Plummer-Vinson Syndrome 

  • Author: Louis-Michel Wong Kee Song, MD; Chief Editor: Julian Katz, MD   more...
 
Updated: Jun 21, 2011
 

Background

The association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia is known as Plummer-Vinson syndrome (PVS) in the United States and Paterson-Brown Kelly syndrome in the United Kingdom.[1, 2, 3, 4, 5] The term sideropenic dysphagia has also been used, since the syndrome can occur with iron deficiency (sideropenia), but it is not associated with anemia.[6]

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Pathophysiology

The pathogenesis of PVS remains speculative, and the existence of the syndrome has been challenged. Postulated etiopathogenic mechanisms include iron and nutritional deficiencies, genetic predisposition, and autoimmune factors, amongst others.[7, 8]

The prevalent iron deficiency theory remains controversial. Older reports have implicated iron deficiency in the pathogenesis of esophageal webs and dysphagia in predisposed individuals. The depletion of iron-dependent oxidative enzymes may produce myasthenic changes in muscles involved in the swallowing mechanism, atrophy of the esophageal mucosa, and formation of webs as epithelial complications.

The improvement in dysphagia after iron therapy provides evidence for an association between iron deficiency and postcricoid dysphagia.[2] Anecdotal reports have also been made of patients with PVS exhibiting impaired esophageal motility (with dysphagia) that recovers following iron therapy.[9] Moreover, the decline in PVS seems to parallel an improvement in nutritional status, including iron supplementation.

However, population-based studies have shown no relationship between postcricoid dysphagia and anemia or sideropenia.[6] Other studies have demonstrated that patients with webs are as likely to be iron deficient as controls, and webs are often found in patients without iron deficiency or dysphagia. Lastly, the iron deficiency theory does not explain the predilection of webs for the upper esophagus and the rarity of the syndrome in populations in which chronic iron deficiency is endemic (eg, eastern and central Africa).[2]

PVS has also been viewed as an autoimmune phenomenon. The syndrome has been associated with autoimmune conditions, such as rheumatoid arthritis, pernicious anemia, celiac disease, and thyroiditis.[10, 7] In one study, a significantly higher proportion of patients with PVS had thyroid cytoplasmic autoimmune antibodies compared to controls with iron deficiency. The autoimmune theory, however, has gained little acceptance to date.

A complicated inlet patch (heterotopic gastric mucosa) has also been implicated in the pathogenesis of PVS.[11, 12] An ulcerated inlet patch in the upper esophagus can cause stricturing (weblike formation) and bleeding (with subsequent iron deficiency).[13] However, most studies with biopsy or autopsy specimens have not demonstrated the presence of gastric metaplasia in the samples.

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Epidemiology

Frequency

United States

Reliable prevalence data on PVS are lacking. The syndrome is now a rarity, and its decline has been attributed to better nutrition and health care. Webs may be found in 5-15% of selected patients presenting with dysphagia, but most of these patients do not have PVS.

International

In the first half of the 20th century, PVS was a relatively common finding, particularly among middle-aged Scandinavian women. The rapid fall in prevalence of the syndrome in the latter part of the 20th century has paralleled an improvement in nutritional status, including widespread addition of iron to flour.[14]

Mortality/Morbidity

Morbidity issues primarily relate to diet modification and repeat esophageal dilations (with a small risk of perforation) in patients with PVS who have recurrent dysphagia. Updated mortality data are unavailable for this rare syndrome, but, presumably, mortality is low.

Race

PVS has mainly been described in whites.

Sex

PVS is more frequently observed in women. In earlier studies from Scandinavia, up to 90% of patients were women.

Age

The typical age range at diagnosis is 40-70 years. A handful of cases have been reported in children.[15]

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Contributor Information and Disclosures
Author

Louis-Michel Wong Kee Song, MD  Associate Professor, Department of Medicine, Division of Gastroenterology and Hepatology, Mayo Clinic

Louis-Michel Wong Kee Song, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Specialty Editor Board

Marco G Patti, MD  Professor of Surgery, Director, Center for Esophageal Diseases, University of Chicago Pritzker School of Medicine

Marco G Patti, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Surgeons, American Gastroenterological Association, American Medical Association, American Surgical Association, Association for Academic Surgery, Pan-Pacific Surgical Association, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, Southwestern Surgical Congress, and Western Surgical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Noel Williams, MD  Professor Emeritus, Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada; Professor, Department of Internal Medicine, Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada

Noel Williams, MD is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

References

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  29. Vinson PP. Hysterical dysphagia. Minn Med. 1922;5:107-8.

 
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Postcricoid web. Courtesy of Alan Cameron, MD.
Upper esophageal web.
Upper esophageal web.
 
 
 
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