Lymphocytic esophagitis

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Lymphocytic esophagitis
Lymphocytic esophagitis histology.jpg
Pathology image of lymphocytic esophagitis (H&E stain). The image demonstrates common findings in the condition: dense peripapillary lymphocytic infiltrate with spongiosis (arrow), and absence of neutrophils and eosinophils.
Specialty Gastroenterology
Symptoms difficulty swallowing, heartburn, abdominal pain, food bolus obstruction
Complications esophageal stricture
Usual onsetunknown
Durationlong term
Causesunknown
Diagnostic method biopsy of the esophagus showing characteristic findings
Differential diagnosis gastroesophageal reflux disease, eosinophilic esophagitis
Medication corticosteroids, such as prednisone and budesonide
Prognosis unknown
Frequencyuncertain; adults 0.1%, as high as 8.5% in children

Lymphocytic esophagitis is a rare and poorly understood medical disorder involving inflammation in the esophagus. The disease is named from the primary inflammatory process, wherein lymphocytes are seen within the esophageal mucosa. Symptoms of the condition include difficulty swallowing, heartburn and food bolus obstruction. The condition was first described in 2006 by Rubio and colleagues. Initial reports questioned whether this was a true medical disorder, or whether the inflammation was secondary to another condition, such as gastroesophageal reflux disease.

Contents

The cause of lymphocytic esophagitis is unknown. The disease may cause different symptoms and be caused by different processes in childhood as compared to adulthood. Some studies have shown that it is associated with either other medical conditions involving the esophagus, including gastroesophageal reflux disease and achalasia, or other inflammatory conditions such as Crohn's disease, coeliac disease, and allergic conditions. The diagnosis is based upon a biopsy of the mucosa of the esophagus, showing a characteristic appearance of inflammation involving lymphocytes, and the relative absence of another group of inflammatory cells, granulocytes.

Complications of the disorder include stricture of the esophagus, which can lead to food bolus obstruction, and weight loss. Treatment of lymphocytic esophagitis includes medications meant to target the esophagus topically, such as budesonide, but also procedures to deal with complications, such as esophageal dilation.

Signs and symptoms

The most common symptom of lymphocytic esophagitis is dysphagia, or difficulty swallowing, prevalent in 53 to 57% of individuals with the condition [1] [2] due to inflammation, narrowing or altered movement of the esophagus. Other symptoms include heartburn, abdominal pain, nausea and food bolus obstruction. [1] [2] Some patients may have no symptoms, as was found in a significant percentage of patients in the first description of the condition, [3] although more recent reports indicate that the absence of symptoms is uncommon. [1]

Complications

Image of a piece of food obstructing the esophagus, a complication in lymphocytic esophagitis Food bolus obstruction.jpg
Image of a piece of food obstructing the esophagus, a complication in lymphocytic esophagitis

Food bolus impaction is a common complication of lymphocytic esophagitis. [1] This occurs when food is acutely obstructing the esophagus at an area of narrowing. It typically requires an endoscopic procedure in order to remove or dislodge the obstructing food.[ citation needed ]

Cause

The cause of lymphocytic esophagitis is unknown. [4] Attempts to better understand the cause of lymphocytic esophagitis include identification of other diseases that associate with the condition. [4] These conditions include other esophageal conditions including gastroesophageal reflux disease [1] [4] [5] and achalasia, [5] as well as other inflammatory diseases such as Crohn's disease, [4] [5] coeliac disease, [4] and allergic conditions such as eczema. [4] Additionally use of tobacco may associate with lymphocytic esophagitis. [1] [4] Lymphocytic esophagitis does not occur with high frequency in other gastrointestinal conditions where lymphocytosis is found in the mucosa, including lymphocytic colitis and lymphocytic gastritis; however, there is a disease association with coeliac disease wherein lymphocytic inflammation occurs in the small bowel after exposure to gluten. [4]

Pathophysiology

Little is known about the pathophysiology of lymphocytic esophagitis. [4] It has been hypothesized that lymphocytic esophagitis may be either a primary inflammatory disorder, or that the inflammation is triggered by an external stimulus leading to injury of the mucosal lining of the esophagus, such as gastric acid in GERD. [4] [6]

