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==Overview==
==Overview==
The '''Plummer-Vinson syndrome''', also called '''Paterson-Brown-Kelly syndrome''' or '''[[sideropenic]] dysphagia''' is a disorder linked to severe, long-term [[iron deficiency anemia]], which causes [[swallowing]] difficulty ([[dysphagia]]) due to web-like membranes of [[biological tissue|tissue]] growing in the [[throat]] ([[esophageal web]]s). <ref name="pmid16978405">{{cite journal |author=Novacek G |title=Plummer-Vinson syndrome |journal=Orphanet J Rare Dis |volume=1 |issue= |pages=36 |year=2006 |pmid=16978405 |doi=10.1186/1750-1172-1-36 |url=http://www.ojrd.com/content/1//36}}</ref> The disease is named after two Americans, the [[physician]] Henry Stanley Plummer, and the surgeon Porter Paisley Vinson. <ref name="WhoNamedIt">{{WhoNamedIt|synd|1777}}</ref><ref>H. S. Plummer. Diffuse dilatation of the esophagus without anatomic stenosis (cardiospasm). A report of ninety-one cases. Journal of the American Medical Association, Chicago, 1912, 58: 2013-2015.</ref><ref>P. P. Vinson. A case of cardiospasm with dilatation and angulation of the esophagus. Medical Clinics of North America, Philadelphia, PA., 1919, 3: 623-627.</ref>
The '''Plummer-Vinson syndrome''', also called '''Paterson-Brown-Kelly syndrome''' or '''[[sideropenic]] dysphagia''' is a disorder linked to severe, long-term [[iron deficiency anemia]], which leads to dysphagia, glossitis and esophageal webs. The disease is named after two American [[physician|physicians]] Henry Stanley Plummer, and Porter Paisley Vinson. It is also called "Kelly-Paterson syndrome", named after two British otolaryngologists, Adam Brown-Kelly and Donald Ross Paterson. The exact [[pathogenesis]] of Plummer-Vinson syndrome is not fully understood. It is postulated that Plummer-Vinson syndrome results from [[iron deficiency]]. Other possible factors include [[malnutrition]], [[genetic predisposition]] and [[autoimmune disorders]]. On gross [[pathology]], [[esophageal web]] and [[esophageal]] [[strictures]] are characteristic findings of Plummer-Vinson syndrome. On microscopic [[histopathological]] [[analysis]], Plummer-Vinson syndrome presents with [[epithelial]] [[atrophy]], [[chronic]] [[submucosal]] [[inflammation]] and [[epithelial]] [[atypia]] or [[dysplasia]] (in advanced cases). There are no established risk factors for Plummer-Vinson syndrome. However, [[chronic]] [[irritation]] of the [[esophagus]] may predispose to an increased risk of developing [[Esophageal web|esophageal webs]] or [[strictures]]. Plummer-Vinson syndrome is a [[rare disease]] and the [[data]] pertaining to its [[incidence]] and [[prevalence]] is not evidently available. Overall improvement in [[Nutritional|nutritional status]] with better [[medical care]] has markedly reduced the number of cases of Plummer-Vinson syndrome. Common complications of Plummer-Vinson syndrome include [[hypopharyngeal cancer]], [[esophageal cancer]] and [[malignant]] lesions of [[oral mucosa]]. Plummer-Vinson syndrome must be differentiated from other [[diseases]] that cause [[dysphagia]] such as [[reflux esophagitis]], [[esophageal carcinoma]], [[systemic sclerosis]], [[esophageal spasm]], [[pseudoachalasia]], [[stroke]], [[esophageal candidiasis]], Zenker's diverticulum and [[Chagas disease]]. Physical examination of patients with Plummer-Vinson syndrome is usually remarkable for [[glossitis]], [[esophageal webs]] or [[Esophageal stricture|strictures]], and [[dysphagia]]. Laboratory findings consistent with the diagnosis of Plummer-Vinson syndrome include presence of [[iron deficiency anemia]]. The diagnosis of Plummer-Vinson syndrome is made in the presence of [[iron-deficiency anemia]] with [[esophageal webs]], [[dysphagia]] and [[glossitis]]. An [[x-ray]] ([[barium]] [[esophagogram]]) is the best initial [[imaging]] study in a patient suspected with Plummer-Vinson syndrome. Other [[imaging]] studies include a videofluoroscopy or an esophagogastroduodenoscopy to visualise the esophageal webs. The mainstay of treatment for Plummer-Vinson syndrome is aimed at correcting [[iron deficiency anemia]]. Effective measures for the prevention of Plummer-Vinson syndrome include good [[nutrition]] with adequate intake of [[iron]] rich foods and an upper [[Gastrointestinal tract|gastrointestinal]] [[endoscopy]] every year to rule out [[malignant]] transformation.
 
