Overlap Syndrome Between Sjögren's Syndrome and Systemic Sclerosis
DOI:
https://doi.org/10.5195/ijms.2023.2393Keywords:
Sjögren's Syndrome, Systemic Sclerosis, Overlap Syndrome, AutoimmunityAbstract
BACKGROUND: Overlap syndrome is a condition in which a patient experiences symptoms that correspond to two or more of these classification standards. More commonly, the overlap is limited to one or more manifestations of each disease. Sjögren's syndrome is a systemic autoimmune disease characterized by the presence of a dry syndrome, primarily xerostomia and xerophthalmia, originating from dysfunction of exocrine glands. Conversely, systemic sclerosis is a rare autoimmune disease characterized by fibrosis development in the skin, internal organs, and vasculopathies.
THE CASE: Our case report presents a 62-year-old woman with a history of Sjögren's syndrome, who exhibited symptoms of xerostomia, xerophthalmia, xerodermia, and had anti-Ro antibodies exceeding 200, along with ultrasound findings of chronic sialadenitis. The patient was under treatment with hydroxychloroquine, pilocarpine, and deflazacort. She attended the rheumatology outpatient clinic for routine follow-up of her disease. During the evaluation, bilateral sclerodactyly and "salt and pepper" lesions on the limbs were observed, along with two reports of ANA with titers of 1:2650 and a centromere pattern. Due to suspicion of coexistence of limited systemic sclerosis, anticentromere antibodies were requested, yielding a positive result, while anti-Scl 70 antibodies were negative. This confirmed the diagnostic suspicion, and azathioprine 50 mg every 12 hours and colchicine 0.5 mg daily were added to her therapy.
CONCLUSION: Although Sjögren's syndrome and systemic sclerosis can manifest independently, in certain instances, both conditions overlap. It is worth noting that among the various overlap syndromes related to connective tissue diseases, this overlap is infrequent. However, within connective tissue diseases, systemic sclerosis tends to exhibit overlap more frequently, reported in up to 20-30% of cases. In cases of connective tissue diseases, consideration should always be given to overlap syndromes, involving a meticulous physical examination and careful assessment of laboratory results to avoid overlooking conditions that may negatively impact patients' future quality of life.
Metrics
References
Maddison PJ. Mixed connective tissue disease, overlap syndromes, and eosinophilic fasciitis. Ann Rheum Dis [Internet]. 1991 [cited 2023 Sep 1];50 Suppl 4(Suppl 4):887–93. Available from: https://pubmed.ncbi.nlm.nih.gov/1836323/
Iaccarino L, Gatto M, Bettio S, Caso F, Rampudda M, Zen M, et al. Overlap connective tissue disease syndromes. Autoimmun Rev [Internet]. 2013 Jan [cited 2023 Sep 1];12(3):363–73. Available from: https://pubmed.ncbi.nlm.nih.gov/22743033/
Wicheta S, Van der Groen T, Faquin WC, August M. Minor Salivary Gland Biopsy—An Important Contributor to the Diagnosis of Sjögren Syndrome. Journal of Oral and Maxillofacial Surgery. 2017 Dec 1;75(12):2573–8.
