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What Is Plummer-Vinson Syndrome Affecting the Esophagus?
Plummer-Vinson syndrome is a manifestation of severe, long-term, iron deficiency anemia that is characterized by post-cricoid esophageal webs and dysphagia.
Q: What are esophageal webs?
A: Esophageal webs are one or more thin horizontal membranes consisting of squamous epithelium (cells that line the surface of the esophagus) and submucosa. They usually protrude from the anterior (front) wall, extending laterally across the inside esophagus but not to the posterior (rear) wall, which means that they rarely encircle the lumen.1
Dysphagia, or difficulty swallowing, from these webs is commonly painless and intermittent or progressive and may cause obstruction.
Webs can be detected by barium swallow X-ray, but the best way for demonstration is videofluoroscopy and by upper gastrointestinal endoscopy. They appear smooth, thin, and gray with eccentric or central lumen space. The webs typically occur in the upper part of the esophagus and may be missed and accidentally ruptured unless the endoscope is introduced under direct visualization.1
Iron deficiency is believed to decrease the contraction amplitude or force of the esophageal muscle resulting in motility impairment. Slower transit times have been recorded at the proximal and middle parts of the esophagus of Plummer-Vinson syndrome patients compared to healthy volunteers.2 Transit time is how fast ingested food and fluids travel through the esophagus.
Gude et al, report that iron replacement does not necessarily reverse the dysphagia in all the cases of Plummer-Vinson syndrome and that close monitoring of the web is mandated to watch for malignant change. In fact, 3 to 15 per cent of the patients with Plummer-Vinson syndrome, mostly women between 15 and 50 years of age, have been reported to develop esophageal or pharyngeal cancer.2
What Is Plummer-Vinson Syndrome Affecting the Esophagus In Celiac Disease and/or Gluten Sensitivity?
- Relationship between Plummer-Vinson syndrome and celiac disease. Plummer-Vinson syndrome is a nutritional deficiency disorder associated with celiac disease and stems from malabsorption of iron.
- Relationship between Plummer-Vinson syndrome and iron deficiency. The high cellular turnover rate in the epithelium of the upper digestive tract makes it vulnerable to iron deficiency because of the deficiency of the iron dependent enzymes. This depletion of the oxidative enzymes of epithelial cells, free radical stress, and DNA damage may be responsible for the epithelial changes observed in patients with Plummer-Vinson syndrome. This also predisposes the patients to malignant change, especially squamous cell carcinomas.2
How Prevalent Is Plummer-Vinson Syndrome In Celiac Disease and/or Gluten Sensitivity?
Plummer-Vinson syndrome has increased frequency in untreated celiac disease patients.3
In a study of 21 consecutive patients diagnosed over a period of 4.5 years with Plummer-Vinson syndrome, 5 (23.8% ) were diagnosed with celiac disease all of whom had evidence of iron deficiency and none of these patients gave a history of chronic diarrhea.4
What Are The Symptoms Of Plummer-Vinson Syndrome ?
Plummer-Vinson syndrome is marked by these symptoms:
- Difficulty swallowing.
- Iron deficiency anemia with its symptoms of pallor, fatigue, weakness, tachycardia (rapid heartbeat), and irritability or restlessness.
- Additional features are glossitis (pale, smooth, burning tongue), angular cheilitis (inflammation of corners of mouth) and koilonychia (thin spooning of nails).
- Enlargement of the spleen and thyroid may also be observed.
How Does Plummer-Vinson Syndrome In Celiac Disease and/or Gluten Sensitivity Develop?
- The pathogenesis of Plummer-Vinson syndrome is unknown.1
- Plummer-Vinson syndrome in celiac disease results from iron deficiency due to malabsorption in celiac disease and most likely the inflammatory effects of gluten.
- Pyridoxine (vitamin B6) deficiency is also believed to contribute to Plummer-Vinson syndrome.
- Other causes include GERD, an associated disorder in celiac disease.
Does Plummer-Vinson Syndrome Respond To Gluten-Free Diet?
Yes. Celiac disease-related Plummer Vinson syndrome resolves on gluten free diet containing adequate iron. Iron supplementation is prescribed based on blood tests to determine iron status in the body.
6 Steps To Improve Plummer-Vinson Syndrome In Celiac Disease and/or Gluten Sensitivity:
- 1Remove the Trigger. Maintain a Strict, Nutritious Gluten Free Diet:
- Gut health is the foundation to restore ALL health. Restored health will enable you to maintain a strict gluten free diet, just as other life tasks will be easier.
