Síndrome de Sjögren

  1. Pijoan Moratalla, C.M. 1
  2. Blanco Cáceres, B.A. 1
  3. Revenga Martínez, M. 2
  4. Loarce Martos, J. 1
  5. Morell Hita, J.L. 1
  1. 1 Servicio de Reumatología. Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS). Hospital Universitario Ramón y Cajal, Madrid, España
  2. 2 Servicio de Reumatología. Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS). Hospital Universitario Ramón y Cajal, Madrid, España Departamento de Medicina y Especialidades Médicas. Universidad de Alcalá. Alcalá de Henares, Madrid, España
Revista:
Medicine: Programa de Formación Médica Continuada Acreditado

ISSN: 0304-5412

Año de publicación: 2021

Serie: 13

Número: 30

Páginas: 1694-1701

Tipo: Artículo

DOI: 10.1016/J.MED.2021.03.029 DIALNET GOOGLE SCHOLAR

Otras publicaciones en: Medicine: Programa de Formación Médica Continuada Acreditado

Resumen

El síndrome de Sjögren (SS) es una enfermedad crónica autoinmune caracterizada por la infiltración y destrucción progresiva de las glándulas exocrinas. Puede aparecer de forma primaria o asociada a otras patologías autoinmunes. Clínicamente se caracteriza por xeroftalmia y xerostomía intensas, pero también pueden desarrollarse diferentes síntomas extraglandulares, siendo los más habituales la afectación articular, pulmonar, renal o neurológica que generalmente condicionan tanto el tratamiento como el pronóstico. El diagnóstico incluye la confirmación de la hiposecreción glandular mediante diferentes técnicas y la presencia de autoanticuerpos anti-Ro/SSA o anti-La/SSB. El tratamiento se basa en el manejo sintomático de la sequedad y la prevención de sus potenciales complicaciones y, en segundo lugar, en fármacos inmunosupresores y tratamientos biológicos para el control de las manifestaciones extraglandulares que son las que confieren un peor pronóstico a largo plazo.

