Official scientific distribution organ of Instituto Panvascular de Occidente
Anterior Necrotizing Scleritis as Sjögren's Syndrome Presentation: Case Report and Literature Review
PDF Reporte de Caso (Spanish)

Keywords

Scleritis
Sjögren's Syndrome
mmunosuppression Therapy

Categories

How to Cite

1.
Anterior Necrotizing Scleritis as Sjögren’s Syndrome Presentation: Case Report and Literature Review. Rev Med Clin [Internet]. 2023 Jun. 1 [cited 2024 Nov. 14];8(1):e01062408002. Available from: https://mail.medicinaclinica.org/index.php/rmc/article/view/567

Abstract

Sjögren's syndrome is a multisystem autoimmune disease that affects the salivary and tear glands, causing dry mouth and eye symptoms. In addition to classic symptoms, it can cause extraglandular ocular manifestations that can be life-threatening but may initially go unnoticed. A clinical case of anterior necrotizing scleritis is described in a patient with ocular symptoms of seven months' duration and poor response to topical therapy, in which infectious and autoimmune diseases more related to this entity were ruled out, finally reaching the diagnosis of syndrome of anterior necrotizing scleritis. Sjögren. The patient responded favorably to immunomodulatory treatment with complete remission of symptoms. The association between scleritis and Sjögren's syndrome is rare in the literature and its prognosis is unknown. This case highlights the importance of recognizing extraglandular ocular manifestations that may be the initial manifestation in this pathology.

PDF Reporte de Caso (Spanish)

References

Jan RL, Ho CH, Wang JJ, Tseng SH, Chang YS. Associations between Sjögren Syndrome, Sociodemographic Factors, Comorbid Conditions, and Scleritis in a Taiwanese Population-Based Study. J Pers Med. 2022 Jan 1;12(1). Doi: 10.3390/jpm12010105.

Pavesio CE, Meier FM. Systemic disorders associated with episcleritis and scleritis. Curr Opin Ophthalmol [Internet]. 2001 [cited 2024 Jan 27];12(6):471–8. Available from: https://pubmed.ncbi.nlm.nih.gov/11734688/. Doi: 10.1097/00055735-200112000-00013.

Honik G, Wong IG, Gritz DC. Incidence and prevalence of episcleritis and scleritis in Northern California. Cornea [Internet]. 2013 Dec [cited 2024 Jan 27];32(12):1562–6. Available from: https://pubmed.ncbi.nlm.nih.gov/24145628/. Doi: 10.1097/ICO.0B013E3182A407C3.

Xu TT, Reynolds MM, Hodge DO, Smith WM. Epidemiology and Clinical Characteristics of Episcleritis and Scleritis in Olmsted County, Minnesota. Am J Ophthalmol [Internet]. 2020 Sep 1 [cited 2024 Jan 27];217:317–24. Available from: https://pubmed.ncbi.nlm.nih.gov/32437669/. Doi: 10.1016/J.AJO.2020.04.043.

Berkenstock MK, Carey AR. Health system wide “big data” analysis of rheumatologic conditions and scleritis. BMC Ophthalmol. 2021 Dec 1;21(1). Doi: 10.1186/s12886-020-01769-3.

Smith JR, Mackensen F, Rosenbaum JT. Therapy Insight: Scleritis and its relationship to systemic autoimmune disease. Vol. 3, Nature Clinical Practice Rheumatology. 2007. p. 219–26. Doi: 10.1038/ncprheum0454.

Foster CS, Forstot SL, Wilson LA. Mortality rate in rheumatoid arthritis patients developing necrotizing scleritis or peripheral ulcerative keratitis. Effects of systemic immunosuppression. Ophthalmology [Internet]. 1984 [cited 2024 Jan 27];91(10):1253–63. Available from: https://pubmed.ncbi.nlm.nih.gov/6514289/. Doi: 10.1016/S0161-6420(84)34160-4.

Murthy SI, Sabhapandit S, Balamurugan S, Subramaniam P, Sainz-De-La-Maza M, Agarwal M, et al. Scleritis: Differentiating infectious from non-infectious entities. Indian J Ophthalmol [Internet]. 2020 Sep 1 [cited 2023 Dec 27];68(9):1818–28. Available from: https://pubmed.ncbi.nlm.nih.gov/32823398/. Doi: 10.4103/IJO.IJO_2032_20.

Arruza C, Requejo Figueroa GA, Colón J, Ramirez Marquez E, Puebla G, Ortega D, et al. Systemic Disease Associations in a Cohort of Hispanic Patients with Scleritis. J Clin Med. 2023 Mar 1;12(5). Doi: 10.3390/jcm12051969.

