Plummer-Vinson Syndrome

What is Plummer-Vinson syndrome (PVS)?

Plummer-Vinson syndrome (PVS) is a rare disorder that involves having three conditions at once. Healthcare providers sometimes call this the “triad” of Plummer-Vinson syndrome. The three conditions are:

• Iron-deficiency anemia. When your red blood cells don’t have enough iron to make hemoglobin. Your red blood cells need hemoglobin to carry oxygen throughout your body.
• Esophageal webs. Atypical tissue in your esophagus (food tube) that causes it to be too narrow. 
• Dysphagia. This is the medical term for “trouble swallowing.”

Another name for Plummer-Vinson syndrome is sideropenic dysphagia. In the United Kingdom it’s called Paterson-Brown Kelly syndrome.

There are treatments available that can relieve symptoms of PVS. Still, your healthcare provider may want to monitor you after treatment. This is because people with PVS are at greater risk of developing esophageal cancer or hypopharyngeal cancer in the future. 

What are the symptoms?

The signs and symptoms of Plummer-Vinson syndrome include:

• Weakness and fatigue
• Shortness of breath (dyspnea)
• Trouble swallowing (especially solid foods)
• Burning or swollen tongue (glossitis)
• Dry mouth (xerostomia)
• Cracked sores on the side of your mouth (angular cheilitis)
• Brittle fingernails
• An enlarged thyroid gland with lumps (thyroid nodules)
• An enlarged spleen (splenomegaly)

What causes the condition?

Researchers don’t know what causes PVS. Currently, several theories exist.

It’s possible that not getting enough nutrients (like a lack of iron) causes changes in your esophagus. These changes may make swallowing difficult. Some people with PVS have autoimmune diseases, like rheumatoid arthritis and celiac disease. This leads some medical experts to think that PVS may be autoimmune-related. Others think that your genes make you more likely to get it.

But more research is needed to know for sure.

Risk factors

Plummer-Vinson syndrome is most common in people assigned female at birth (AFAB) who are white. Most people diagnosed are between ages 40 to 70 years old. Although it’s rare, children have been diagnosed, too.

The condition was more common in the early 1900s, especially in Scandinavian countries. In the late 1900s, cases dropped significantly. This is likely because of improved access to iron-rich foods in those areas.

What are the complications of PVS?

Plummer-Vinson syndrome can increase your risk of esophageal and hypopharyngeal cancer. But this condition is so rare that there’s not enough research to know just how often people with PVS get cancer.

If you have this condition, your healthcare provider will work with you to diagnose any cancers in the early stages, when treatments work best.

How is Plummer-Vinson syndrome diagnosed?

Your healthcare provider will do blood tests and imaging procedures to diagnose PVS.

• Blood tests help your provider diagnose iron-deficiency anemia. They may include a complete blood count (CBC), peripheral blood smear (PBS) and tests to check your iron levels.
• Imaging procedures help your provider diagnose esophageal webs and dysphagia. You may need an esophagram, upper endoscopy or videofluoroscopy (an X-ray that shows how your mouth, throat and esophagus work when you swallow). 

Your provider may also need to do other tests to learn what’s causing iron-deficiency anemia.

How is Plummer-Vinson syndrome treated?

Treatment involves correcting the anemia by giving you iron. To rebuild your iron stores, you may need to take iron supplements. Or you may need to get iron through an IV. You may also need treatment if you have a condition that’s causing iron-deficiency anemia. Often, treating the iron deficiency is enough to reverse the dysphagia.

If iron treatments aren’t enough to help you swallow, you may need esophageal dilation to stretch your esophagus. This is a noninvasive procedure that doesn’t require any incisions (physical cuts) in your esophagus.

Can Plummer-Vinson syndrome be prevented?

As medical experts don’t know what causes PVS, there’s no known way to prevent it. Still, it’s possible that you can reduce your risk by getting treated ASAP for any condition causing iron-deficiency anemia. You can also ensure you’re eating enough foods with iron.

What is the outlook for this condition?

The outlook for Plummer-Vinson syndrome is excellent. For most people, addressing the iron deficiency is enough to improve anemia symptoms. It usually helps with swallowing, too. If that doesn’t work, stretching your esophagus typically helps with swallowing. Some people will need more than one esophageal dilation to correct the issue. But it’s a simple, low-risk procedure.

Your healthcare provider may want to monitor you for cancer in your esophagus or throat. But it’s important to remember having PSV is only a risk factor. Having PVS doesn’t mean you’ll develop one of these cancers.

Ask your healthcare provider how your PVS diagnosis impacts your cancer risk and care going forward.

When should I see my healthcare provider?

See your healthcare provider if you have symptoms of anemia (like fatigue or shortness of breath) that don’t improve within two weeks. Schedule an appointment if you’re having trouble swallowing and the situation doesn’t improve.

What questions should I ask my healthcare provider?

Questions to ask include:

• What’s likely causing my anemia?
• What tests will I need?
• What treatments do you recommend?
• Will I need repeat procedures to fix my dysphagia?
• Should I be concerned about my cancer risk?