Diagnosis

Endoscopic image of lymphocytic esophagitis, demonstrating narrow lumen esophagus (left), linear furrows (right) and esophageal rings (right) Lymphocytic esophagitis endoscopy.jpg
Endoscopic image of lymphocytic esophagitis, demonstrating narrow lumen esophagus (left), linear furrows (right) and esophageal rings (right)

The diagnosis of lymphocytic esophagitis is made by biopsy of the mucosal lining of the esophagus. This is typically achieved at the time of esophagogastroduodenoscopy, a medical procedure wherein an endoscope is inserted through the mouth, into the esophagus, in order to visualize and biopsy the mucosa.[ citation needed ]

While the histologic changes in the biopsies are characterized by the presence of an inflammatory infiltrate, consisting primarily of lymphocytes in the absence of other inflammatory cells such as granulocytes, the criteria for making the diagnosis are still unclear. [1] [4] The location of the biopsies, cutoff of number of lymphocytes found in each high-power field of view of the microscope, the presence of spongiosis, and the need for immunohistochemical staining to define lymphocytes are all unclear still. [1]

While the diagnosis of lymphocytic esophagitis depends on the biopsy results, certain changes can be visualized directly at the time of endoscopy. The esophagus may be narrow in calibre, [5] may show multiple rings, [5] redness, [5] linear furrows [1] or the mucosal lining may slide demonstrating a "crepe-paper" appearance. [1] Complications such as strictures of the esophagus can also be detected with endoscopy. [5] These changes are very similar to those found in eosinophilic esophagitis, a more common and better understood esophageal disorder thought to be of allergic origin. [5] Narrow-band imaging with magnification endoscopy is another imaging modality that can show characteristic changes of lymphocytic esophagitis. [7]

Management

The treatment of lymphocytic esophagitis is still undefined. [5] Treatment either targets symptoms, inflammation or complications. For example, heartburn is a symptom in lymphocytic esophagitis, and proton pump inhibitors, which reduce acidity in the stomach, are consequently used for treatment. With respect to treatment of inflammation, steroids that are topical and coat the lining of the esophagus, such as budesonide have been used to treat the condition, in one German study. [8] Prednisone has also been used to treat the inflammation of lymphocytic esopahgitis. [5] With respect to complications, strictures of the esophagus can be treated with esophageal dilation. [5]

Prognosis

There has been little studied about the natural history of lymphocytic esophagitis. [4] The longest study of the condition involved a median follow-up of 3.3 years, wherein participants were surveyed, and demonstrated that 87% of patients were alive but that the vast majority (97%) of patients still had symptoms. [4] [9] There have been two reports of esophageal perforation associated with the condition, one case occurring during endoscopic treatment of complications. [4]

Epidemiology

The percentage of individuals with lymphocytic esophagitis is uncertain. [1] Early studies indicated that the prevalence was 0.1% in adults. [3] However, reports in children indicate a higher prevalence of 8.5%, [10] perhaps suggestive of incidental findings of lymphocytes in endoscopies performed for other reasons. [1] The disease may occur more frequently in women over the age of 60. [4]

There is increasing awareness of lymphocytic esophagitis among physicians, perhaps affecting the prevalence over time. [1] The majority of the studies on lymphocytic esophagitis are after 2015. [1]

History

The condition was first described in a series of 20 patients at the Karolinska Institutet by Carlos Rubio in 2006. [3] Much of the reports of the condition over the subsequent several years were focused on ascertaining whether lymphocytic esophagitis was a true condition, and if it was, establishing uniform criteria for its diagnosis. [1] [2] [4]

Research

The majority of the research into lymphocytic esophagitis has been performed after 2015. [1] There remain many questions unanswered about the condition, such as establishing its natural history, [4] clarifying its diagnostic criteria, [1] and ascertaining the best treatment for the condition. [4]

See also

Related Research Articles

<span class="mw-page-title-main">Gastroenterology</span> Branch of medicine focused on the digestive system and its disorders

Gastroenterology is the branch of medicine focused on the digestive system and its disorders. The digestive system consists of the gastrointestinal tract, sometimes referred to as the GI tract, which includes the esophagus, stomach, small intestine and large intestine as well as the accessory organs of digestion which include the pancreas, gallbladder, and liver. The digestive system functions to move material through the GI tract via peristalsis, break down that material via digestion, absorb nutrients for use throughout the body, and remove waste from the body via defecation. Physicians who specialize in the medical specialty of gastroenterology are called gastroenterologists or sometimes GI doctors. Some of the most common conditions managed by gastroenterologists include gastroesophageal reflux disease, gastrointestinal bleeding, irritable bowel syndrome, inflammatory bowel disease (IBD) which includes Crohn's disease and ulcerative colitis, peptic ulcer disease, gallbladder and biliary tract disease, hepatitis, pancreatitis, colitis, colon polyps and cancer, nutritional problems, and many more.

