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The American Gastroenterological Association (AGA) has released an expert review and clinical practice update focusing on esophageal dysfunction caused by immune-mediated and infectious diseases.

“Many different disorders can lead to esophageal dysfunction, which is characterized by symptoms including dysphagia, odynophagia, chest pain and heartburn. These symptoms can be caused either by immune or infectious conditions and can either be localized to the esophagus or part of a larger systemic process,” co–first author Emily McGowan, MD, PhD, with the Division of Allergy and Immunology, University of Virginia School of Medicine, Charlottesville, Virginia, said in an AGA podcast. 

However, without a “high index of suspicion,” these conditions can be overlooked, leading to delays in diagnosis and unnecessary procedures. “With this clinical practice update, we wanted to help providers more readily recognize these conditions so that patients can be diagnosed and treated earlier in the course of their disease,” McGowan explained. 

“This is a fantastic review that highlights how many different systemic disorders can affect the esophagus,” Scott Gabbard, MD, gastroenterologist and section head Charlottesville, who wasn’t involved in the review, told Medscape Medical News.

“Honestly, for the practicing gastroenterologist, this is one of those reviews that I could envision someone either saving to his or her desktop for reference or printing it and pinning it next to his or her desk,” Gabbard said.

Best Practice Advice

The clinical practice update is published in Clinical Gastroenterology and Hepatology. It includes 10“best practice advice” statements and a table highlighting “important” considerations when evaluating patients with esophageal dysfunction.

The review authors note that esophageal dysfunction may result from localized infections — most commonly Candida, herpes simplex virus and cytomegalovirus — or systemic immune-mediated diseases, such as systemic sclerosis (SSc), mixed connective tissue disease (MCTD), and eosinophilic esophagitis (EoE).

They advise clinicians to identify if there are risks for inflammatory or infectious possibilities for a patient’s esophageal symptoms and investigate for these disorders as a potential cause of esophageal dysfunction.

Once esophageal infection is identified, it’s important to identify whether accompanying signs and symptoms point to immunocompromise leading to a more systemic infection. Consultation with an infectious disease expert is recommended to guide appropriate treatment, the authors said.

If symptoms fail to improve after therapy for infectious esophagitis, the patient should be evaluated for refractory infection or additional underlying sources of esophageal and immunologic dysfunction is advised.

It’s also important to recognize that patients with EoE who continue to have symptoms of esophageal dysfunction despite histologic and endoscopic disease remission, may develop a motility disorder and evaluation of esophageal motility may be warranted, the authors said. 

In patients with histologic and endoscopic features of lymphocytic esophagitis, treatment of lymphocytic-related inflammation with proton-pump inhibitor (PPI) therapy or swallowed topical corticosteroids and esophageal dilation as needed should be considered.

In patients who present with esophageal symptoms in the setting of hypereosinophilia (absolute eosinophil count > 1500 cells/uL), the authors advise further work-up of non-EoE eosinophilic gastrointestinal disease, hypereosinophilic syndrome, and eosinophilic granulomatosis with polyangiitis should be considered, with consultation with an allergy/immunology specialist if helpful.

In patients with rheumatologic diseases, especially SSc and MCTD, it’s important to be aware that esophageal symptoms can occur due to involvement of the esophageal muscle layer, resulting in dysmotility and/or incompetence of the lower esophageal sphincter, they said.

In the setting of Crohn’s disease, some patients can develop esophageal involvement from inflammation, stricturing, or fistulizing changes with granulomas seen histologically. Esophageal manifestations of Crohn’s disease tend to occur in patients with active intestinal disease.

In patients with dermatologic diseases of lichen planus or bullous disorders, dysphagia can occur due to endoscopically visible esophageal mucosal involvement. Esophageal lichen planus, in particular, can occur without skin involvement and can be difficult to define on esophageal histopathology.

The authors also advise clinicians to consider infectious and inflammatory causes of secondary achalasia during initial evaluation.

“Achalasia and EoE might coexist more commonly than what gastroenterologists think, especially in younger patients,” co–first author Chanakyaram Reddy, MD, gastroenterologist with Baylor University Medical Center, Dallas, Texas, said in the podcast. 

He noted that in a recent population-based study, the estimated relative risk of EoE was over 30-fold higher in patients with achalasia aged ≤ 40 years. 

“In any suspected achalasia case, it would be wise to obtain biopsies throughout the entire esophagus when the patient is off confounding medications such as PPI therapy to establish if significant esophageal eosinophilia is coexistent,” Reddy said.

“If EoE level eosinophilia is found, it would be reasonable to consider treating medically for EoE prior to committing to achalasia-specific interventions, which often involve permanent disruption of the esophageal muscle layer,” he added.

Gabbard said this review helps the clinician think beyond gastroesophageal reflux disease (GERD) — the most common cause of esophageal dysfunction — and consider other causes for esophageal dysfunction. 

“We are seeing more complex disorders affect the esophagus. It’s not just GERD and you absolutely need a high index of suspicion because you can find varying disorders to blame for many esophageal symptoms that could otherwise be thought to be just reflux,” he told Medscape Medical News.

This research had no commercial funding. Disclosures for the authors are listed with the original article. Gabbard had no relevant disclosures.