Mossel E, Delli K, Van Nimwegen JF, Stel AJ, Kroese FGM, Spijkervet FKL, et al. Ultrasonography of major salivary glands compared with parotid and labial gland biopsy and classification criteria in patients with clinically suspected primary Sjögren’s syndrome. Ann Rheum Dis [Internet]. 2017 Nov 1 [cited 2023 Sep 1];76(11):1883–9. Available from: https://ard.bmj.com/content/76/11/1883
Tincani A, Andreoli L, Cavazzana I, Doria A, Favero M, Fenini MG, et al. Novel aspects of Sjögren’s syndrome in 2012. BMC Med [Internet]. 2013 Apr 4 [cited 2023 Sep 1];11(1). Available from: https://pubmed.ncbi.nlm.nih.gov/23556533/
Fernández-Ávila DG, Rincón-Riaño DN, Bernal-Macías S, Gutiérrez Dávila JM, Rosselli D. Prevalencia y características demográficas del síndrome de Sjögren en Colombia, según información del Sistema Integral de Información de la Protección Social. Reumatol Clin [Internet]. 2020 Jul 1 [cited 2023 Sep 1];16(4):286–9. Available from: http://www.reumatologiaclinica.org/es-prevalencia-caracteristicas-demograficas-del-sindrome- articulo-S1699258X18302195
Vivino FB. Sjogren’s syndrome: Clinical aspects. Clin Immunol [Internet]. 2017 Sep 1 [cited 2023 Sep 1];182:48–54. Available from: https://pubmed.ncbi.nlm.nih.gov/28428095/
Romão VC, Talarico R, Scirè CA, Vieira A, Alexander T, Baldini C, et al. Sjögren’s syndrome: state of the art on clinical practice guidelines. RMD Open [Internet]. 2018 Oct 1 [cited 2023 Sep 1];4(Suppl 1):e000789. Available from: https://rmdopen.bmj.com/content/4/Suppl_1/e000789
Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A, Bosch X. Primary Sjogren syndrome. BMJ [Internet]. 2012 Sep 1 [cited 2023 Sep 1];344(7872). Available from: https://pubmed.ncbi.nlm.nih.gov/22700787/
Rozo-Ortiz EJ, Vargas-Rodríguez LJ, Ruiz-Muñoz JL, Medina-Nieto SE, Morales-Ríos G, Sandoval- Laguado DV, et al. Sindrome de Sjögren: una revisión de la literatura. Medicas UIS [Internet]. 2022 Dec 24 [cited 2023 Sep 1];35(3):9–17. Available from: http://www.scielo.org.co/scielo.php?script=sci_arttext&pid=S0121- 03192022000300009&lng=en&nrm=iso&tlng=es
Graña D, Vargas A, Bérez A, Goñi M, Danza A. Esclerosis sistémica: forma de presentación y manejo terapéutico: experiencia de un grupo de trabajo en Enfermedades Autoinmunes Sistémicas. Revista Uruguaya de Medicina Interna [Internet]. 2018 Mar 17 [cited 2023 Sep 1];3(1):15–22. Available from: http://www.scielo.edu.uy/scielo.php?script=sci_arttext&pid=S2393- 67972018000100015&lng=es&nrm=iso&tlng=es
Castañeda NG, Rubio ER, Emperiale V, Hajkhan AM. Esclerosis sistémica. Medicine - Programa de Formación Médica Continuada Acreditado. 2021 Apr 1;13(31):1769–78.
Klein-Weigel P, Opitz C, Riemekasten G. Systemic sclerosis - a systematic overview: part 1 - disease characteristics and classification, pathophysiologic concepts, and recommendations for diagnosis and surveillance. Vasa [Internet]. 2011 Jan [cited 2023 Sep 1];40(1):6–19. Available
from: https://pubmed.ncbi.nlm.nih.gov/21283969/
Robert M, Mouthon L. Esclerodermia o esclerosis sistémica. EMC - Tratado de Medicina. 2022 Aug 1;26(3):1–6.
Fernández-Ávila DG, Bernal-Macías S, Gutiérrez JM, Rincón DN, Rosselli D. Prevalence of systemic sclerosis in Colombia: Data from the National Health Registry 2012-2016. J Scleroderma Relat Disord [Internet]. 2020 [cited 2023 Sep 1];5(2). Available from: https://pubmed.ncbi.nlm.nih.gov/35382022/
Jaramillo Salazar JC, Bonilla Poma WC, Borja Guzmán ME, Macias Sánchez YE. Esclerosis Sistémica. RECIMUNDO. 2021 Dec 10;5(1):91–100.
Jury EC, D’Cruz D, Morrow WJW. Autoantibodies and overlap syndromes in autoimmune rheumatic disease. J Clin Pathol [Internet]. 2001 [cited 2023 Sep 1];54(5):340–7. Available from: https://pubmed.ncbi.nlm.nih.gov/11328831/
Turkcapar N, Olmez U, Ozer D, Duzgun N, Duman M. A case of overlap syndrome with rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and secondary sjögren’s syndrome. Rheumatol Int. 2006 Jul 22;26(9):841–5.