- A strict gluten free diet means removing 100% of wheat, barley, rye and oats from the diet.
- Cutting out bread and other obvious sources of gluten is not good enough for recovery. Even 1/8th teaspoon of flour or bread crumb is enough to sustain the inflammation that is damaging your small intestine, causing increased permeability (leaky gut) and allowing undigested gluten to enter your body where it can damage structures and function, and instigate immune inflammatory responses.
Correct Your Individual Nutritional Needs.
- Eat foods that can replenish missing nutrients. Find them under NUTRIENT DEFICIENCIES.
- Take nutritional supplements as needed. Find them under NUTRIENT DEFICIENCIES.
Recovery. You should begin to feel better within a week and notice more energy as inflammation subsides and the absorbing cells that make up the surface lining of your small intestine are better able to function.
- Intestinal lining cells are replaced every 5 days. The healing process is like sunburn where the damaged surface layer of skin sloughs off and is replaced with new normal cells.
- Leaky gut normally resolves in two month after starting a gluten free diet and brings about a big improvement in health. Improvement in intestinal permeability precedes morphometric recovery (cell appearance and structure) of the small intestine in celiac disease.5
- The intestinal lining may take up to a year to heal.
- 2 Reduce Inflammation. Foods to Eat and Foods Not to Eat:
Because gluten is inflammatory, eliminate OTHER inflammatory foods from your diet to reduce an additive effect to gluten. At the same time, try to eat foods that reduce inflammation (anti-inflammatory).
- Damaging Foods. In susceptible persons, includes corn, dairy (cow), and soy. Lactose, the sugar in any animal milk disrupts intestinal permeability causing leaky gut.6
- Allergenic Foods. Includes foods that trigger the immune sytem to produce IgE antibodies. Allergy testing is the usual way to discover these offending foods.
- Shelf Stable Processed Foods. Includes any that contain additives and preservatives. Look for them on the nutrition label of the box or package. Additives and preservatives also disrupt intestinal permeability causing leaky gut.6
- Fats. Limit deep fried foods, trans-fats, saturated fats (animal fat/butter), and EXCESSIVE omega-6 fatty acid oils like corn oil. Rancid fats, sodium caprate (a medium chain fat), and sucrose monester fatty acid (a food grade surfactant) induce significant disruption of the intestinal barrier that causes leaky gut.6.
- Excessive Refined White Flours (bran layer removed). Includes products made from them such as cookies, bread, cakes, pies. Bran contains the vitamins and minerals that metabolize grains and slows the otherwise rapid entry of sugar from their digestion into the bloodstream. Also disrupt intestinal permeability causing leaky gut.6
- Refined Sugars. Includes white sugar, corn fructose and high fructose corn syrup.
- Certain Spices. Includes paprika and cayenne pepper which disrupt intestinal permeability causing leaky gut.6
- Alcohol and Caffeine. Disrupt intestinal permeability causing leaky gut.6
- Fruits. Contain ample amounts of vitamins, minerals and phytochemicals which are naturally occuring components in plants that detoxify toxins, carcinogens (reducing the risk by 50%) and mutagens.
- Non-Starchy Vegetables. Support intestinal integrity and provide ample amounts of vitamins, minerals and phytochemicals. Includes lettuce, kale, onion, broccoli, garlic, and others.
- High Quality Complex Carbohydrates. Provide vitamins, minerals, phytochemicals, and fiber while boosting serotonin levels to help you relax and feel calm. Includes whole grains, legumes, and root vegetables such as carrots, parsnips, sweet potatoes, turnips, red beets, and others.
- Antioxidants. Protect the body from inflammatory oxidant molecules that continually occur and help us handle stress and reduce irritability. Includes vitamin C-containing foods such as lemon, grapefruit, apricot, Brussels sprouts and strawberries, and others. Also, includes vitamin E-containing foods such as nuts, seeds, avocado, olive oil, and others. Cocoa is good, too.
- Omega-3 Fatty Acids. Balance opposing omega-6 fatty acids and bad fats. Fish sources include tuna, salmon, cod, and others. Plant sources include flax, chia seeds, canola oil, and others.
