Eosinophilic Esophagitis Remains Enigma in Adults : Dysphagia, the most dominant symptom in adults, may be intermittent—or catastrophic in some cases.

LOS ANGELES — Eosinophilic esophagitis appears to be a different disease in adults than it is in children, sharing similar pathophysiologic features and perhaps an allergic etiology, but displaying a different pattern of symptoms, Dr. David A. Katzka said at the annual Digestive Disease Week.

“Certainly this is a new kid on the block, with many of us feeling this is a totally new disease,” said Dr. Katzka, director of the swallowing program at the University of Pennsylvania in Philadelphia.

It is generally agreed that eosinophilia is on the rise, paralleling patterns seen with asthma, atopic dermatitis, and other allergy-driven diseases.

Children with eosinophilic esophagitis complain of a “plethora” of symptoms, including nausea and vomiting, epigastric pain, heartburn, and dysphagia.

“In adults, by far and away, the most dominant symptom is dysphagia,” Dr. Katzka stressed. “It may be intermittent. It may be catastrophic,” he said, noting that it has become a highly prevalent cause of food impaction, despite the fact the disease was only described in 1993.

At first believed to be a variant of gastroesophageal reflux disease (GERD), the disease is clearly a distinct entity with a genetic component in some families. On the other hand, many adults with the disease have GERD symptoms and some respond to aggressive acid suppression with proton pump inhibitors, making the connection between the two entities vexing.

In both children and adults, the diagnosis is pathologic and based on an ill-defined elevated rate of eosinophils found in a patchy pattern in the esophageal mucosa.

Most pathologists now consider 20 eosinophils per high-powered field to be diagnostic, “but clearly there's a spectrum here,” Dr. Katzka said.

He emphasized the importance of taking multiple biopsies, since there may be 5 eosinophils per high-powered field in one spot, and “50 in another.”

Longitudinal furrows are very common findings on endoscopy in all age groups.

In addition, rings throughout the esophagus and strictures are commonly seen in adults (and less often, in children) even on radiographic films.

A small caliber esophagus and extreme mucosal fragility may also be present.

Eosinophilic abscesses, visualized as white specks, are seen in 17%–20% of children with the disease and are “almost pathopneumonic” in adults.

Finally, the esophagus may have a “firm, woody feel,” Dr. Katzka said.

Debate rages as to whether a person with a normal-appearing esophagus can have the disease. Dr. Katzka said he believes it is possible.

The peripheral eosinophil count is normal in about 90% of patients.

Although studies have not been done in adults to direct management, Dr. Katzka recommends RAST testing, patch testing, and skin testing to try to identify an allergen or combination of allergens that may be responsible. However, he warned of an “imprecise correlation between skin, blood, and esophageal findings” and said some adult patients do not respond to avoidance of known allergens.

The biggest problem is convincing patients to avoid foods that may be contributing to the condition.

“It is very hard to convince teens and adults to go on an elemental diet and avoid pizza and beer and all of these things they like to eat on a regular basis,” he said.

In children, treatment with steroids, leukotriene inhibitors, and mast cell stabilizers have been shown effective.

In adults, “we're flying by the seat of our pants” in regard to treatment, he said.

He recommends a 2-month course of fluticasone propionate and possibly, maintenance with montelukast, noting that some specialists also suggest a 1–2 month course of proton pump inhibitors prior to performing a second endoscopy.

Dilation of the esophagus may be indicated, but should be attempted with caution. Adults may experience severe chest pain, tearing, or perforation, and endoscopists may encounter diffuse wall fibrosis.

“We will treat these patients very aggressively for 2 months before thinking about dilation,” he said.

In children, eosinophilic esophagitis seems to stabilize and improve over time, while in adults, limited studies suggest it persists or worsens.

“One of our fears is that in adults, this is a progressive or static disease that has to be recognized and treated early.”

But there are so many unanswered questions about the disease in adults that easy recognition is difficult and the best course of treatment uncertain, for now.

Clearly, some patients accommodate dysphagia for years by chewing their food carefully and avoiding difficult-to-swallow foods such as meats and bread. Others, however, seem to have suffered no symptoms during childhood or young adulthood.

“It's striking to look at individual patients diagnosed at 62 years old or 53 years old. Was this person born with the disease and didn't feel it until he was 62 years old? Do we have a silent, slowly progressive disease that [begins in childhood but] doesn't become apparent until later in life, or is this really a different disease in some patients? We don't know,” he said.