It is also sometimes called "Kelly-Paterson syndrome", after Adam Brown-Kelly and Donald Ross Paterson.<ref name="WhoNamedIt">{{WhoNamedIt|synd|1777}}</ref><ref>A. B. Kelly. Spasm at the entrance of the esophagus. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 34: 285-289.</ref><ref>D. R. Paterson. A clinical type of dysphagia. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 24: 289-291.</ref>


==Historical Perspective==
==Historical Perspective==
Plummer-Vinson syndrome was first discovered by Henry Plummer an American [[internist]], in a case series of [[patients]] with long-standing [[iron deficiency anemia]], [[dysphagia]] and [[spasm]] of the upper [[esophagus]] without [[anatomic]] [[stenosis]] in his article "Diffuse dilatation of the esophagus without anatomic stenosis." In the year 1919, Porter Paisley Vinson an American [[surgeon]] at the Mayo Clinic further described Plummer-Vinson syndrome in his article "A case of cardiospasm with dilatation and angulation of the esophagus." He reported a case of angulation of [[esophagus]] and attributed his findings to be consistent as described by Henry Plummer. In the year 1919, Donald Ross Patterson and Adam Brown Kelly, both British [[Otolaryngologists|otolaryngologist]] described the characteristic clinical features of Plummer-Vinson syndrome in their article "A clinical type of dysphagia" and "Spasm at the entrance of the esophagus" respectively.
Plummer-Vinson syndrome was first discovered by Henry Plummer an American [[internist]], in a case series of [[patients]] with long-standing [[iron deficiency anemia]], [[dysphagia]] and [[spasm]] of the upper [[esophagus]] without [[anatomic]] [[stenosis]] in his article "Diffuse dilatation of the esophagus without anatomic stenosis." In the year 1919, Porter Paisley Vinson an American [[surgeon]] at the Mayo Clinic further described Plummer-Vinson syndrome in his article "A case of cardiospasm with dilatation and angulation of the esophagus." He reported a case of angulation of [[esophagus]] and attributed his findings to be consistent as described by Henry Plummer. In the year 1919, Donald Ross Patterson and Adam Brown Kelly, both British [[Otolaryngologists|otolaryngologists]] described the characteristic clinical features of Plummer-Vinson syndrome in their article "A clinical type of dysphagia" and "Spasm at the entrance of the esophagus" respectively.


==Classification==
==Classification==
Line 17: Line 13:


==Pathophysiology==
==Pathophysiology==
Plummer-Vinson syndrome is a rare [[condition]] characterized by [[iron-deficiency anemia]], [[glossitis]] and [[dysphagia]]. The exact [[pathogenesis]] of Plummer-Vinson syndrome is not fully understood. It is postulated that Plummer-Vinson syndrome results from [[iron deficiency]]. Other possible factors include [[malnutrition]], [[genetic predisposition]] and [[autoimmune disorders]]. In [[patients]] with [[iron deficiency]], the [[iron]]-dependent [[oxidative]] [[enzymes]] are unable to function at optimum level and the dependent [[metabolic pathways]] ([[oxidative phosphorylation]]) are reduced. This promotes [[anaerobic metabolism]] with increased consumption of [[glucose]] and increased production of [[lactic acid]] and may lead to [[Myasthenia|myasthenic]] changes in [[muscles]]. These [[Myasthenia|myasthenic]] changes are often seen in [[muscles]] involved in [[swallowing]] and may lead to [[atrophy]] of the [[esophageal]] [[mucosa]] and formation of [[esophageal webs]]. Patients who do not exhibit obstructive [[lesions]] ([[Esophageal web|web]] or [[stricture]]) may have [[dysphagia]] resulting from [[muscular]] in-coordination. Patients with [[iron deficiency]] have low levels of [[myoglobin]] which may affect the [[muscles]] of the [[tongue]] and lead to glossitis. In Plummer-Vinson syndrome, [[Iron deficiency|deficiency of iron]] can lead to [[epithelial]] [[atrophy]] and a decrease in the [[regenerative]] capacity of the [[mucosa]]. The decrease in rate of [[healing]] allows the [[chronic]] [[irritants]] to act progressively, predisposing the [[oral cavity]] and [[esophagus]] to [[malignant]] [[transformation]] ([[squamous cell carcinoma]]). [[Genes]] involved in the [[pathogenesis]] of [[iron deficiency anemia]] associated with Plummer-Vinson syndrome include [[mutation]] in TMPRSS6 [[gene]]. The TMPRSS6 gene encodes instructions for the [[protein]] [[hepcidin]]. Increased levels of [[hepcidin]] leads to decreased release of [[iron]] from [[ferritin]] and subsequently presents as [[iron deficiency anemia]]. On gross [[pathology]], [[esophageal web]] and [[esophageal]] [[strictures]] are characteristic findings of Plummer-Vinson syndrome. On microscopic [[histopathological]] [[analysis]], Plummer-Vinson syndrome presents with [[epithelial]] [[atrophy]], [[chronic]] [[submucosal]] [[inflammation]] and [[epithelial]] [[atypia]] or [[dysplasia]] (in advanced cases).
Plummer-Vinson syndrome is a rare [[condition]] characterized by [[iron-deficiency anemia]], [[glossitis]] and [[dysphagia]]. The exact [[pathogenesis]] of Plummer-Vinson syndrome is not fully understood. It is postulated that Plummer-Vinson syndrome results from [[iron deficiency]]. Other possible factors include [[malnutrition]], [[genetic predisposition]] and [[autoimmune disorders]]. In [[patients]] with [[iron deficiency]], the [[iron]]-dependent [[oxidative]] [[enzymes]] are unable to function at optimum level and the dependent [[metabolic pathways]] ([[oxidative phosphorylation]]) are reduced. This promotes [[anaerobic metabolism]] with increased consumption of [[glucose]] and increased production of [[lactic acid]] and may lead to [[Myasthenia|myasthenic]] changes in [[muscles]]. These [[Myasthenia|myasthenic]] changes are often seen in [[muscles]] involved in [[swallowing]] and may lead to [[atrophy]] of the [[esophageal]] [[mucosa]] and formation of [[esophageal webs]]. Patients who do not exhibit obstructive [[lesions]] ([[Esophageal web|web]] or [[stricture]]) may have [[dysphagia]] resulting from [[muscular]] in-coordination. Patients with [[iron deficiency]] have low levels of [[myoglobin]] which may affect the [[muscles]] of the [[tongue]] and lead to glossitis. In Plummer-Vinson syndrome, [[Iron deficiency|deficiency of iron]] can lead to [[epithelial]] [[atrophy]] and a decrease in the [[regenerative]] capacity of the [[mucosa]]. The decrease in rate of [[healing]] allows the [[chronic]] [[irritants]] to act progressively, predisposing the [[oral cavity]] and [[esophagus]] to [[malignant]] [[transformation]] ([[squamous cell carcinoma]]). [[Genes]] involved in the [[pathogenesis]] of [[iron deficiency anemia]] associated with Plummer-Vinson syndrome include [[mutation]] in TMPRSS6 [[gene]]. The TMPRSS6 gene encodes instructions for the [[protein]] [[hepcidin]]. Increased levels of [[hepcidin]] leads to decreased release of [[iron]] from [[ferritin]] and subsequently presents as [[iron deficiency anemia]]. On gross [[pathology]], [[esophageal web]] and [[esophageal]] [[strictures]] are characteristic findings of Plummer-Vinson syndrome. On microscopic [[histopathological]] [[analysis]], Plummer-Vinson syndrome presents with [[epithelial]] [[atrophy]], [[chronic]] [[submucosal]] [[inflammation]] and [[epithelial]] [[atypia]] or [[dysplasia]] (in advanced cases).  


