Plummer-Vinson syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief:

Overview

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

Natural History, Complications, and Prognosis

Natural History

  • The symptoms of Plummer-Vinson syndrome usually develop in the fourth decade of life, and start with symptoms such as fatigue, swollen tongue and pain while swallowing.
  • If left untreated, patients of Plummer-Vinson syndrome may progress to develop dysphagia, esophageal strictures, pharyngeal and esophageal cancer.
    • Initially patients of Plummer-Vinson syndrome presents with dysphagia for solid food.
    • As the disease progresses, esophageal webs and strictures become more apparent and progress to present with dysphagia for both solid and liquids, choking spells and aspiration.
    • Untreated Plummer-Vinson syndrome has the potential to transform into hypopharyngeal and esophageal carcinoma.

Complications

  • Common complications of Plummer-Vinson syndrome include:[1][2]
    • Hypopharyngeal cancer
    • Esophageal cancer
    • Malignant lesions of oral mucosa
    • High-output heart failure
    • Preterm delivery
    • Developmental delay

Prognosis

  • Depending on the extent of Plummer-Vinson syndrome at the time of diagnosis, the prognosis may vary.[3][4][5]
  • Prognosis is generally good for patients of Plummer-Vinson syndrome who receive treatment unless complicated by pharyngeal or esophageal carcinoma.
  • Anemia and esophageal webs seen in Plummer-Vinson syndrome can be rapidly reversed with iron replacement therapy and dilatation respectively.
  • Studies have shown that patients of Plummer-Vinson syndrome are at a high risk of developing malignant lesions of the oral mucosa, hypopharynx, and esophagus. Therefore, untreated patients require regular surveillance and close follow up.

References

  1. Larsson LG, Sandström A, Westling P (1975). "Relationship of Plummer-Vinson disease to cancer of the upper alimentary tract in Sweden". Cancer Res. 35 (11 Pt. 2): 3308–16. PMID 1192404.
  2. Rashid Z, Kumar A, Komar M (1999). "Plummer-Vinson syndrome and postcricoid carcinoma: late complications of unrecognized celiac disease". Am. J. Gastroenterol. 94 (7): 1991. doi:10.1111/j.1572-0241.1999.01991.x. PMID 10406289.
  3. Tahara T, Shibata T, Okubo M, Yoshioka D, Ishizuka T, Sumi K, Kawamura T, Nagasaka M, Nakagawa Y, Nakamura M, Arisawa T, Ohmiya N, Hirata I (2014). "A case of plummer-vinson syndrome showing rapid improvement of Dysphagia and esophageal web after two weeks of iron therapy". Case Rep Gastroenterol. 8 (2): 211–5. doi:10.1159/000364820. PMC 4086037. PMID 25028578.
  4. Samad A, Mohan N, Balaji RV, Augustine D, Patil SG (2015). "Oral manifestations of plummer-vinson syndrome: a classic report with literature review". J Int Oral Health. 7 (3): 68–71. PMC 4385731. PMID 25878483.
  5. Jessner W, Vogelsang H, Püspök A, Ferenci P, Gangl A, Novacek G, Bodisch A, Wenzl E (2003). "Plummer-Vinson syndrome associated with celiac disease and complicated by postcricoid carcinoma and carcinoma of the tongue". Am. J. Gastroenterol. 98 (5): 1208–9. doi:10.1111/j.1572-0241.2003.07438.x. PMID 12809857.

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