Nevares A, Raut R, Libman B, Hajj-Ali R. Noninfectious Autoimmune Scleritis: Recognition, Systemic Associations, and Therapy. Vol. 22, Current Rheumatology Reports. Springer; 2020. Doi: 10.1007/s11926-020-0885-y.

de la Maza MS, Foster CS, Jabbur NS. Scleritis Associated with Systemic Vasculitic Diseases. Ophthalmology. 1995;102(4):687–92. Doi: 10.1016/S0161-6420(95)30970-0.

Akpek EK, Bunya VY, Saldanha IJ. Sjögren’s Syndrome: More Than Just Dry Eye. Vol. 38, Cornea. Lippincott Williams and Wilkins; 2019. p. 658–61. Doi: 10.1097/ICO.0000000000001865.

de-la-Torre A, Cabrera-Pérez M, Durán C, García S, Cuevas M, Carreño N, et al. Clinical patterns and risk factors in scleritis: a multicentric study in Colombia. Graefe’s Archive for Clinical and Experimental Ophthalmology. 2022 Dec 1;260(12):3957–67. Doi: 10.1007/s00417-022-05754-y.

Vergouwen DPC, Kolijn PM, de Hoog J, de Boer JH, Los LI, Gijs M, et al. Potential Biomarkers for Noninfectious Scleritis Identified by Serum and Tear Fluid Proteomics. Ophthalmology Science [Internet]. 2024 Jan 1 [cited 2024 Jan 26];4(1):100407. Available from: /pmc/articles/PMC10694766/. Doi: 10.1016/J.XOPS.2023.100407.

Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, et al. Sjögren syndrome. Nat Rev Dis Primers. 2016 Jul 7;2. Doi: 10.1038/nrdp.2016.47.

Vergouwen DPC, Ten Berge JC, Boukhrissi S, Rothova A, Schreurs MWJ. Clinical Relevance of Autoantibodies and Inflammatory Parameters in Non-infectious Scleritis. Ocul Immunol Inflamm [Internet]. 2022 [cited 2024 Jan 27];30(7–8):1859–65. Available from: https://pubmed.ncbi.nlm.nih.gov/34464233/. Doi: 10.1080/09273948.2021.1966050.

Fox RI. Sjögren’s syndrome. Lancet [Internet]. 2005 Jul 23 [cited 2024 Jan 27];366(9482):321–31. Available from: https://pubmed.ncbi.nlm.nih.gov/16039337/. Doi: 10.1016/S0140-6736(05)66990-5.

Mathews PM, Robinson SA, Gire A, Baer AN, Akpek EK. Extraglandular ocular involvement and morbidity and mortality in primary Sjögren’s Syndrome. PLoS One. 2020 Sep 1;15(9 September). Doi: 10.1371/journal.pone.0239769.

Akpek EK, Mathews P, Hahn S, Hessen M, Kim J, Grader-Beck T, et al. Ocular and systemic morbidity in a longitudinal cohort of Sjögren’s syndrome. Ophthalmology. 2015 Jan 1;122(1):56–61. Doi: 10.1016/j.ophtha.2014.07.026.

Mathews PM, Hahn S, Hessen M, Kim J, Grader-Beck T, Birnbaum J, et al. Ocular complications of primary Sjögren syndrome in men. Am J Ophthalmol [Internet]. 2015 Sep 1 [cited 2024 Jan 27];160(3):447-452.e1. Available from: https://pubmed.ncbi.nlm.nih.gov/26093285/. Doi: 10.1016/J.AJO.2015.06.004.

Fox RI, Stern M, Michelson P. Update in Sjögren syndrome. Curr Opin Rheumatol [Internet]. 2000 [cited 2024 Jan 27];12(5):391–8. Available from: https://pubmed.ncbi.nlm.nih.gov/10990175/. Doi: 10.1097/00002281-200009000-00007.

Akpek EK, Thorne JE, Qazi FA, Do D V., Jabs DA. Evaluation of patients with scleritis for systemic disease. Ophthalmology [Internet]. 2004 [cited 2024 Jan 27];111(3):501–6. Available from: https://pubmed.ncbi.nlm.nih.gov/15019326/. Doi: 10.1016/J.OPHTHA.2003.06.006.

Stem MS, Todorich B, Faia LJ. Ocular Pharmacology for Scleritis: Review of Treatment and a Practical Perspective. J Ocul Pharmacol Ther [Internet]. 2017 May 1 [cited 2024 Jan 27];33(4):240–6. Available from: https://pubmed.ncbi.nlm.nih.gov/28355124/. Doi: 10.1089/JOP.2016.0127.

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Copyright (c) 2024 Luis Fernanda Jimenez-Arcia, Carolina López-Estrada, Carlos Jaime Velásquez-Franco, Juan David Romero-Marín

Downloads

Download data is not yet available.