==Causes==
==Causes==
The cause of Plummer-Vinson syndrome is unknown; however, [[iron deficiency anemia]], [[Genetics|genetic]] factors and [[nutrition|nutritional deficiencies]] may play a role. [[Iron deficiency anemia]] is the most widely regarded cause of Plummer-Vinson syndrome and can be due to increased [[iron]] demand, decreased intake and [[malabsorption]] [[syndromes]].
The exact cause of Plummer-Vinson syndrome is unknown; however, [[iron deficiency anemia]], [[Genetics|genetic]] factors and [[nutrition|nutritional deficiencies]] may play a role. [[Iron deficiency anemia]] is the most widely regarded cause of Plummer-Vinson syndrome and can be due to increased [[iron]] demand, decreased intake and [[malabsorption]] [[syndromes]].


==Differentiating {{PAGENAME}} from Other Diseases==
==Differentiating {{PAGENAME}} from Other Diseases==
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===History and Symptoms===
===History and Symptoms===
Obtaining a [[History and Physical examination|history]] gives important information in making a [[diagnosis]] of Plummer-Vinson syndrome. Complete history should be obtained regarding onset, duration, and progression of [[symptoms]] such as [[dysphagia]] (solids or liquids), [[weakness]], [[fatigue]], [[dyspnea]], and history of [[choking]] spells or [[aspiration]]. The common symptoms of Plummer-Vinson syndrome are difficulty in [[swallowing]] (more for [[solids]]), burning sensation in [[mouth]], dry [[tongue]] and [[Pale skin color|pale color of the skin]]. Less common symptoms include cold intolerance, reduced resistance to [[infection]] and craving for for unusual items (such as [[ice]] or cold vegetables).


===Physical Examination===
===Physical Examination===
Physical examination of patients with Plummer-Vinson syndrome is usually remarkable for [[glossitis]], [[esophageal webs]] or [[Esophageal stricture|strictures]], and [[dysphagia]]. Other findings on physical examination include [[pallor]], [[stomatitis]], [[atrophy]] of [[Lingual|lingual papillae,]] [[splenomegaly]] (33%), [[achlorhydria]] and [[koilonychia]].


===Laboratory Findings===
===Laboratory Findings===
Line 52: Line 50:


===Other Diagnostic Studies===
===Other Diagnostic Studies===
[[Esophagogastroduodenoscopy]] (EGD) may be helpful in the [[diagnosis]] of Plummer-Vinson syndrome. EGD can directly visualize the upper [[gastrointestinal tract]] and aid in diagnosing [[esophageal webs]] seen in Plummer-Vinson syndrome. Findings suggestive of [[esophageal webs]] include thin elevated [[Mucous membrane|mucosal membrane]] covered by normal [[squamous epithelium]] on the walls of [[esophagus]].


==Treatment==
==Treatment==
===Medical Therapy===
===Medical Therapy===
The mainstay of treatment for Plummer-Vinson syndrome is aimed at correcting [[iron deficiency anemia]]. Patients with Plummer-Vinson syndrome should receive [[oral]] [[iron]] salts ([[ferrous sulphate]]) and [[iron]] supplementation in their [[Diet (nutrition)|diet]]. [[Parenteral]] [[iron]] is used in patients who are unable to tolerate [[oral]] [[iron]] or with [[Malabsorption syndrome|malabsorption syndromes]]. Another important aspect in treating Plummer-Vinson syndrome is to identify the cause of [[iron deficiency]] in order to exclude active [[hemorrhage]], [[malignancy]] or [[celiac disease]].


