Desarrollo de esclerosis sistémica en pacientes con síndrome de Sjögren primario y fenómeno de Raynaud
Palabras clave:
Sjögren primario, fenómeno de Raynaud, capilaroscopia, esclerosis sistémica muy temprana
Resumen
Introducción: una manifestación extraglandular de prevalencia variable, pero frecuente del Sjögren primario (SSp) es el fenómeno de Raynaud (FR), el cual es una constante de la esclerosis sistémica (ES). Objetivos: evaluar la presencia del FR en pacientes con SSp; describir las características clínicas e inmunológicas; determinar el desarrollo de ES en los pacientes con SSp y FR. Materiales y métodos: se incluyeron pacientes con SSp y se registraron aquellos que presentaron FR durante el seguimiento. La esclerosis sistémica muy temprana (ESMT) se definió como FR asociado con autoanticuerpos marcadores de ES y/o patrón esclerodermiforme (SDP) en capilaroscopia. Resultados: de 207 pacientes con diagnóstico de SSp, solo 32 (15,5%) presentaron FR, y de ellos 18 cumplieron criterios para ESMT. De los que cumplieron criterios para ESMT, 5 desarrollaron ES limitada con anticentrómero (ACA) positivo. Conclusiones: encontramos una frecuencia de FR en pacientes con SSp similar a la descripta en diversas series. Más del 50% de los pacientes con FR y SSp de esta cohorte cumplió criterios para ESMT, evolucionando a ES 5 de ellos con una mediana de seguimiento de 62 meses siendo ACA positivos.Citas
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XV. Salliot C, Mouthon L, Ardizzone M, et al. Sjogren's syndrome is associated with and not secondary to systemic sclerosis. Rheumatology (Oxford). 2007 Feb;46(2):321-6.
XVI. Valentini G, Marcoccia A, Cuomo G, et al. Early systemic sclerosis: marker autoantibodies and videocapillaroscopy patterns are each associated with distinct clinical, functional and cellular activation markers. Arthritis Res Ther. 2013;15(3):R63.
II. Salliot C, Mouthon L, Ardizzone M, et al. Sjogren's syndrome is associated with and not secondary to systemic sclerosis. Rheumatology (Oxford). 2007 Feb;46(2):321-6.
III. Maslinska M, Kostyra-Grabczak K. The role of virus infections in Sjögren's syndrome. Front Immunol. 2022 Sep 6;13:823659.
IV. Tzioufas AG, Voulgarelis M. Update on Sjögren's syndrome autoimmune epithelitis: from classification to increased neoplasias. Best Pract Res Clin Rheumatol. 2007 Dec;21(6):989-1010.
V. García-Carrasco M, Sisó A, Ramos-Casals M, et al. Raynaud's phenomenon in primary Sjögren's syndrome. Prevalence and clinical characteristics in a series of 320 patients. J Rheumatol. 2002 Apr;29(4):726-30.
VI. Tektonidou M, Kaskani E, Skopouli FN, Moutsopoulos HM. Microvascular abnormalities in Sjögren’s syndrome: nailfold capillaroscopy. Rheumatology. 1999;38(826):30
VII. Bellando-Randone S, Matucci-Cerinic M. Very early systemic sclerosis and pre-systemic sclerosis. Definition, recognition, clinical relevance and future directions. Curr Rheumatol Rep. 2017 Sep 18;19(10):65.
VIII. Tsukamoto M, Suzuki K, Takeuchi T. Clinical and immunological features of anti-centromere antibody-positive primary Sjögren's syndrome. Rheumatol Ther. 2018 Dec;5(2):499-505.
IX. Pauling JD, Hughes M, Pope JE. Raynaud's phenomenon-an update on diagnosis, classification and management. Clin Rheumatol. 2019 Dec;38(12):3317-3330.
X. Vitali C, Bombardieri S, Jonsson R, et al. European Study Group on Classification Criteria for Sjögren's Syndrome. Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002 Jun;61(6):554-8.
XI. Shiboski CH, Shiboski SC, Seror R, et al. International Sjögren's Syndrome Criteria Working Group. 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 Jan;69(1):35-45.
XII. van den Hoogen F, Khanna D, Fransen J, et al. 2013 Classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55.
XIII. Shiboski SC, Shiboski CH, Criswell L, et al. Sjögren's International Collaborative Clinical Alliance (SICCA) Research Groups. American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort. Arthritis Care Res (Hoboken). 2012 Apr;64(4):475-87.
XIV. Liu Y, Guo L, Lin W, et al. Anticentromere antibody positive patients with primary Sjögren's syndrome have distinctive clinical and immunological characteristics. Clin Exp Rheumatol. 2023 Dec;41(12):2371-2378.
XV. Salliot C, Mouthon L, Ardizzone M, et al. Sjogren's syndrome is associated with and not secondary to systemic sclerosis. Rheumatology (Oxford). 2007 Feb;46(2):321-6.
XVI. Valentini G, Marcoccia A, Cuomo G, et al. Early systemic sclerosis: marker autoantibodies and videocapillaroscopy patterns are each associated with distinct clinical, functional and cellular activation markers. Arthritis Res Ther. 2013;15(3):R63.
Publicado
2025-06-01
Cómo citar
1.
Perdomo AM, Morbiducci J, Arguissain C, Bejarano M, Tamborenea MN, Secco A. Desarrollo de esclerosis sistémica en pacientes con síndrome de Sjögren primario y fenómeno de Raynaud. Rev. Argent. Reumatol. [Internet]. 1 de junio de 2025 [citado 27 de octubre de 2025];36(2). Disponible en: https://ojs.reumatologia.org.ar/index.php/revistaSAR/article/view/959
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