- Probiotics. Supply normal microbes needed for colon health and health of the body such as these fermented foods: yogurt, kefir, and unpasteurized apple cider vinegar.
- Prebiotics/ High Fiber Foods. Food with fiber keeps our population of colonic microbes healthy.
- Protective Herbs and Spices. See below #6 for examples such as ginger.
- 3 Information Sheet You Can Take to Your Doctor or Other Health Professional:
- 4 Manage Your Medications Safely:
Certain prescription drugs deplete iron which is the cause of Plummer-Vinson syndrome and vitamin B6 which promotes the condition. Ask your doctor or pharmacist about this possible adverse effect if you are taking any of the drugs listed below. Do not stop prescribed medications without supervision.
This is not a complete listing.
CHOLESTEROL DRUGS
- Colestid® and Questran® deplete Iron and others.
ANTIBIOTICS disrupt intestinal permeability which complicates celiac disease.
- Tetracyclines deplete Iron, Vitamin B6.
ANTI-INFLAMMATORIES disrupt intestinal permeability.
- Aspirin and Salicylates deplete Iron and others.
- Corticosteroids (Prednisone, Medrol®, Aristocort®, Decadron) deplete Vitamin B6.
ANTACIDS / ULCER MEDICATIONS
- Pepcid®, Tagamet®, Zantac® deplete Iron and others.
- Magnesium and Aluminum Antacid preparations (Gaviscon®, Maalox®, Mylanta®) deplete Iron and others.
CARDIOVASCULAR DRUGS
- Antihypertensives (Catapres®, Aldomet) deplete Vitamin B6.
DIURETICS
- Loop Diuretics (Lasix®, Bumex®, Edecrin®) deplete Vitamin B6.
FEMALE HORMONES disrupt intestinal permeability which complicate celiac disease.
- Oral Contraceptives (Norinyl®, Ortho-Novum®, Triphasil®, and others) deplete Vitamin B6.
- Oral Estrogen/Hormone Replacement (Evista®, Prempro®, Premarin®, Estratab® and others) deplete Vitamin B6.
- 5Nutritional Supplements To Help Correct Deficiencies:
The type and quantity of nutritional supplements that may be needed depend on which nutrients are deficient.
- Multi-vitamin/mineral supplement once a day giving 100% (not thousands).
- Ferrous fumarate as prescribed by doctor based on blood studies to determine iron needs. Do not take with other supplements because of interactions.
- Vitamin B6 as needed to restore normal level.
Storage Note for Supplements: Store container tightly sealed, away from heat, moisture and direct light to avoid loss of potency. That is, in a safe kitchen cabinet – not in the bathroom or on the kitchen table.
- 6Manage Natural Remedies:
- Eight glasses of water are recommended per day unless there is a contraindication such as kidney or heart disease. The Institute of Medicine recommends approximately 2.7 liters (91 ounces) of total water, from all beverages and foods, each day for women and 3.7 liters (125 ounces) daily of total water for men.
- If you are thirsty, drink water. Add fresh, squeezed lemon to water. Lemon is anti-inflammatory, alkalizing and provides vitamin C.
- Hydration Test: Urine should be pale yellow. Fingertips should be plump, without pruning but this may not be reliable when fingers are swollen with edema. Lips should be plump, without puckering. The feeling of thirst can be unreliable.
- What is wrong with soda, coffee, tea, and alcohol? These drinks are dehydrating, increase acid, and deplete nutrients.
Carminative Food Remedies:
- Raspberry.
- Carrot is also a cleansing digestive tonic.
- Grape is also bile stimulating and a cleansing remedy for sluggish digestion and laxative.
- Redbeets stimulate and improve digestion and are easily digested.
- Cabbage stimulates and improves digestion and is also a liver decongestant.
- Lettuce stimulates and improves digestion and is also an alterative, meaning it improves the function of organs involved with the digestion and excretion of waste products to bring about a gradual change.
- Potatoes are antispasmodic (due to atropine like properties) and a liver remedy.
Carminative Herb Remedies:
- Sage is also a digestive, astringent, bile stimulant and energy tonic that heals the mucosa. Drink as tea or use in cooking.
- Chamomile (as a tea) also promotes healing and help relieve nervous tension. Drink as a tea.
- Parsley relieves colic, gas and indigestion.
- Rosemary as a tea and in cooking also is a nervous system tonic for stress and fatigue, bile stimulant, and can relieve headaches and indigestion.
