Lymphocytic Esophagitis

What is Lymphocytic Esophagitis?

Lymphocytic esophagitis (LE) is a unique type of long-term inflammation of the esophagus. It was introduced by medical researchers named Rubio and his team in 2006. LE is special because it is associated with an increased amount of specific immune cells called lymphocytes in parts of the esophagus. These cells are often minimal or completely absent when other types of immune cells, called granulocytes, are present. It seems like more people are being diagnosed with LE currently. This trend could be because medical professionals are more aware of this condition. It can also be due to the fact that more medical tests called esophageal biopsies are being done to investigate swallowing difficulties.

The symptoms of LE can vary greatly and may include difficulty swallowing, an upset stomach, feelings of nausea, or chest pain. It is often found in older women who have a history of smoking, gastroesophageal reflux disease (GERD, a condition where stomach acid flows back into the esophagus causing heartburn and other issues), and achalasia (a rare disorder that makes it hard for food and liquid to pass into your stomach).

This text aims to provide an understanding of the causes, how widespread this rare subtype of chronic esophagitis is, its clinical characteristics, how it is diagnosed, how it is managed, and its complications. It emphasizes the valuable role of all health care professionals in looking after patients with this gastrointestinal disorder.

What Causes Lymphocytic Esophagitis?

Lymphocytic esophagitis is a less common type of long-term inflammation of the esophagus, and we’re not sure what causes it. It’s not clear if this condition is a unique kind of inflammation of the esophagus by itself or just a sign of another problem with the esophagus or another disease involving the body’s immune system. However, because it mainly shows up as long-term difficulty swallowing, tightness in the esophagus that can be seen during an endoscopy watchful check, and lab tests showing a high number of certain white blood cells (lymphocytes), it is likely a chronic inflammatory disease of the esophagus. We need more careful and thorough studies to understand the natural progression and outcome of this condition.

Risk Factors and Frequency for Lymphocytic Esophagitis

Learning about the course and outcome of lymphocytic esophagitis (LE) is difficult because there are not many studies on this disease. The number of people who have LE is also hard to determine. However, one large preliminary study in the U.S., involving over 40,000 patients, found LE in 0.1% of all esophagus tissue samples.

The symptoms of LE are very similar to another condition called eosinophilic esophagitis, and both are diagnosed through a procedure where a small piece of tissue is taken from the esophagus (endoscopic esophageal biopsy). More and more people have been getting tested for eosinophilic esophagitis, meaning more biopsies are being done. This may be the reason why we’re diagnosing more cases of LE now.

• The first research on LE found that it might be linked to immune disorders including:
  • rheumatic diseases,
  • common variable immunodeficiency,
  • Crohn’s disease, mainly in children.
• However, later studies on things that might increase the risk of getting LE found that the condition is more likely:
  • in women in their 60s,
  • when the person has gastroesophageal reflux disease (GERD),
  • in people with primary abnormal movements of the esophagus like achalasia,
  • in people with a history of smoking,
  • in people with certain allergies,
  • in people with an underactive thyroid (hypothyroidism),
  • in people taking aspirin or statin drugs.

Signs and Symptoms of Lymphocytic Esophagitis

Unlike eosinophilic esophagitis, which is usually diagnosed in young adult men, Lymphocytic Esophagitis (LE) is more commonly observed in women around their sixties. The symptoms typically include difficulty swallowing, heartburn, chest pain, generic abdominal pain, and nausea, sometimes accompanied by vomiting.

According to a study involving over 100 patients with LE, it was found that while the patients’ symptoms were similar to those of eosinophilic esophagitis, they did not have the same hallmark endoscopic signs of the disease, including distinctive rings and furrows.

In a separate study looking at adults who had food stuck in their esophagus, they found that 17% of the 228 participating patients showed signs of both eosinophilic esophagitis and LE, while 9% had signs of only LE. This suggests that food impaction in the esophagus can sometimes be a symptom of LE.

Testing for Lymphocytic Esophagitis

To diagnose an inflammatory condition known as lymphocytic esophagitis (LE) in your esophagus (the tube that connects your mouth to your stomach), doctors will need to take a small sample of tissue from your esophagus. This is done through a procedure called an endoscopic esophageal biopsy. Currently, there isn’t a standard set of rules for carrying out this biopsy in patients with suspected LE.

In some cases, doctors will use a procedure called an esophagogastroduodenoscopy to look at the inside of your esophagus. Symptoms of LE vary a lot, so your doctor might notice anything from a perfectly normal-looking esophagus to severe tightening or rings, inflated patches, or white patches. Interestingly, in about one-quarter to one-half of patients with LE, the lining of the esophagus looks completely normal under the endoscope.

Recent studies have tried to zoom in closer and enhance certain features of the esophagus using a technique called narrow-band imaging magnifying endoscopy (NBI-ME). This technique can hone in on subtle features like changes in the color of the esophageal tissue and abnormally looped blood vessels. However, only about 82% of patients with LE show these features, compared to 100% of patients with eosinophilic esophagitis, which is a similar condition.

Even without these visual clues, doctors can look at the biopsy under a microscope to help diagnose LE. There isn’t a consensus on the number of white blood cells, specifically lymphocytes, needed in a biopsy to diagnose LE. However, more than 20 lymphocytes in a microscopic field, a lack of other types of white blood cells, granulocytes, and evidence of damage to the tissues like cell swelling (spongiosis) are widely accepted as signs of LE.

Other types of imaging tests like a barium swallow test can look for any structural problems in your esophagus that could explain your symptoms, such as severe inflammation, narrowing, a hiatal hernia (where the top part of your stomach pokes through the diaphragm), or cancer. But these tests don’t play a significant role in diagnosing LE itself.