Peptic ulcer disease (PUD) is a break in the inner lining of the stomach, the first part of the small intestine, or sometimes the lower esophagus. An ulcer in the stomach is called a gastric ulcer, while one in the first part of the intestines is a duodenal ulcer. The most common symptoms of a duodenal ulcer are waking at night with upper abdominal pain, and upper abdominal pain that improves with eating. With a gastric ulcer, the pain may worsen with eating. The pain is often described as a burning or dull ache. Other symptoms include belching, vomiting, weight loss, or poor appetite. About a third of older people have no symptoms. Complications may include bleeding, perforation, and blockage of the stomach. Bleeding occurs in as many as 15% of cases.

<span class="mw-page-title-main">Esophageal achalasia</span> Rare, incurable, progressive motility disorder due to failure of esophogeal motor neurons

Esophageal achalasia, often referred to simply as achalasia, is a failure of smooth muscle fibers to relax, which can cause the lower esophageal sphincter to remain closed. Without a modifier, "achalasia" usually refers to achalasia of the esophagus. Achalasia can happen at various points along the gastrointestinal tract; achalasia of the rectum, for instance, may occur in Hirschsprung's disease. The lower esophageal sphincter is a muscle between the esophagus and stomach that opens when food comes in. It closes to avoid stomach acids from coming back up. A fully understood cause to the disease is unknown, as are factors that increase the risk of its appearance. Suggestions of a genetically transmittable form of achalasia exist, but this is neither fully understood, nor agreed upon.

Heartburn, also known as pyrosis, cardialgia or acid indigestion, is a burning sensation in the central chest or upper central abdomen. Heartburn is usually due to regurgitation of gastric acid into the esophagus. It is the major symptom of gastroesophageal reflux disease (GERD).

<span class="mw-page-title-main">Gastroesophageal reflux disease</span> Medical condition

Gastroesophageal reflux disease (GERD) or gastro-oesophageal reflux disease (GORD) is one of the upper gastrointestinal chronic diseases in which stomach content persistently and regularly flows up into the esophagus, resulting in symptoms and/or complications. Symptoms include dental corrosion, dysphagia, heartburn, odynophagia, regurgitation, non-cardiac chest pain, extraesophageal symptoms such as chronic cough, hoarseness, reflux-induced laryngitis, or asthma. In the long term, and when not treated, complications such as esophagitis, esophageal stricture, and Barrett's esophagus may arise.

<span class="mw-page-title-main">Barrett's esophagus</span> Medical condition

Barrett's esophagus is a condition in which there is an abnormal (metaplastic) change in the mucosal cells lining the lower portion of the esophagus, from stratified squamous epithelium to simple columnar epithelium with interspersed goblet cells that are normally present only in the small intestine and large intestine. This change is considered to be a premalignant condition because it is associated with a high incidence of further transition to esophageal adenocarcinoma, an often-deadly cancer.

<span class="mw-page-title-main">Esophagitis</span> Medical condition

Esophagitis, also spelled oesophagitis, is a disease characterized by inflammation of the esophagus. The esophagus is a tube composed of a mucosal lining, and longitudinal and circular smooth muscle fibers. It connects the pharynx to the stomach; swallowed food and liquids normally pass through it.

<span class="mw-page-title-main">Hiatal hernia</span> Type of hernia

A hiatal hernia or hiatus hernia is a type of hernia in which abdominal organs slip through the diaphragm into the middle compartment of the chest. This may result in gastroesophageal reflux disease (GERD) or laryngopharyngeal reflux (LPR) with symptoms such as a taste of acid in the back of the mouth or heartburn. Other symptoms may include trouble swallowing and chest pains. Complications may include iron deficiency anemia, volvulus, or bowel obstruction.

