Abstract
This study aimed to describe a patient with Sjögren syndrome who developed Plummer-Vinson syndrome, and to review the literature and describe shared aspects of this rare association. A systematic screening of articles was conducted in PubMed/MEDLINE, LILACS, SciELO, Scopus, Web of Science, and Cochrane, dating 1940 to 2020. All the articles included the association between Sjögren syndrome and Plummer-Vinson syndrome. No language restriction was applied. The following terms were used: “Sjögren syndrome” or “sicca syndrome” and “Plummer-Vinson syndrome” or “Paterson-Kelly syndrome.” We performed our analysis by adding our present case, with a total of 4 cases. Three out of four were female (75%), age varied from 56 to 58 years old. In 2 cases, Sjögren syndrome preceded Plummer-Vinson syndrome diagnosis, and in 1 report, Plummer-Vinson syndrome appeared before Sjögren syndrome. Disease duration varied from 7 to 20 years. In two cases, autoantibodies were available, and antinuclear antibodies and anti-Ro/SS-A were positive in both, and anti-La/SS-B in one of them was associated with anti-dsDNA; however, no data regarding lupus was available in the article. Treatment involved iron supplementation in 3/3. Two out of three received parenteral iron supplementation, and in these two cases, mechanical esophageal dilatation was needless. In the other case, an additional endoscopic esophageal dilatation was necessary to receive the oral iron supplement. All 3 cases had a good outcome. This case illustrates a patient with Sjögren syndrome who developed the rare Plummer-Vinson syndrome. In Sjögren syndrome, the presence of iron-deficiency anemia, dysphagia, and weight loss should alert the physician to search for associated Plummer-Vinson syndrome.
Highlights
-
Plummer-Vinson syndrome (PVS) is a sporadic disease characterized by the triad of dysphagia, iron-deficient anemia, and webs on the esophagus.
-
There are only three previous cases of PVS in Sjögren syndrome.
-
The parenteral iron supplementation treats PVS adequately and seems to preclude the need for endoscopic esophageal dilatation.
Similar content being viewed by others
References
Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A, Bosch X. Primary Sjogren syndrome. BMJ. 2012;344:e3821.
Zhou JG, Qing YF, Jiang L, Yang QB, Luo WF. Clinical analysis of primary Sjögren’s syndrome complicating anemia. Clin Rheumatol. 2010;29(5):525–9.
Ouakaa-Kchaou A, Jebali S, Elloumi H, et al. Association d’un syndrome de Gougerot Sjögren et d’un syndrome de Plummer Vinson [Association of Sjögren’s syndrome and Plummer Vinson syndrome]. Rev Med Interne. 2011;32(2):e21–2.
Godtfredsen E. Relation between Sjögren’s disease, the Plummer-Vinson syndrome, and ariboflavinosis. Acta Ophthalmol (Copenh). 1947;25(1):95–109.
Charvat J. Zespół Sjögrena i Plummer-Vinsona i jego udzial w powstawaniu raka [The Sjögren and Plummer-Vinson syndrome and its role in etiology of cancer]. Pol Tyg Lek (Wars). 1951;6(37):1169–74.
Doig JA, Whaley K, Dick WC, Nuki G, Williamson J, Buchanan WW. Otolaryngological aspects of Sjögren’s syndrome. Br Med J. 1971;4(5785):460–3.
Liu CC, Lin YS. Plummer-Vinson syndrome. Ear Nose Throat J. 2019;98(5):259–260.; Novacek G. Plummer-Vinson syndrome. Orphanet J Rare Dis. 2006;1:36.
Bakshi SS. Plummer-Vinson syndrome. Mayo Clin Proc. 2016;91(3):404.
Fragkioudaki S, Mavragani CP, Moutsopoulos HM. Predicting the risk for lymphoma development in Sjogren syndrome: an easy tool for clinical use. Medicine (Baltimore). 2016;95(25):e3766.
Mackay IR, Rose NR. Autoimmunity and lymphoma: tribulations of B cells. Nat Immunol. 2001;2(9):793–5.
Hefaiedh R, Boutreaa Y, Ouakaa-Kchaou A, et al. Plummer-Vinson syndrome. Tunis Med. 2010;88(10):721–4.
Goel A, Bakshi SS, Soni N, Chhavi N. Iron deficiency anemia and Plummer-Vinson syndrome: current insights. J Blood Med. 2017;8:175–84.
Novacek G. Plummer-Vinson syndrome. Orphanet J Rare Dis. 2006;1:36.
Dutta U, Khaliqa A, Noora MT, Kochhara R, Singh K. Recurrent multiple cervical esophageal webs: an unusual presentation of celiac disease. Gastroenterology Res. 2009;2:356–7.
Zhou J-G, Qing Y-F, Li Jiang, Yang Q-B, Luo W-F. Clinical analysis of primary Sjögren’s syndrome complicating anemia. Clin Rheumatol. 2010;29:525–9.
Bécheur H, Poyet A, Delahaye F, Mostefa-Kara N. Syndrome de GougerotSjögren et achalasie. Gastroenterol Clin Biol. 2006;30:918–20.
Anselmino M, Zaninotto G, Costantini M, Ostuni P, Ianniello A, Boccu C. Esophageal motor function in primary Sjogren’s syndrome: correlation with dysphagia and xerostomia. Dig Dis Sci. 1997;42:113–8.
Author information
Authors and Affiliations
Contributions
JFC: conception, analysis, writing, interpretation, revision, submission.
AL: analysis, writing, interpretation, revision.
Corresponding author
Ethics declarations
Ethics approval
The authors declare that they followed the World Medical Association Declaration of Helsinki in this study. An informed consent was obtained from the patients for publication of their cases. No image of her is used.
Conflict of interest
The authors declare no competing interests.
Additional information
Publisher's note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
de Carvalho, J.F., Lerner, A. Plummer-Vinson syndrome in primary Sjögren syndrome: a case-based review. Immunol Res 70, 19–22 (2022). https://doi.org/10.1007/s12026-021-09243-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12026-021-09243-y