LOS ANGELES — Eosinophilic esophagitis appears to be a different disease in adults than it is in children, sharing similar pathophysiologic features and perhaps an allergic etiology, but displaying a different pattern of symptoms, Dr. David A. Katzka said at the annual Digestive Disease Week.

“Certainly this is a new kid on the block, with many of us feeling this is a totally new disease,” said Dr. Katzka, director of the swallowing program at the University of Pennsylvania in Philadelphia.

It is generally agreed that eosinophilia is on the rise, paralleling patterns seen with asthma, atopic dermatitis, and other allergy-driven diseases.

Children with eosinophilic esophagitis complain of a “plethora” of symptoms, including nausea and vomiting, epigastric pain, heartburn, and dysphagia.

“In adults, by far and away, the most dominant symptom is dysphagia,” Dr. Katzka stressed. “It may be intermittent. It may be catastrophic,” he said, noting that it has become a highly prevalent cause of food impaction, despite the fact the disease was only described in 1993.

At first believed to be a variant of gastroesophageal reflux disease (GERD), the disease is clearly a distinct entity with a genetic component in some families. On the other hand, many adults with the disease have GERD symptoms and some respond to aggressive acid suppression with proton pump inhibitors, making the connection between the two entities vexing.

In both children and adults, the diagnosis is pathologic and based on an ill-defined elevated rate of eosinophils found in a patchy pattern in the esophageal mucosa.

Most pathologists now consider 20 eosinophils per high-powered field to be diagnostic, “but clearly there's a spectrum here,” Dr. Katzka said.

He emphasized the importance of taking multiple biopsies, since there may be 5 eosinophils per high-powered field in one spot, and “50 in another.”

Longitudinal furrows are very common findings on endoscopy in all age groups.

In addition, rings throughout the esophagus and strictures are commonly seen in adults (and less often, in children) even on radiographic films.

A small caliber esophagus and extreme mucosal fragility may also be present.

Eosinophilic abscesses, visualized as white specks, are seen in 17%–20% of children with the disease and are “almost pathopneumonic” in adults.

Finally, the esophagus may have a “firm, woody feel,” Dr. Katzka said.

Debate rages as to whether a person with a normal-appearing esophagus can have the disease. Dr. Katzka said he believes it is possible.

The peripheral eosinophil count is normal in about 90% of patients.

Although studies have not been done in adults to direct management, Dr. Katzka recommends RAST testing, patch testing, and skin testing to try to identify an allergen or combination of allergens that may be responsible. However, he warned of an “imprecise correlation between skin, blood, and esophageal findings” and said some adult patients do not respond to avoidance of known allergens.

The biggest problem is convincing patients to avoid foods that may be contributing to the condition.

“It is very hard to convince teens and adults to go on an elemental diet and avoid pizza and beer and all of these things they like to eat on a regular basis,” he said.

In children, treatment with steroids, leukotriene inhibitors, and mast cell stabilizers have been shown effective.

In adults, “we're flying by the seat of our pants” in regard to treatment, he said.

He recommends a 2-month course of fluticasone propionate and possibly, maintenance with montelukast, noting that some specialists also suggest a 1–2 month course of proton pump inhibitors prior to performing a second endoscopy.

Dilation of the esophagus may be indicated, but should be attempted with caution. Adults may experience severe chest pain, tearing, or perforation, and endoscopists may encounter diffuse wall fibrosis.

“We will treat these patients very aggressively for 2 months before thinking about dilation,” he said.

In children, eosinophilic esophagitis seems to stabilize and improve over time, while in adults, limited studies suggest it persists or worsens.

“One of our fears is that in adults, this is a progressive or static disease that has to be recognized and treated early.”

But there are so many unanswered questions about the disease in adults that easy recognition is difficult and the best course of treatment uncertain, for now.

Clearly, some patients accommodate dysphagia for years by chewing their food carefully and avoiding difficult-to-swallow foods such as meats and bread. Others, however, seem to have suffered no symptoms during childhood or young adulthood.

“It's striking to look at individual patients diagnosed at 62 years old or 53 years old. Was this person born with the disease and didn't feel it until he was 62 years old? Do we have a silent, slowly progressive disease that [begins in childhood but] doesn't become apparent until later in life, or is this really a different disease in some patients? We don't know,” he said.