===Surgery===
===Surgery===
[[Surgery]] is not the first-line treatment option for patients with Plummer-Vinson syndrome. However, procedure such as mechanical [[dilatation]] with the use of an [[endoscope]] may be used in patients who are unresponsive to medical [[therapy]], have multiple obstructive [[esophageal webs]] and long-standing [[dysphagia]].


===Prevention===
===Prevention===
Effective measures for the [[primary prevention]] of Plummer-Vinson syndrome include good [[nutrition]] with adequate intake of [[iron]] rich foods. Patients of Plummer-Vinson syndrome are at a risk (10-15%) of developing [[malignant]] lesions ([[squamous cell carcinoma]]) of the [[oral mucosa]], [[hypopharynx]] and [[esophagus]]. Effective measures for the [[secondary prevention]] of Plummer-Vinson syndrome include an upper [[Gastrointestinal tract|gastrointestinal]] [[endoscopy]] every year to rule out [[malignant]] transformation.


==References==
==References==

Latest revision as of 16:52, 6 November 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Akshun Kalia M.B.B.S.[2]

Overview

The Plummer-Vinson syndrome, also called Paterson-Brown-Kelly syndrome or sideropenic dysphagia is a disorder linked to severe, long-term iron deficiency anemia, which leads to dysphagia, glossitis and esophageal webs. The disease is named after two American physicians Henry Stanley Plummer, and Porter Paisley Vinson. It is also called "Kelly-Paterson syndrome", named after two British otolaryngologists, Adam Brown-Kelly and Donald Ross Paterson. The exact pathogenesis of Plummer-Vinson syndrome is not fully understood. It is postulated that Plummer-Vinson syndrome results from iron deficiency. Other possible factors include malnutrition, genetic predisposition and autoimmune disorders. On gross pathology, esophageal web and esophageal strictures are characteristic findings of Plummer-Vinson syndrome. On microscopic histopathological analysis, Plummer-Vinson syndrome presents with epithelial atrophy, chronic submucosal inflammation and epithelial atypia or dysplasia (in advanced cases). There are no established risk factors for Plummer-Vinson syndrome. However, chronic irritation of the esophagus may predispose to an increased risk of developing esophageal webs or strictures. Plummer-Vinson syndrome is a rare disease and the data pertaining to its incidence and prevalence is not evidently available. Overall improvement in nutritional status with better medical care has markedly reduced the number of cases of Plummer-Vinson syndrome. Common complications of Plummer-Vinson syndrome include hypopharyngeal cancer, esophageal cancer and malignant lesions of oral mucosa. Plummer-Vinson syndrome must be differentiated from other diseases that cause dysphagia such as reflux esophagitis, esophageal carcinoma, systemic sclerosis, esophageal spasm, pseudoachalasia, stroke, esophageal candidiasis, Zenker's diverticulum and Chagas disease. Physical examination of patients with Plummer-Vinson syndrome is usually remarkable for glossitis, esophageal webs or strictures, and dysphagia. Laboratory findings consistent with the diagnosis of Plummer-Vinson syndrome include presence of iron deficiency anemia. The diagnosis of Plummer-Vinson syndrome is made in the presence of iron-deficiency anemia with esophageal webs, dysphagia and glossitis. An x-ray (barium esophagogram) is the best initial imaging study in a patient suspected with Plummer-Vinson syndrome. Other imaging studies include a videofluoroscopy or an esophagogastroduodenoscopy to visualise the esophageal webs. The mainstay of treatment for Plummer-Vinson syndrome is aimed at correcting iron deficiency anemia. Effective measures for the prevention of Plummer-Vinson syndrome include good nutrition with adequate intake of iron rich foods and an upper gastrointestinal endoscopy every year to rule out malignant transformation.

