Primary Sjögren’s Syndrome - A Review
DOI:
https://doi.org/10.22270/ajprd.v7i5.568Keywords:
Autoimmune disorder, Vitiligo, Depigmentation.Abstract
Primary Sjögren’s syndrome is a systemic autoimmune disease. The hallmark of the disease is exocrinopathy, which often results in dryness of the mouth and eyes, fatigue, and joint pain. These three symptoms are present in more than 80% of the patients with this disease and have a major effect on quality of life, primarily because of disabling fatigue, with associated loss of work productivityThe global prevalence calculated for the rarer pSS is 61 per 100 000 inhabitants. The patients’ quality of life is reduced by the diverse manifestations of the disease. Pharmacists can help SjS(Sjögren’s Syndrome) patients improve their quality of life.
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References
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11. Theander E, Vasaitis L, Baecklund E, et al.Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sjögren’s syndrome. Ann Rheum Dis 2011; 70:1363–8.
12. Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S: Pulmonary manifestations of Sjögren’s syndrome. Eur Respir Rev 2016; 25:110–23.
13. Evans R, Zdebik A, Ciurtin C, Walsh SB: Renal involvement in primary Sjögren’s syndrome. Rheumatology (Oxford) 2015; 549:1541–8.
14. Pavlakis PP, Alexopoulos H, Kosmidis ML, et al.: Peripheral neuropathies in Sjögren’s syndrome: a critical update on clinical features and pathogenetic mechanisms. J Autoimmun 2012; 39: 27–33.
15. Harms L, Hiepe F: Neurological manifestations in connective tissue disease. Z Rheumatol 2012; 71: 564–71.
16. Westhoff G, Dörner T, Zink A: Fatigue and depression predict physician visits and work disability in women with primary Sjogren’s syndrome: results from a cohort study. Rheumatology (Oxford) 2012; 51:262–9.
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22. Nardi N, Brito-Zeron P, Ramos-Casals M, et al.: Circulating autoantibodies against nuclear and non-nuclear antigens in primary Sjögren’s syndrome: prevalence and clinical significance in 335 patients. Clin Rheumatol 2006; 25:341–6.
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28. Daniels TE, Cox D, Shiboski CH, et al.: Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjögren’s syndrome among 1,726 registry participants. Arthritis Rheum 2011; 63:2021–30.
29. Seror R, Bowman SJ, Brito-Zeron P, et al.: EULAR Sjögren’s syndrome disease activity index (ESSDAI): a user guide. RMD Open 2015; 1: e000022.
30. Saraux A, Pers JO, Devauchelle-Pensec V: Treatment of primary Sjögren syndrome. Nat Rev Rheumatol 2016; 12:456–71.
31. Leonardi A, Van Setten G, Amrane M, et al.: Efficacy and safety of 0.1% cyclosporine A cationic emulsion in the treatment of severe dry eye disease: a multicenter randomized trial. Eur J Ophthalmol 2016; 26:287–96.
32. Romero-Rangel T, Stavrou P, Cotter J, Rosenthal P, Baltatzis S, Foster CS: Gas-permeable scleral contact lens therapy in ocular surface disease. Am J Ophthalmol 2000; 130: 25–32.
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34. Furness S, Bryan G, McMillan R, Worthington HV: Interventions for the management of dry mouth: non-pharmacological interventions. Cochrane Database Syst Rev 2013; (8): CD009603.
35. Albrecht K, Callhoff J, Westhoff G, Dietrich T, Dorner T, Zink A: The prevalence of dental implants and related factors in patients with Sjogren syndrome: results from a cohort study. J Rheumatol 2016; 43: 1380–5.
36. Carsons SE, Vivino FB, Parke A, et al.: Treatment guidelines for rheumatologic manifestations of Sjogren’s: use of biologics, management of fatigue and inflammatory musculoskeletal pain. Arthritis Care Res (Hoboken) 2016.
37. Gottenberg JE, Ravaud P, Puechal X, et al.: Effects of hydroxychloroquine on symptomatic improvement in primary Sjögren syndrome: the JOQUER randomized clinical trial. JAMA 2014; 312: 249–58.
38. Dass S, Bowman SJ, Vital EM, et al.: Reduction of fatigue in Sjögren’s syndrome with rituximab: results of a randomised, double-blind, placebo controlled pilot study. Ann Rheum Dis 2008; 67:1541–4.
39. Bowman S, Everett C, Bombardieri M, et al.: Preliminary results of a double-blind randomised trial of rituximab anti-B-cell therapy in patients with primary Sjogrens syndrome. Arthritis Rheumatol 2015; 67:3955–7.
40. Izmirly PM, Kim MY, Llanos C, et al.: Evaluation of the risk of anti- SSA/Ro-SSB/La antibody- associated cardiac manifestations of neonatal lupus in fetuses of mothers with systemic lupus erythematosus exposed to hydroxychloroquine. Ann Rheum Dis 2010; 6910: 1827–30.