Once you’re confirmed to have LE, especially if there don’t seem to be any visible abnormalities in your esophagus, your doctor may suggest further tests to evaluate for conditions that cause abnormal muscular contractions in your esophagus, like achalasia (a condition where the muscle at the bottom of your esophagus doesn’t relax properly) and other similar conditions. The best test for this is high-resolution esophageal manometry (HREM). If your doctor suspects you may also have a rheumatological (disease involving joints, muscles, and bones) or autoimmune (where your body mistakenly attacks its own cells) condition based on your medical history and physical exam, they’ll ask for more tests to confirm this.

Treatment Options for Lymphocytic Esophagitis

Treating Lymphocytic Esophagitis (LE), a condition that affects the esophagus, is still a bit undefined because there aren’t any clear clinical guidelines yet. The way LE is managed currently is very similar to how eosinophilic esophagitis – another condition that affects the esophagus – is treated, due to the similarities in clinical symptoms and appearance of the esophagus under endoscopy. That said, LE is more likely to respond positively to medications than eosinophilic esophagitis. The effect of systemic steroids and biological therapy (medications that help your immune system to fight illnesses) on patients with LE hasn’t been studied yet.

Regarding lifestyle changes, it’s recommended that patients with LE who smoke receive education and support to quit smoking.

The medical treatment for LE usually involves high doses of proton pump inhibitors (PPI), a type of medicine that reduces stomach acid, taken twice daily for 12 weeks. After this therapy, doctors will evaluate changes in the patient’s symptoms or the appearance of their esophagus under endoscopy. If the PPI therapy doesn’t lead to any improvements, a topical steroid called budesonide, taken twice daily, is recommended. Most patients report feeling better after the start of PPI therapy. It’s not clear if this improvement results from treating underlying gastroesophageal reflux disease (GERD – a condition in which stomach acid frequently flows back into the tube connecting your mouth and stomach). One small study including 22 patients with confirmed LE found that 40% of them reached remission with high-dose PPI therapy, but we’ll need larger studies to confirm this finding.

Regarding endoscopic therapeutic interventions, these are procedures that include the use of an endoscope, a flexible tube that helps doctors examine your esophagus. Doctors recommend repeated endoscopic dilation for patients with LE if they still have symptoms and esophageal strictures (narrowing) or rings (circles of hard tissue) despite medication. Dilation is a procedure that would stretch and widen the esophagus.

What else can Lymphocytic Esophagitis be?

When doctors are trying to diagnose LE (lower esophageal issues), they consider several other conditions that might cause similar symptoms. These conditions include:

• Gastroesophageal reflux disease (acid reflux)
• Erosive esophagitis (inflammation that damages the esophagus)
• Candida esophagitis (a yeast infection in the esophagus)
• Eosinophilic esophagitis (an allergic reaction that inflames the esophagus)
• Esophageal lichen planus (a rare skin condition that can affect the esophagus)
• Achalasia (a condition that makes it hard for the esophagus to move food into the stomach)
• Non-achalasia esophageal motility disorder (other conditions that affect the movement of food through the esophagus)
• Peptic stricture (narrowing of the esophagus due to damage from stomach acid)
• Esophageal neoplasm (tumors or cancer in the esophagus)
• IgG4-related esophagitis (an inflammatory condition that can affect various parts of the body, including the esophagus).

What to expect with Lymphocytic Esophagitis

There isn’t a lot of information on what typically happens over time to people with a condition called LE. However, according to how patients react to a standard high-dose medication therapy with a drug type known as PPI, it seems like the condition is long-lasting but generally harmless.² A study looking back at the experiences of 81 patients with LE suggests that most of these patients claim to have a good quality of life².

Diagnosing LE early and keeping an eye out for complications like narrowing of the esophagus is critical to prevent long-term health problems.

Possible Complications When Diagnosed with Lymphocytic Esophagitis

The side effects of Lower Esophageal Sphincter (LE) problems can consist of issues like narrowings in the esophagus, thin tissue growths blocking the esophagus, and instances where the esophagus develops a hole.

Potential Side Effects:

• Narrowings in the Esophagus (esophageal strictures)
• Thin tissue growths blocking the esophagus (esophageal webs)
• Holes developing in the esophagus (esophageal perforation)

Preventing Lymphocytic Esophagitis

Things that you can change, or “modifiable risk factors”, for developing a condition called LE (Lichen Esophagitis, a chronic condition affecting the tube connecting your mouth to your stomach) include smoking and having GERD (Gastroesophageal Reflux Disease, a chronic and more severe form of acid reflux). If you have LE and use tobacco products, the doctor will educate you and provide supporting ways for you to quit.

The treatment of LE usually involves high-dose PPI (Proton Pump Inhibitor) therapy, which decreases the stomach acid that can worsen GERD. This medication also manages any symptoms caused by GERD. Moreover, the doctor will guide you to make certain lifestyle modifications to reduce the impacts of GERD. This might mean avoiding certain foods that trigger acid reflux symptoms, not lying down or reclining until at least three hours after you eat, steering clear of late-night snacks, and elevating the head of your bed during sleep.

The doctor will educate you that while LE is a long-term disease, it’s not life-threatening. To manage this condition, it’s crucial to follow the medication and lifestyle modification strategies that your doctor suggests, keep track of your symptoms, and attend all check-up appointments. These appointments might also include repeat inspections of your esophagus and possible biopsies (procedures where small samples are taken from your body to examine them more closely).