Introduction
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. The term sideropenic dysphagia has also been used, since the syndrome can occur with iron deficiency (sideropenia), but it is not associated with anemia.
Pathophysiology
The pathogenesis of PVS remains speculative. Recently, even the existence of the syndrome has been challenged. Postulated etiopathogenic mechanisms include iron and nutritional deficiencies, genetic predisposition, and autoimmune factors, amongst others.
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. Anecdotal reports have also been made of patients with PVS exhibiting impaired esophageal motility (with dysphagia) that recovers following iron therapy. 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. 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).
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. 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. An ulcerated inlet patch in the upper esophagus can cause stricturing (weblike formation) and bleeding (with subsequent iron deficiency). However, most studies with biopsy or autopsy specimens have not demonstrated the presence of gastric metaplasia in the samples.
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.
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.
Clinical
History
- Dysphagia, if present, is typically intermittent and limited to solids. It is usually felt in the throat.
- Choking spells and aspiration may occur because of the proximal location of the web.
- Weakness, fatigue, and dyspnea are secondary to iron deficiency anemia.
- Weight loss is uncommon.
Physical
- Manifestations of iron deficiency (with or without anemia) may be evident, including the following:
- Angular cheilitis
- Glossitis
- Koilonychia (spoon nails)
- Pallor
- Splenomegaly, edentia, and enlarged nodular thyroid glands are also described in a few patients with PVS.
Causes
- The cause of PVS is unclear. Proposed etiopathogenic mechanisms include iron and nutritional deficiencies, genetic predisposition, and autoimmunity.
- Seek the underlying cause of iron deficiency anemia (eg, gastrointestinal blood loss, celiac sprue).
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Further Reading
Keywords
PVS, Plummer-Vinson’s syndrome, Paterson-Brown Kelly syndrome, Paterson-Kelly syndrome, sideropenic dysphagia, iron deficiency anemia, esophageal stenosis, atrophic glossitis, postcricoid dysphagia, upper esophageal webs, rheumatoid arthritis, pernicious anemia, celiac disease, thyroiditis, thyroid cytoplasmic autoimmune antibodies, heterotopic gastric mucosa, ulcerated inlet patch, Zenker diverticulum, iron replacement, esophageal dilation, postcricoid carcinoma, hypopharyngeal carcinoma, esophageal carcinoma, squamous esophageal cancer