41. Wallace DJ. The New Sjögren’s Syndrome Handbook. Oxford, UK: Oxford University Press; 2005; 24-32.
42. National Institute of Arthritis and Musculoskeletal and Skin Diseases website. www.niams.nih.gov. Accessed December. 2013.
43. Mengshoel AM, Norheim KB, Omdal R. Primary Sjögren’s syndrome: fatigue is an ever- present, fluctuating and uncontrollable lack of energy.2013; 10.
44. Vivino FB, Al-Hashimi I, Khan Z, et al. Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjögren syndrome: a randomized, placebo-controlled, fixed- dose, multicenter trial. P92-01 Study Group. Arch Intern Med. 1999; 159:174-181.
2. Meijer JM, Meiners PM, Huddleston Slater JJR, et al. Health-related quality of life, employment and disability in patients with Sjogren’s syndrome. Rheumatology (Oxford) 2009; 48:1077-82.
3. Westhoff G, Zink A: Epidemiology of primary Sjogren’s syndrome. Z Rheumatol 2010; 69: 41–9.
4. Shiboski CH, Shiboski SC, Seror R, et al. 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.
5. Fox PC, Bowman SJ, Segal B, et al.: Oral involvement in primary Sjögren syndrome. J Am Dent Assoc 2008; 139:1592–601.
6. Bron AJ, Tomlinson A, Foulks GN, et al.: Rethinking dry eye disease: a perspective on clinical implications. Ocul Surf 2014; 12:1–31.
7. Villa A, Abati S: Risk factors and symptoms associated with xerostomia: a cross-sectional study. Aust Dent J 2011; 56:290–5.
8. Cornec D, Saraux A, Jousse-Joulin S, et al.: The differential diagnosis of dry eyes, dry mouth, and parotidomegaly: a comprehensive review. Clin Rev Allergy Immunol 2015; 49:278–87.
9. Ramos-Casals M, Brito-Zeron P, Solans R, et al.: Systemic involvement in primary Sjögren’s syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry). Rheumatology (Oxford) 2014; 53:321–31.
10. Nishishinya MB, Pereda CA, Munoz-Fernandez S, et al.: Identifica - tion of lymphoma predictors in patients with primary Sjögren’s syndrome: a systematic literature review and meta- analysis. Rheumatol Int 2015; 35:17–26.
11. Theander E, Vasaitis L, Baecklund E, et al.Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sjögren’s syndrome. Ann Rheum Dis 2011; 70:1363–8.
12. Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S: Pulmonary manifestations of Sjögren’s syndrome. Eur Respir Rev 2016; 25:110–23.
13. Evans R, Zdebik A, Ciurtin C, Walsh SB: Renal involvement in primary Sjögren’s syndrome. Rheumatology (Oxford) 2015; 549:1541–8.
14. Pavlakis PP, Alexopoulos H, Kosmidis ML, et al.: Peripheral neuropathies in Sjögren’s syndrome: a critical update on clinical features and pathogenetic mechanisms. J Autoimmun 2012; 39: 27–33.
15. Harms L, Hiepe F: Neurological manifestations in connective tissue disease. Z Rheumatol 2012; 71: 564–71.
16. Westhoff G, Dörner T, Zink A: Fatigue and depression predict physician visits and work disability in women with primary Sjogren’s syndrome: results from a cohort study. Rheumatology (Oxford) 2012; 51:262–9.
17. Brito-Zeron P, Izmirly PM, Ramos-Casals M, Buyon JP, Khamashta MA: The clinical spectrum of autoimmune congenital heart block. Nat Rev Rheumatol 2015; 11: 301–12.
18. Singh AG, Singh S, Matteson EL: Rate, risk factors and causes of mortality in patients with Sjögren’s syndrome: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford) 2016; 55:450–60.
19. Moutsopoulos HM: Sjögren’s Syndrome: autoimmune epithelitis. Clinical Immunology and Immunopathology 1994; 72:162–5.
20. Shiboski CH, Shiboski SC, Seror R, et al.: 2016 American College of Rheuma tology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome: a consensus and datadriven methodology involving three international patient cohorts. Arthritis Rheumatol.2017; 69:35–45.
21. Cornec D, Devauchelle-Pensec V, Saraux A, Jousse-Joulin S: Clinical usefulness of salivary gland ultrasonography in Sjögren’s syndrome: where are we now? Rev Med Interne. 2016; 37: 186– 94.
22. Nardi N, Brito-Zeron P, Ramos-Casals M, et al.: Circulating autoantibodies against nuclear and non-nuclear antigens in primary Sjögren’s syndrome: prevalence and clinical significance in 335 patients. Clin Rheumatol 2006; 25:341–6.
23. Pisetsky DS: Antinuclear antibodies in healthy people: the tip of autoimmunity’s iceberg? Arthritis Res Ther 2011; 13:109.