Coffee ground vomitus refers to a particular appearance of vomit. Within organic heme molecules of red blood cells is the element iron, which oxidizes following exposure to gastric acid. This reaction causes the vomitus to look like ground coffee.

<span class="mw-page-title-main">Plummer–Vinson syndrome</span> Medical condition

Plummer–Vinson syndrome is a rare disease characterized by difficulty swallowing, iron-deficiency anemia, glossitis, cheilosis and esophageal webs. Treatment with iron supplementation and mechanical widening of the esophagus generally provides an excellent outcome.

<span class="mw-page-title-main">Microscopic colitis</span> Medical condition

Microscopic colitis refers to two related medical conditions which cause diarrhea: collagenous colitis and lymphocytic colitis. Both conditions are characterized by the presence of chronic non-bloody watery diarrhea, normal appearances on colonoscopy and characteristic histopathology findings of inflammatory cells.

<span class="mw-page-title-main">Eosinophilic esophagitis</span> Allergic inflammatory condition of the esophagus

Eosinophilic esophagitis (EoE) is an allergic inflammatory condition of the esophagus that involves eosinophils, a type of white blood cell. In healthy individuals, the esophagus is typically devoid of eosinophils. In EoE, eosinophils migrate to the esophagus in large numbers. When a trigger food is eaten, the eosinophils contribute to tissue damage and inflammation. Symptoms include swallowing difficulty, food impaction, vomiting, and heartburn.

Esophageal dysphagia is a form of dysphagia where the underlying cause arises from the body of the esophagus, lower esophageal sphincter, or cardia of the stomach, usually due to mechanical causes or motility problems.

<span class="mw-page-title-main">Esophageal candidiasis</span> Medical condition

Esophageal candidiasis is an opportunistic infection of the esophagus by Candida albicans. The disease usually occurs in patients in immunocompromised states, including post-chemotherapy and in AIDS. However, it can also occur in patients with no predisposing risk factors, and is more likely to be asymptomatic in those patients. It is also known as candidal esophagitis or monilial esophagitis.

<span class="mw-page-title-main">Nutcracker esophagus</span> Medical condition

Nutcracker esophagus, jackhammer esophagus, or hypercontractile peristalsis, is a disorder of the movement of the esophagus characterized by contractions in the smooth muscle of the esophagus in a normal sequence but at an excessive amplitude or duration. Nutcracker esophagus is one of several motility disorders of the esophagus, including achalasia and diffuse esophageal spasm. It causes difficulty swallowing, or dysphagia, with both solid and liquid foods, and can cause significant chest pain; it may also be asymptomatic. Nutcracker esophagus can affect people of any age but is more common in the sixth and seventh decades of life.

<span class="mw-page-title-main">Esophageal food bolus obstruction</span> Medical condition

An esophageal food bolus obstruction is a medical emergency caused by the obstruction of the esophagus by an ingested foreign body.

<span class="mw-page-title-main">Acute esophageal necrosis</span> Medical condition

Acute esophageal necrosis (AEN), black esophagus, or Gurvits syndrome is a rare esophageal disorder. AEN defines itself with dark pigmentation of the esophagus, found during an upper gastrointestinal endoscopy. Pigmentation is usually black friable mucosa. The disorder is extremely rare, as only 89 patients over a span of 40 years have received this diagnosis. Specific study of the disorder's mortality rate is mentioned at 31.8%, but new research suggests mortality rates vary from 30–50%. The exact triggering mechanism for this disorder is still unknown, but is likely multifactorial.

<span class="mw-page-title-main">Esophageal pH monitoring</span>

Esophageal pH monitoring is the current gold standard for diagnosis of gastroesophageal reflux disease (GERD). It provides direct physiologic measurement of acid in the esophagus and is the most objective method to document reflux disease, assess the severity of the disease and monitor the response of the disease to medical or surgical treatment. It can also be used in diagnosing laryngopharyngeal reflux.

<span class="mw-page-title-main">Herpes esophagitis</span> Medical condition

Herpes esophagitis is a viral infection of the esophagus caused by Herpes simplex virus (HSV).