Referencias bibliográficas

  • Qin B, Wang J, Yang Z, Yang M, Ma N, Huang F, Epidemiology of primary Sjögren’s syndrome: a systematic review and meta analysis. Ann Rheum Dis. 2015;74(11):1983-1989.
  • Nocturne G, Mariette X. Advances in understanding the pathogenesis of primary Sjögren’s syndrome. Nat Rev Rheumatol. 2013;9(9):544-556.
  • Huang Y, Cheng Q, Jiang C, An S, Xiao L, Gou Y, The immune factors involved in the pathogenesis, diagnosis, and treatment of Sjogren’s syndrome. Clin Dev Immunol. 2013:160491.
  • Kramer JM. Early events in Sjögren’s syndrome pathogenesis: the importance of innate immunity in disease initiation. Cytokine. 2014;67(2):92-101.
  • Taylor KE, Wong Q, Levine DM, McHugh C, Laurie C, Doheny K, Genome Wide Association Analysis Reveals Genetic Heterogeneity of Sjögren’s syndrome according to ancestry. Arthritis Rheumatol. 2017; 69(6):1294-1305.
  • Bolstad AI, Le Hellard S, Kristjansdottir G, Vasaitis L, Kvarnström M, Sjöwall C, Association between genetic variants in the tumour necrosis factor/lymphotoxin _/lymphotoxin ` locus and primary Sjogren’s syndrome in Scandinavian samples. Ann Rheum Dis. 2012;71(6):981-988.
  • Nakamura H, Kawakami A. What is the evidence for Sjögren’s syndrome being triggered by viral infection? Subplot: infections that cause clinical features of Sjögren’s syndrome. Curr Opin Rheumatol. 2016;28(4):390-397.
  • Triantafyllopoulou A, Moutsopoulos H. Persistent viral infection in primary Sjogren’s syndrome: review and perspectives. Clin Rev Allergy Immunol. 2007;32(3):210-214.
  • Jonsson R, Theander E, Sjöström B, Brokstad K, Henriksson G. Autoantibodies present before symptom onset in primary Sjögren syndrome. JAMA. 2013;310(17):1854-1855.
  • Ittah M, Miceli Richard C, Eric Gottenberg J, Lavie F, Lazure T, Ba N, B cell activating factor of the tumor necrosis factor family (BAFF) is expressed under stimulation by interferon in salivary gland epithelial cells in primary Sjögren’s syndrome. Arthritis Res Ther. 2006;8(2):R51.
  • Ittah M, Miceli Richard C, Gottenberg JE, Sellam J, Eid P, Lebon P, Viruses induce high expression of BAFF by salivary gland epithelial cells through TLR and type-I IFN dependent and independent pathways. Eur J Immunol. 2008;38(4):1058-1064.
  • Hjelmervik TOR, Petersen K, Jonassen I, Jonsson R, Bolstad AI. Gene expression profiling of minor salivary glands clearly distinguishes primary Sjögren’s syndrome patients from healthy control subjects. Arthritis Rheum. 2005;52(5):1534-1544.
  • Ramos Casals M, Solans R, Rosas J, Camps MT, Gil A, Del Pino Montes J, Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore). 2008;87(4):210-219.
  • Ramos Casals M, Brito Zerón P, Seror R, Bootsma H, Bowman SJ, Dörner T, Characterization of systemic disease in primary Sjögren’s syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements. Rheumatology (Oxford). 2015;54(12):2230-2238.
  • Afonso AA, Monroy D, Stern ME, Feuer WJ, Tseng SC, Pflugfelder SC. Correlation of tear fluorescein clearance and Schirmer test scores with ocular irritation symptoms. Ophthalmology. 1999;106(4):803-810.
  • Lee M, Rutka JA, Slomovic AR, McComb J, Bailey DJ, Bookman AA. Establishing guidelines for the role of minor salivary gland biopsy in clinical practice for Sjögren’s syndrome. J Rheumatol. 1998;25(2):247-253.
  • Menéndez A, Gómez J, Escanlar E, Caminal Montero L, Mozo L. Clinical associations of anti SSA/Ro60 and anti Ro52/TRIM21 antibodies: Diagnostic utility of their separate detection. Autoimmunity. 2013;46(1):32-39.
  • Jousse Joulin S, Nowak E, Cornec D, Brown J, Carr A, Carotti M, Salivary gland ultrasound abnormalities in primary Sjögren’s syndrome: Consensual US-SG core items definition and reliability. RMD open. 2017;3(1):e000364.
  • Mossel E, Delli K, Nimwegen JF van, Stel AJ, Kroese FGM, Spijkervet FKL, Ultrasonography of major salivary glands compared with parotid and labial gland biopsy and classification criteria in patients with clinically suspected primary Sjogren’s syndrome. Ann Rheum Dis. 2017; 76(11):1883-1889.
  • Niemelä RK, Takalo R, Pääkkö E, Suramo I, Päivänsalo M, Salo T, Ultrasonography of salivary glands in primary Sjogren’s syndrome. A comparison with magnetic resonance imaging and magnetic resonance sialography of parotid glands. Rheumatology (Oxford). 2004;43(7):875-879.
  • Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, 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. Arthritis Rheumatol. 2017;69(1):35-45.
  • Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LTH. Lymphoma and other malignancies in primary Sjögren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis. 2006;65(6):796-803.
  • Ahn JK, Hwang J, Seo GH. Risk of non Hodgkin’s lymphoma and thyroid cancer in primary Sjögren’s syndrome measured using the Korean Health Insurance Claims Database. Clin Exp Rheumatol. 2020;38 Suppl 126(4):40-46.
  • Baimpa E, Dahabreh IJ, Voulgarelis M, Moutsopoulos HM. Hematologic manifestations and predictors of lymphoma development in primary Sjögren syndrome: clinical and pathophysiologic aspects. Medicine (Baltimore). 2009;88(5):284-293.
  • Significance of anti-La/SSB antibodies in primary Sjögren’s syndrome patients with combined positivity for anti-Ro/SSA and salivary gland biopsy [Internet]. Clin Exp Rheumatol. 2020.
  • Ramos Casals M, Brito-Zerón P, Bombardieri S, Bootsma H, Vita SD, Dörner T, EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies. Ann Rheum Dis. 2020;79(1):3-18.
  • Chen S, Wang Y, Chen S, Wu Q, Chen S. Virtual touch quantification of the salivary glands for diagnosis of primary Sjögren syndrome. J Ultrasound Med. 2016;35(12):2607-2613.
  • Papas AS, Sherrer YS, Charney M, Golden HE, Medsger TA, Walsh BT, Successful treatment of dry mouth and dry eye symptoms in Sjögren’s syndrome patients with oral pilocarpine: a randomized, placebo controlled, dose adjustment study. J Clin Rheumatol. 2004;10(4): 169-177.
  • Aragona P, Stilo A, Ferreri F, Mobrici M. Effects of the topical treatment with NSAIDs on corneal sensitivity and ocular surface of Sjögren’s syndrome patients. Eye (Lond). 2005;19(5):535-539.
  • Hong S, Kim T, Chung SH, Kim EK, Seo KY. Recurrence after topical nonpreserved methylprednisolone therapy for keratoconjunctivitis Sicca in Sjögren’s syndrome. J Ocul Pharmacol Ther. 2007;23(1):78-82.
  • Yavuz S, Asfuro’lu E, Bicakcigil M, Toker E. Hydroxychloroquine improves dry eye symptoms of patients with primary Sjogren’s syndrome. Rheumatol Int. 2011;31(8):1045-1049.
  • van Woerkom JM, Kruize AA, Geenen R, van Roon EN, Goldschmeding R, Verstappen SMM, Safety and efficacy of leflunomide in primary Sjögren’s syndrome: a phase II pilot study. Ann Rheum Dis. 2007;66(8): 1026-1032.
  • Kim EC, Choi JS, Joo CK. A comparison of vitamin a and cyclosporine a 0.05% eye drops for treatment of dry eye syndrome. Am J Ophthalmol. 2009;147(2):206-213.e3.
  • Devauchelle Pensec V, Mariette X, Jousse Joulin S, Berthelot J-M, Perdriger A, Puéchal X, Treatment of primary Sjögren syndrome with rituximab: a randomized trial. Ann Intern Med. 2014;160(4):233-242.
  • Meiners PM, Vissink A, Kroese FGM, Spijkervet FKL, Smitt Kamminga NS, Abdulahad WH, Abatacept treatment reduces disease activity in early primary Sjögren’s syndrome (open label proof of concept ASAP study). Ann Rheum Dis. 2014;73(7):1393-1396.
  • Mariette X, Seror R, Quartuccio L, Baron G, Salvin S, Fabris M, Efficacy and safety of belimumab in primary Sjögren’s syndrome: results of the BELISS open label phase II study. Ann Rheum Dis. 2015;74(3): 526-531.
  • Andréu Sánchez JL, Fernández Castro M, Del Campo Fontecha PD, Corominas H, Narváez García FJ, Gómez de Salazar JR, SER recommendations on the use of biological drugs in primary Sjögren’s syndrome. Reumatol Clin. 2019;15(6):315-326.
  • Albrecht K, Dörner T, Redeker I, Karberg K, Marschall U, Zink A, Comorbidity and health care utilisation in persons with Sjögren’s syndrome: a claims data analysis. Clin Exp Rheumatol. 2020;38 Suppl 126(4): 78-84.
  • Seror R, Bowman SJ, Brito Zeron P, Theander E, Bootsma H, Tzioufas A, EULAR Sjögren’s syndrome disease activity index (ESSDAI): a user guide. RMD Open. 2015;1(1):e000022.
  • Nishishinya MB, Pereda CA, Muñoz Fernández S, Pego Reigosa JM, Rúa Figueroa I, Andreu JL, Identification of lymphoma predictors in patients with primary Sjögren’s syndrome: a systematic literature review and meta analysis. Rheumatol Int. 2015;35(1):17-26
Fuente de los datos: Dialnet