A 34-year-old woman applied for life insurance. She was 5 feet 2 inches tall and weighed 230 pounds. Review of the medical history revealed thyroidectomy and hysterectomy (for endometriosis) in the distant past. Laboratory abnormalities included hemoglobin of 10.6 to 11.8g/dl in 2017 and 2018. There was an elevated erythrocyte sedimentation rate (ESR) of 61 and 69 mm/hr in 2017. There was a remote history of pancreatitis, cholecystectomy and a biliary stent. There was also a history of fibromyalgia. The proposed insured was prescribed thyroid replacement and iron supplementation. In addition, there was a diagnosis of iron deficiency anemia due to sideropenic dysphagia without details.
What is sideropenic dysphagia and what are the mortality implications?
Sideropenic dysphagia is more commonly known as Plummer-Vinson syndrome (PVS) in the United States. The disorder is also known as Patterson-Brown-Kelly syndrome in the UK. It is a rare disorder consisting of the clinical triad of dysphagia, an esophageal web or membrane and iron deficiency anemia. Other findings, probably related to the iron deficiency, can include glossitis, cheilosis and koilonychia (spoon-shaped nails).
Incidence
While exact figures on the frequency of PVS are unknown, it was not uncommon in Scandinavian countries in the early 1900s. More recently it is extremely rare, occurring primarily in Caucasian women in their fourth to seventh decades of life. Very rarely it can present in children, adolescents or men.
The exact pathogenesis is unknown, but is thought to be related to a combination of autoimmune, genetic and dietary influences. An association with celiac disease, thyroid disease and rheumatoid arthritis has been noted.
Reduction in the incidence of PVS over time is thought to be related to better nutrition and less iron deficiency in the population. However, in areas of Africa where malnutrition and iron deficiency anemia are endemic, PVS is very rarely encountered, raising the possibility that there must be other factors in the pathogenesis.
Dysphagia, often with solid foods, is the most common presenting complaint. It can be intermittent, progressive and associated with weight loss. Esophageal webs can be identified using barium swallow, videoflouroscopy or upper gastrointestinal endoscopy. The webs are usually below the cricoid cartilage and adhere to the anterior wall of the esophagous. Once other causes of dysphagia and iron deficiency anemia are excluded, the diagnosis can be made based on the classic triad.
Treatment
Treatment of PVS consists of iron replacement or other treatment of anemia. Often symptoms of dysphagia will improve with resolution of the anemia. If symptomatic, dysphagia can be treated with balloon dilatation. PVS is recognized as a risk factor for developing pharyngeal or esophageal squamous cell carcinoma. For that reason, some experts recommend monitoring with periodic endoscopy.
Returning to the Case
There were no details as to how the diagnosis was made. It would be prudent to get details of the work-up and the monitoring given the association of PVS with squamous carcinoma. In general, those with PVS respond well to iron treatment and esophageal dilatation as needed. If a thorough evaluation was done and ongoing monitoring is planned, there would be minimal excess mortality anticipated.
References
- Larsson LG, et al. “Relationship of Plummer-Vinson Disease to Cancer of the Upper Alimentary Tract in Sweden,” Cancer Research (1975);35:3308-16.
- Novacek G “Plummer-Vinson Syndrome,” Orphanet J of Rare Dis (2006);1:36.
- Kuwabara M, et al. “A Web Effect: Plummer-Vinson Syndrome,” Am J Med (2018);131(5):504-5.
- Up to Date: last accessed 8/31/18.
- Goel A, et al. “Single-center prospective study of Plummer-Vinson Syndrome,” Dis of the Esophagus (2016);29:837-841.