Sarmiento-Monroy JC, Gómez-Puerta JA. Poliautoinmunidad en síndrome de Sjögren. Revista Colombiana de Reumatología. 2020 Oct;27:58–66.
Avouac J, Sordet C, Depinay C, Ardizonne M, Vacher-Lavenu MC, Sibilia J, et al. Systemic sclerosis–associated Sjögren’s syndrome and relationship to the limited cutaneous subtype: Results of a prospective study of sicca syndrome in 133 consecutive patients. Arthritis Rheum. 2006 Jul;54(7):2243–9.
Salliot C, Gottenberg JE, Bengoufa D, Desmoulins F, Miceli-Richard C, Mariette X. Anticentromere antibodies identify patients with Sjögren’s syndrome and autoimmune overlap syndrome. J Rheumatol. 2007 Nov;34(11):2253–8.
Hunzelmann N, Genth E, Krieg T, Lehmacher W, Melchers I, Meurer M, et al. The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology. 2008 Apr 29;47(8):1185–92.
Downloads
Published
How to Cite
Issue
Section
Categories
License
Copyright (c) 2023 Alejandro Londoño Amariles, Bryam Fernando Holguín Muñoz, Lina María Saldarriaga Rivera
This work is licensed under a Creative Commons Attribution 4.0 International License.
Authors who publish with this journal agree to the following terms:
- The Author retains copyright in the Work, where the term “Work” shall include all digital objects that may result in subsequent electronic publication or distribution.
- Upon acceptance of the Work, the author shall grant to the Publisher the right of first publication of the Work.
- The Author shall grant to the Publisher and its agents the nonexclusive perpetual right and license to publish, archive, and make accessible the Work in whole or in part in all forms of media now or hereafter known under a Creative Commons Attribution 4.0 International License or its equivalent, which, for the avoidance of doubt, allows others to copy, distribute, and transmit the Work under the following conditions:
- Attribution—other users must attribute the Work in the manner specified by the author as indicated on the journal Web site; with the understanding that the above condition can be waived with permission from the Author and that where the Work or any of its elements is in the public domain under applicable law, that status is in no way affected by the license.
- The Author is able to enter into separate, additional contractual arrangements for the nonexclusive distribution of the journal's published version of the Work (e.g., post it to an institutional repository or publish it in a book), as long as there is provided in the document an acknowledgment of its initial publication in this journal.
- Authors are permitted and encouraged to post online a prepublication manuscript (but not the Publisher’s final formatted PDF version of the Work) in institutional repositories or on their Websites prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work. Any such posting made before acceptance and publication of the Work shall be updated upon publication to include a reference to the Publisher-assigned DOI (Digital Object Identifier) and a link to the online abstract for the final published Work in the Journal.
- Upon Publisher’s request, the Author agrees to furnish promptly to Publisher, at the Author’s own expense, written evidence of the permissions, licenses, and consents for use of third-party material included within the Work, except as determined by Publisher to be covered by the principles of Fair Use.
- The Author represents and warrants that:
- the Work is the Author’s original work;
- the Author has not transferred, and will not transfer, exclusive rights in the Work to any third party;
- the Work is not pending review or under consideration by another publisher;
- the Work has not previously been published;
- the Work contains no misrepresentation or infringement of the Work or property of other authors or third parties; and
- the Work contains no libel, invasion of privacy, or other unlawful matter.
- The Author agrees to indemnify and hold Publisher harmless from the Author’s breach of the representations and warranties contained in Paragraph 6 above, as well as any claim or proceeding relating to Publisher’s use and publication of any content contained in the Work, including third-party content.
Enforcement of copyright
The IJMS takes the protection of copyright very seriously.
If the IJMS discovers that you have used its copyright materials in contravention of the license above, the IJMS may bring legal proceedings against you seeking reparation and an injunction to stop you using those materials. You could also be ordered to pay legal costs.
If you become aware of any use of the IJMS' copyright materials that contravenes or may contravene the license above, please report this by email to contact@ijms.org
Infringing material
If you become aware of any material on the website that you believe infringes your or any other person's copyright, please report this by email to contact@ijms.org