24. Patel R, Shahane A: The epidemiology of Sjögren’s syndrome. Clin Epidemiol 2014; 6:247–55.
25. Theander E, Jonsson R, Sjostrom B, Brokstad K, Olsson P, Henriksson G: Prediction of Sjögren’s syndrome years before diagnosis and identification of patients with early onset and severe disease course by autoantibody profiling. Arthritis Rheumatol 2015; 67:2427–36.
26. Gottenberg JE, Seror R, Miceli-Richard C, et al.: Serum levels of beta2-microglobulin and free light chains of immunoglobulins are associated with systemic disease activity in primary Sjögren’s syndrome. Data at enrollment in the prospective ASSESS cohort. PLoS One 2013; 8: e59868.
27. Rose T, Szelinski F, Lisney A, et al.: SIGLEC1 is a biomarker of disease activity and indicates extraglandular manifestation in primary Sjögren’s syndrome. RMD Open 2016; 2:e000292.
28. Daniels TE, Cox D, Shiboski CH, et al.: Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjögren’s syndrome among 1,726 registry participants. Arthritis Rheum 2011; 63:2021–30.
29. Seror R, Bowman SJ, Brito-Zeron P, et al.: EULAR Sjögren’s syndrome disease activity index (ESSDAI): a user guide. RMD Open 2015; 1: e000022.
30. Saraux A, Pers JO, Devauchelle-Pensec V: Treatment of primary Sjögren syndrome. Nat Rev Rheumatol 2016; 12:456–71.
31. Leonardi A, Van Setten G, Amrane M, et al.: Efficacy and safety of 0.1% cyclosporine A cationic emulsion in the treatment of severe dry eye disease: a multicenter randomized trial. Eur J Ophthalmol 2016; 26:287–96.
32. Romero-Rangel T, Stavrou P, Cotter J, Rosenthal P, Baltatzis S, Foster CS: Gas-permeable scleral contact lens therapy in ocular surface disease. Am J Ophthalmol 2000; 130: 25–32.
33. Furness S, Worthington HV, Bryan G, Birchenough S, Mc Millan R: Interventions for the management of dry mouth: topical therapies. Cochrane Database Syst Rev 2011; (12): CD008934.
34. Furness S, Bryan G, McMillan R, Worthington HV: Interventions for the management of dry mouth: non-pharmacological interventions. Cochrane Database Syst Rev 2013; (8): CD009603.
35. Albrecht K, Callhoff J, Westhoff G, Dietrich T, Dorner T, Zink A: The prevalence of dental implants and related factors in patients with Sjogren syndrome: results from a cohort study. J Rheumatol 2016; 43: 1380–5.
36. Carsons SE, Vivino FB, Parke A, et al.: Treatment guidelines for rheumatologic manifestations of Sjogren’s: use of biologics, management of fatigue and inflammatory musculoskeletal pain. Arthritis Care Res (Hoboken) 2016.
37. Gottenberg JE, Ravaud P, Puechal X, et al.: Effects of hydroxychloroquine on symptomatic improvement in primary Sjögren syndrome: the JOQUER randomized clinical trial. JAMA 2014; 312: 249–58.
38. Dass S, Bowman SJ, Vital EM, et al.: Reduction of fatigue in Sjögren’s syndrome with rituximab: results of a randomised, double-blind, placebo controlled pilot study. Ann Rheum Dis 2008; 67:1541–4.
39. Bowman S, Everett C, Bombardieri M, et al.: Preliminary results of a double-blind randomised trial of rituximab anti-B-cell therapy in patients with primary Sjogrens syndrome. Arthritis Rheumatol 2015; 67:3955–7.
40. Izmirly PM, Kim MY, Llanos C, et al.: Evaluation of the risk of anti- SSA/Ro-SSB/La antibody- associated cardiac manifestations of neonatal lupus in fetuses of mothers with systemic lupus erythematosus exposed to hydroxychloroquine. Ann Rheum Dis 2010; 6910: 1827–30.
41. Wallace DJ. The New Sjögren’s Syndrome Handbook. Oxford, UK: Oxford University Press; 2005; 24-32.
42. National Institute of Arthritis and Musculoskeletal and Skin Diseases website. www.niams.nih.gov. Accessed December. 2013.
43. Mengshoel AM, Norheim KB, Omdal R. Primary Sjögren’s syndrome: fatigue is an ever- present, fluctuating and uncontrollable lack of energy.2013; 10.
44. Vivino FB, Al-Hashimi I, Khan Z, et al. Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjögren syndrome: a randomized, placebo-controlled, fixed- dose, multicenter trial. P92-01 Study Group. Arch Intern Med. 1999; 159:174-181.
Published
2019-10-15
How to Cite
Pittala, V., Mallannagari, S., Sherla, H., & Kadarla, R. K. (2019). Primary Sjögren’s Syndrome - A Review. Asian Journal of Pharmaceutical Research and Development, 7(5), 113–123. https://doi.org/10.22270/ajprd.v7i5.568
Issue
Section
Review Articles
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