<span class="mw-page-title-main">Esophageal intramural pseudodiverticulosis</span> Medical condition

Esophageal intramucosal pseudodiverticulosis (EIPD) is a rare condition wherein the wall of the esophagus develops numerous small outpouchings (pseudodiverticulae). Individuals with the condition typically develop difficulty swallowing. The outpouchings represent the ducts of submucosal glands of the esophagus. It typically affects individuals in their sixth and seventh decades of life. While it is associated with certain chronic conditions, particularly alcoholism, diabetes and gastroesophageal reflux disease, the cause of the condition is unknown. Treatment involves medications to treat concomitant conditions such as reflux and esophageal spasm, and dilation of strictures in the esophagus.

References

  1. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 Habbal M, Scaffidi MA, Rumman A, Khan R, Ramaj M, Al-Mazroui A, Abunassar MJ, Jeyalingam T, Shetty A, Kandel GP, Streutker CJ, Grover SC (October 2018). "Clinical, endoscopic, and histologic characteristics of lymphocytic esophagitis: a systematic review". Esophagus. 16 (2): 123–132. doi:10.1007/s10388-018-0649-1. PMID   30370453. S2CID   53092746.
  2. 1 2 3 Haque S, Genta RM (August 2012). "Lymphocytic oesophagitis: clinicopathological aspects of an emerging condition". Gut. 61 (8): 1108–14. doi:10.1136/gutjnl-2011-301014. PMID   22157333. S2CID   206955866.
  3. 1 2 3 Rubio CA, Sjödahl K, Lagergren J (March 2006). "Lymphocytic esophagitis: a histologic subset of chronic esophagitis". Am. J. Clin. Pathol. 125 (3): 432–7. doi: 10.1309/7lablgy08uem3h26 . PMID   16613348.
  4. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 Rouphael C, Gordon IO, Thota PN (February 2017). "Lymphocytic esophagitis: Still an enigma a decade later". World J. Gastroenterol. 23 (6): 949–956. doi: 10.3748/wjg.v23.i6.949 . PMC   5311104 . PMID   28246468.
  5. 1 2 3 4 5 6 7 8 9 10 11 Pasricha S, Gupta A, Reed CC, Speck O, Woosley JT, Dellon ES (October 2016). "Lymphocytic Esophagitis: An Emerging Clinicopathologic Disease Associated with Dysphagia". Dig. Dis. Sci. 61 (10): 2935–2941. doi:10.1007/s10620-016-4230-2. PMC   5021567 . PMID   27343035.
  6. ↑ Purdy JK, Appelman HD, Golembeski CP, McKenna BJ (October 2008). "Lymphocytic esophagitis: a chronic or recurring pattern of esophagitis resembling allergic contact dermatitis". Am. J. Clin. Pathol. 130 (4): 508–13. doi: 10.1309/D3PCF6D6YYMQRX9A . PMID   18794041.
  7. ↑ Tanaka K, Rubio CA, Dlugosz A, Truskaite K, Befrits R, Lindberg G, Schmidt PT (October 2013). "Narrow-band imaging magnifying endoscopy in adult patients with eosinophilic esophagitis/esophageal eosinophilia and lymphocytic esophagitis". Gastrointest. Endosc. 78 (4): 659–64. doi:10.1016/j.gie.2013.05.025. PMID   23827349.
  8. ↑ Paparoupa M, Linnemüller S, Schuppert F (November 2017). "[Off-label use of Budesonide suspensions to treat a patient with lymphocytic esophagitis]". Z Gastroenterol (in German). 55 (11): 1127–1130. doi:10.1055/s-0043-117187. PMID   28787751. S2CID   256830276.
  9. ↑ Cohen S, Saxena A, Waljee AK, Piraka C, Purdy J, Appelman H, McKenna B, Elmunzer BJ, Singal AG (2012). "Lymphocytic esophagitis: a diagnosis of increasing frequency". J. Clin. Gastroenterol. 46 (10): 828–32. doi:10.1097/MCG.0b013e3182500de8. PMC   3465631 . PMID   22751335.
  10. ↑ Sutton LM, Heintz DD, Patel AS, Weinberg AG (August 2014). "Lymphocytic esophagitis in children". Inflamm. Bowel Dis. 20 (8): 1324–8. doi:10.1097/MIB.0000000000000100. PMID   24983984. S2CID   27039021.
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