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Immunologic Research Feb 2022This 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... (Review)
Review
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.
Topics: Anemia, Iron-Deficiency; Deglutition Disorders; Female; Humans; Iron; Male; Middle Aged; Plummer-Vinson Syndrome; Sjogren's Syndrome
PubMed: 34651287
DOI: 10.1007/s12026-021-09243-y -
Orphanet Journal of Rare Diseases Sep 2006Plummer-Vinson or Paterson-Kelly syndrome presents as a classical triad of dysphagia, iron-deficiency anemia and esophageal webs. Exact data about epidemiology of the... (Review)
Review
Plummer-Vinson or Paterson-Kelly syndrome presents as a classical triad of dysphagia, iron-deficiency anemia and esophageal webs. Exact data about epidemiology of the syndrome are not available; the syndrome is extremely rare. Most of the patients are white middle-aged women, in the fourth to seventh decade of life but the syndrome has also been described in children and adolescents. The dysphagia is usually painless and intermittent or progressive over years, limited to solids and sometimes associated with weight loss. Symptoms resulting from anemia (weakness, pallor, fatigue, tachycardia) may dominate the clinical picture. Additional features are glossitis, angular cheilitis and koilonychia. Enlargement of the spleen and thyroid may also be observed. One of the most important clinical aspects of Plummer-Vinson syndrome is the association with upper alimentary tract cancers. Etiopathogenesis of Plummer-Vinson syndrome is unknown. The most important possible etiological factor is iron deficiency. Other possible factors include malnutrition, genetic predisposition or autoimmune processes. Plummer-Vinson syndrome can be treated effectively with iron supplementation and mechanical dilation. In case of significant obstruction of the esophageal lumen by esophageal web and persistent dysphagia despite iron supplementation, rupture and dilation of the web are necessary. Since Plummer-Vinson syndrome is associated with an increased risk of squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely.
Topics: Adolescent; Adult; Age Distribution; Aged; Aged, 80 and over; Deglutition Disorders; Diagnosis, Differential; Female; Global Health; Humans; Iron; Male; Middle Aged; Plummer-Vinson Syndrome; Prevalence; Prognosis; Rare Diseases; Sex Distribution; Young Adult
PubMed: 16978405
DOI: 10.1186/1750-1172-1-36 -
Ear, Nose, & Throat Journal Jun 2019
Topics: Anemia, Iron-Deficiency; Dyspnea; Fatigue; Female; Humans; Middle Aged; Plummer-Vinson Syndrome
PubMed: 31208220
DOI: 10.1177/0145561319850412 -
The New England Journal of Medicine Feb 2024
Topics: Humans; Plummer-Vinson Syndrome; Deglutition Disorders
PubMed: 38345572
DOI: 10.1056/NEJMicm2309721 -
Journal of Multidisciplinary Healthcare 2019Plummer-Vinson syndrome is a rare condition associated with dysphagia, iron deficiency, and esophageal webs. Data regarding this condition is limited to mostly case... (Review)
Review
Plummer-Vinson syndrome is a rare condition associated with dysphagia, iron deficiency, and esophageal webs. Data regarding this condition is limited to mostly case reports and a few small cohort studies. Although most cases have a benign and indolent course, the risk of malignancy warrants long-term surveillance. A multidisciplinary approach among healthcare providers is of the utmost importance in the management of this condition.
PubMed: 31417270
DOI: 10.2147/JMDH.S180410 -
Diseases of the Esophagus : Official... 2003Plummer-Vinson syndrome is characterized by dysphagia, iron deficiency, anemia and the presence of esophageal web or webs. Two cases of this syndrome are reported in... (Review)
Review
Plummer-Vinson syndrome is characterized by dysphagia, iron deficiency, anemia and the presence of esophageal web or webs. Two cases of this syndrome are reported in middle-aged women, which were treated over the last eight years. Both patients presented with dysphagia, anemia, sideropenia, glossitis and cheilitis. Radiological examination of the pharynx showed the presence of webs in both cases. The patients were treated with iron supplementation, which resulted in elimination of the symptoms. Both patients remain in good general condition and without any dysphagic complaints, 5 and 8 years after the diagnosis, respectively.
Topics: Adult; Diagnosis, Differential; Esophagus; Female; Humans; Iron; Middle Aged; Plummer-Vinson Syndrome
PubMed: 12823219
DOI: 10.1046/j.1442-2050.2003.00316.x -
Mayo Clinic Proceedings Mar 2016
Topics: Adult; Anemia; Comorbidity; Esophagus; Female; Hematinics; Humans; Plummer-Vinson Syndrome; Treatment Outcome
PubMed: 26944249
DOI: 10.1016/j.mayocp.2015.11.002 -
Journal of Blood Medicine 2017Plummer-Vinson syndrome (PVS), a rare clinical condition, is characterized by a triad of dysphagia, iron deficiency anemia and esophageal web in the post-cricoid region.... (Review)
Review
Plummer-Vinson syndrome (PVS), a rare clinical condition, is characterized by a triad of dysphagia, iron deficiency anemia and esophageal web in the post-cricoid region. It was first described over a century ago. However, literature on this condition remains scanty, and its prevalence appears to be declining worldwide, possibly due to improvements in nutrition over time. The condition has been reported most commonly in thin-built, middle-aged, white women. The esophageal webs in PVS are thin mucosal folds, which are best seen either in lateral views at barium swallow or at esophagoscopy. These are usually semilunar or crescentic, being located most often along the anterior esophageal wall, but can be concentric. The exact cause and pathogenesis of PVS remain unclear, though iron and other nutritional deficiencies, genetic predisposition and autoimmunity have all been implicated in formation of the webs. Treatment includes correction of iron deficiency and endoscopic dilation of the esophageal webs to relieve dysphagia. PVS is associated with an increased risk of hypopharyngeal and esophageal malignancies. Correction of iron deficiency may arrest and reverse the mucosal changes and possibly reduces this risk.
PubMed: 29089792
DOI: 10.2147/JBM.S127801 -
Clinical Gastroenterology and... Dec 2013
Topics: Adult; Esophagoscopy; Esophagus; Female; Humans; Nails, Malformed; Pallor; Plummer-Vinson Syndrome; Radiography
PubMed: 23707464
DOI: 10.1016/j.cgh.2013.05.012