LOS ANGELES — Eosinophilic esophagitis appears to be a different disease in adults than it is in children, sharing similar pathophysiologic features and perhaps an allergic etiology, but displaying a different pattern of symptoms, Dr. David A. Katzka said at the annual Digestive Disease Week.

“Certainly this is a new kid on the block, with many of us feeling this is a totally new disease,” said Dr. Katzka, director of the swallowing program at the University of Pennsylvania in Philadelphia.

It is generally agreed that eosinophilia is on the rise, paralleling patterns seen with asthma, atopic dermatitis, and other allergy-driven diseases.

Children with eosinophilic esophagitis complain of a “plethora” of symptoms, including nausea and vomiting, epigastric pain, heartburn, and dysphagia.

“In adults, by far and away, the most dominant symptom is dysphagia,” Dr. Katzka stressed. “It may be intermittent. It may be catastrophic,” he said, noting that it has become a highly prevalent cause of food impaction, despite the fact the disease was only described in 1993.

At first believed to be a variant of gastroesophageal reflux disease (GERD), the disease is clearly a distinct entity with a genetic component in some families. On the other hand, many adults with the disease have GERD symptoms and some respond to aggressive acid suppression with proton pump inhibitors, making the connection between the two entities vexing.

In both children and adults, the diagnosis is pathologic and based on an ill-defined elevated rate of eosinophils found in a patchy pattern in the esophageal mucosa.

Most pathologists now consider 20 eosinophils per high-powered field to be diagnostic, “but clearly there's a spectrum here,” Dr. Katzka said.

He emphasized the importance of taking multiple biopsies, since there may be 5 eosinophils per high-powered field in one spot, and “50 in another.”

Longitudinal furrows are very common findings on endoscopy in all age groups.

In addition, rings throughout the esophagus and strictures are commonly seen in adults (and less often, in children) even on radiographic films.

A small caliber esophagus and extreme mucosal fragility may also be present.

Eosinophilic abscesses, visualized as white specks, are seen in 17%–20% of children with the disease and are “almost pathopneumonic” in adults.

Finally, the esophagus may have a “firm, woody feel,” Dr. Katzka said.

Debate rages as to whether a person with a normal-appearing esophagus can have the disease. Dr. Katzka said he believes it is possible.

The peripheral eosinophil count is normal in about 90% of patients.

Although studies have not been done in adults to direct management, Dr. Katzka recommends RAST testing, patch testing, and skin testing to try to identify an allergen or combination of allergens that may be responsible. However, he warned of an “imprecise correlation between skin, blood, and esophageal findings” and said some adult patients do not respond to avoidance of known allergens.

The biggest problem is convincing patients to avoid foods that may be contributing to the condition.

“It is very hard to convince teens and adults to go on an elemental diet and avoid pizza and beer and all of these things they like to eat on a regular basis,” he said.

In children, treatment with steroids, leukotriene inhibitors, and mast cell stabilizers have been shown effective.

In adults, “we're flying by the seat of our pants” in regard to treatment, he said.

He recommends a 2-month course of fluticasone propionate and possibly, maintenance with montelukast, noting that some specialists also suggest a 1–2 month course of proton pump inhibitors prior to performing a second endoscopy.

Dilation of the esophagus may be indicated, but should be attempted with caution. Adults may experience severe chest pain, tearing, or perforation, and endoscopists may encounter diffuse wall fibrosis.

“We will treat these patients very aggressively for 2 months before thinking about dilation,” he said.

In children, eosinophilic esophagitis seems to stabilize and improve over time, while in adults, limited studies suggest it persists or worsens.

“One of our fears is that in adults, this is a progressive or static disease that has to be recognized and treated early.”

But there are so many unanswered questions about the disease in adults that easy recognition is difficult and the best course of treatment uncertain, for now.

Clearly, some patients accommodate dysphagia for years by chewing their food carefully and avoiding difficult-to-swallow foods such as meats and bread. Others, however, seem to have suffered no symptoms during childhood or young adulthood.

“It's striking to look at individual patients diagnosed at 62 years old or 53 years old. Was this person born with the disease and didn't feel it until he was 62 years old? Do we have a silent, slowly progressive disease that [begins in childhood but] doesn't become apparent until later in life, or is this really a different disease in some patients? We don't know,” he said.