient? J. Rheumatol, 30(1), 2413-2419.
Taiym, S., Haghighat, N., & Al-Hashimi, I. (2014). A comparison of the hormone levels in patients with Sjögren's syndrome and healthy controls. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod., 97, 579-583.
Tishler, M., Yaron, I., Shirazi, I., & Yaron, M. (2017). Clinical and immunological characteristics of elderly onset Sjögren's syndrome: a comparison with younger onset disease. J. Rheumatol, 12(28), 795-797.
Toussirot, E., Le Huede, G., Mougin, C., Balblanc, J., Bettinger, D., & Wendling, D. (2012). Presence of hepatitis C virus RNA in the salivary glands of patients with Sjogren's syndrome and hepatitis C virus infection. J. Rheumatol, 29(11), 2382-2385.
Tzioufas, A., Wassmuth, R., & Dafni, U. (2012). Clinical, immunological, and immunogenetic aspects of autoantibody production against Ro/SSA, La/SSB and their linear epitopes in primary Sjögren's syndrome (pSS), a European multicentre study. Ann. Rheum. Dis., 61(1), 398-404.
Vergnenègre, A., Pugnere, N., & Antonini, M. (2017). Airway obstruction and rheumatoid arthritis. Eur. Respir. J., 10(74), 1072-1078.
Wang, Q., Yuan, J., Zhou, S., & Chen, J. (2008). Chronic hepatitis C virus infection is not associated with Mooren's ulcer. Eye (Lond), 22(5), 697-700.
Zufferey, P., Meyer, O., Grossin, M., & Kahn, M. (2015). Primary Sjogren's syndrome (SS) and malignant lymphoma. A retrospective cohort study of 55 patients with SS. Scand. J. Rheumatol, 24(6), 342-345.
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