Historical Perspective

Plummer-Vinson syndrome was first discovered by Henry Plummer an American internist, in a case series of patients with long-standing iron deficiency anemia, dysphagia and spasm of the upper esophagus without anatomic stenosis in his article "Diffuse dilatation of the esophagus without anatomic stenosis." In the year 1919, Porter Paisley Vinson an American surgeon at the Mayo Clinic further described Plummer-Vinson syndrome in his article "A case of cardiospasm with dilatation and angulation of the esophagus." He reported a case of angulation of esophagus and attributed his findings to be consistent as described by Henry Plummer. In the year 1919, Donald Ross Patterson and Adam Brown Kelly, both British otolaryngologists described the characteristic clinical features of Plummer-Vinson syndrome in their article "A clinical type of dysphagia" and "Spasm at the entrance of the esophagus" respectively.

Classification

There is no established system for the classification of Plummer-Vinson syndrome.

Pathophysiology

Plummer-Vinson syndrome is a rare condition characterized by iron-deficiency anemia, glossitis and dysphagia. The exact pathogenesis of Plummer-Vinson syndrome is not fully understood. It is postulated that Plummer-Vinson syndrome results from iron deficiency. Other possible factors include malnutrition, genetic predisposition and autoimmune disorders. In patients with iron deficiency, the iron-dependent oxidative enzymes are unable to function at optimum level and the dependent metabolic pathways (oxidative phosphorylation) are reduced. This promotes anaerobic metabolism with increased consumption of glucose and increased production of lactic acid and may lead to myasthenic changes in muscles. These myasthenic changes are often seen in muscles involved in swallowing and may lead to atrophy of the esophageal mucosa and formation of esophageal webs. Patients who do not exhibit obstructive lesions (web or stricture) may have dysphagia resulting from muscular in-coordination. Patients with iron deficiency have low levels of myoglobin which may affect the muscles of the tongue and lead to glossitis. In Plummer-Vinson syndrome, deficiency of iron can lead to epithelial atrophy and a decrease in the regenerative capacity of the mucosa. The decrease in rate of healing allows the chronic irritants to act progressively, predisposing the oral cavity and esophagus to malignant transformation (squamous cell carcinoma). Genes involved in the pathogenesis of iron deficiency anemia associated with Plummer-Vinson syndrome include mutation in TMPRSS6 gene. The TMPRSS6 gene encodes instructions for the protein hepcidin. Increased levels of hepcidin leads to decreased release of iron from ferritin and subsequently presents as iron deficiency anemia. On gross pathology, esophageal web and esophageal strictures are characteristic findings of Plummer-Vinson syndrome. On microscopic histopathological analysis, Plummer-Vinson syndrome presents with epithelial atrophy, chronic submucosal inflammation and epithelial atypia or dysplasia (in advanced cases).

Causes

The exact cause of Plummer-Vinson syndrome is unknown; however, iron deficiency anemia, genetic factors and nutritional deficiencies may play a role. Iron deficiency anemia is the most widely regarded cause of Plummer-Vinson syndrome and can be due to increased iron demand, decreased intake and malabsorption syndromes.

Differentiating Plummer-Vinson syndrome overview from Other Diseases

Plummer-Vinson syndrome must be differentiated from other diseases that cause dysphagia such as reflux esophagitis, esophageal carcinoma, systemic sclerosis, esophageal spasm, pseudoachalasia, stroke, esophageal candidiasis, Zenker's diverticulum and Chagas disease.

Epidemiology and Demographics

Plummer-Vinson syndrome is a rare disease and the data pertaining to incidence and prevalence is not evidently available. Overall improvement in nutritional status with better medical care has markedly reduced the number of cases of Plummer-Vinson syndrome. However, individuals of any age groups may develop Plummer-Vinson syndrome and it is most commonly seen in the age group of 40-70 years. Plummer-Vinson syndrome usually affects individuals of the caucasian race. Females are commonly affected than males with female to male ratio of 4:1. The majority of Plummer-Vinson syndrome cases are reported in Scandinavian countries or north European countries.

Risk Factors

There are no established risk factors for Plummer-Vinson syndrome. However, chronic irritation of the esophagus may predispose to an increased frequency of esophageal webs or strictures. Conditions which can irritate esophagus includes thermal injury, mechanical injury, achalasia, esophageal diverticulum, chronic lye stricture, radiation therapy, injection sclerotherapy, gastric resection, celiac disease, tylosis and scleroderma.