- Thyme is a soothing remedy useful for stimulating digestion of rich, fatty foods.
Carminative Spice Remedies:
- Cloves are also antispasmodic.
- Nutmeg is also useful for abdominal bloating, indigestion and colic.
- Ginger also supresses inflammation.
Exercise improves circulation and rids the body of toxins.
- Walking is aerobic exercise that reconditions the whole body to improve stamina. Read more about Exercise and Fitness.
- Weight training builds muscle. Read more about Exercise and Fitness.
- Stretching improves flexibilty. Read more about Exercise and Fitness.
Note: Exercise is important, but the amount and type of exercise undertaken depends on your health. Your first priority is to heal.
What Do Medical Research Studies Tell About Plummer-Vinson Syndrome In Celiac Disease and/or Gluten Sensitivity?
RESEARCH STUDY SUMMARIES
“Cervical esophageal web and celiac disease.” This prospective study investigating the association of cervical esophageal web with celiac disease found that there is association between cervical esophageal web and celiac disease. All adult patients with cervical esophageal web and iron deficiency need screening for celiac disease even in the absence of chronic diarrhea.
Subjects included were consecutive patients with symptomatic cervical esophageal web diagnosed over a period of 4.5 years. Tissue transglutaminase antibody was measured in serum of each patient. Patients with elevated tissue transglutaminase antibody titer were subjected to esophagogastroduodenoscopy and biopsies were obtained from the descending duodenum to look for histological changes of celiac disease. Esophageal web was treated with bougie dilatation. Celiac disease was diagnosed on the basis of elevated tissue transglutaminase antibody and suggestive duodenal histology.
RESULTS: Twenty one patients were diagnosed to have cervical esophageal web. Eighteen (85.7%) had evidence of iron deficiency. Five (23.8%) patients with cervical esophageal web fulfilled criteria for diagnosis of celiac disease. All five had evidence of iron deficiency. None of these patients gave a history of chronic diarrhea. All patients were treated with bougie dilatation. Patients with celiac disease were advised of a gluten-free diet. All five celiac disease patients are free of dysphagia without recurrence after a mean follow up of 10 months (range: 3 to 16 months).7
CASE REPORT SUMMARIES
“Plummer Vinson syndrome: unusual features.” This case report describes unusual clubbing instead of koilonychia of the nails and tortuous esophagus in 3 patients diagnosed with celiac disease and Plummer-Vinson syndrome.8
- Novacek G. Plummer-Vinson syndrome. Orphanet J Rare Dis. 2006; 1: 36. Published online 2006 September 15. doi: 10.1186/1750-1172-1-36. [↩] [↩] [↩]
- Gude D, Bansal DP, and Malu A. Revisiting Plummer Vinson Syndrome. Annals of Medical and Health Sciences Research. 2013 Jan-Mar;3(1):119-121. [↩] [↩] [↩]
- Makharia GK, Nandi B, Garg PK, Tandon RK. Plummer Vinson syndrome: unusual features. Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology. Mar-Apr 2002;21(2):74-5. [↩]
- Sinha SK, Nain CK, Udawat HP, Prasad KK, Das R, Nagi B, Singh K. Cervical esophageal web and celiac disease. J Gastroenterol Hepatol. 2008 Jul;23(7 Pt 1):1149-52. doi: 10.1111/j.1440-1746.2008.05452.x. Epub 2008 Jun 28. [↩]
- Cummins AG, Thompson FM, Butler RN, et al. Improvement in intestinal permeability precedes morphometric recovery of the small intestine in coeliac disease. Clinical Science. Apr 2001;100(4):379-86. [↩]
- Farhadi A, Banan A, Fields J, Keshavarzian A. Intestinal barrier: an interface between health and disease. Journal of Gastroenterology and Hepatology. 2003;18:479-91. [↩] [↩] [↩] [↩] [↩] [↩]
- Sinha SK, Nain CK, Udawat HP, Prasad KK, Das R, Nagi B, Singh K. Cervical esophageal web and celiac disease. J Gastroenterol Hepatol. 2008 Jul;23(7 Pt 1):1149-52. doi: 10.1111/j.1440-1746.2008.05452.x. [↩]
- Makharia GK, Nandi B, Garg PK, Tandon RK. Plummer Vinson syndrome: unusual features. Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology. Mar-Apr 2002;21(2):74-5. [↩]