Screening

There is insufficient evidence to recommend routine screening for Plummer-Vinson syndrome.

Natural History, Complications, and Prognosis

If left untreated, patients of Plummer-Vinson syndrome may progress to develop fatigue, dyspnea on exertion, esophageal strictures, and malignant lesions of the mouth and oral cavity. Common complications of Plummer-Vinson syndrome include hypopharyngeal cancer, esophageal cancer and malignant lesions of oral mucosa. Depending on the extent of Plummer-Vinson syndrome at the time of diagnosis, the prognosis may vary. Prognosis is generally good for patients who receive treatment. Iron replacement therapy and dilatation of esophageal web leads to rapid reversal of symptoms.

Diagnosis

Diagnostic Criteria

The diagnosis of Plummer-Vinson syndrome is made in the presence of iron-deficiency anemia with esophageal webs, dysphagia and glossitis.

History and Symptoms

Obtaining a history gives important information in making a diagnosis of Plummer-Vinson syndrome. Complete history should be obtained regarding onset, duration, and progression of symptoms such as dysphagia (solids or liquids), weakness, fatigue, dyspnea, and history of choking spells or aspiration. The common symptoms of Plummer-Vinson syndrome are difficulty in swallowing (more for solids), burning sensation in mouth, dry tongue and pale color of the skin. Less common symptoms include cold intolerance, reduced resistance to infection and craving for for unusual items (such as ice or cold vegetables).

Physical Examination

Physical examination of patients with Plummer-Vinson syndrome is usually remarkable for glossitis, esophageal webs or strictures, and dysphagia. Other findings on physical examination include pallor, stomatitis, atrophy of lingual papillae, splenomegaly (33%), achlorhydria and koilonychia.

Laboratory Findings

Laboratory findings consistent with the diagnosis of Plummer-Vinson syndrome include presence of iron deficiency anemia. Patients suspected of Plummer-Vinson syndrome should be tested with complete blood count (CBC), iron studies, peripheral smear, stool test for occult blood, blood lead levels and bone marrow biopsy for stainable iron.

Imaging Findings

Videofluoroscopy may be helpful in the diagnosis of Plummer-Vinson syndrome. Videofluoroscopy is done in patients with normal barium esophagogram who have a high probability of Plummer-Vinson syndrome. Videofluoroscopy is superior to barium esophagogram and has the ability to detect small esophageal webs resulting from insignificant mucosal and submucosal foldings which may otherwise go undiagnosed.

Other Diagnostic Studies

Esophagogastroduodenoscopy (EGD) may be helpful in the diagnosis of Plummer-Vinson syndrome. EGD can directly visualize the upper gastrointestinal tract and aid in diagnosing esophageal webs seen in Plummer-Vinson syndrome. Findings suggestive of esophageal webs include thin elevated mucosal membrane covered by normal squamous epithelium on the walls of esophagus.

Treatment

Medical Therapy

The mainstay of treatment for Plummer-Vinson syndrome is aimed at correcting iron deficiency anemia. Patients with Plummer-Vinson syndrome should receive oral iron salts (ferrous sulphate) and iron supplementation in their diet. Parenteral iron is used in patients who are unable to tolerate oral iron or with malabsorption syndromes. Another important aspect in treating Plummer-Vinson syndrome is to identify the cause of iron deficiency in order to exclude active hemorrhage, malignancy or celiac disease.

Surgery

Surgery is not the first-line treatment option for patients with Plummer-Vinson syndrome. However, procedure such as mechanical dilatation with the use of an endoscope may be used in patients who are unresponsive to medical therapy, have multiple obstructive esophageal webs and long-standing dysphagia.

Prevention

Effective measures for the primary prevention of Plummer-Vinson syndrome include good nutrition with adequate intake of iron rich foods. Patients of Plummer-Vinson syndrome are at a risk (10-15%) of developing malignant lesions (squamous cell carcinoma) of the oral mucosa, hypopharynx and esophagus. Effective measures for the secondary prevention of Plummer-Vinson syndrome include an upper gastrointestinal endoscopy every year to rule out malignant transformation.

References