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Clinical Practice Update

Esophageal dysfunction due to disordered immunity and infection

Best practices for diagnostic considerations of immune-mediated disorders to consider when treating patients with dysphagia, heartburn, and odynophagia.

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Best practice advice 

  1. Gastroenterologists should be aware of the esophageal manifestations of systemic immunologic and infectious diseases to reduce diagnostic delay. Clinicians should 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. 
  2. Gastroenterologists should be aware of the esophageal manifestations of systemic immunologic and infectious diseases to reduce diagnostic delay. Clinicians should 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. 
  3. Gastroenterologists should be aware of the esophageal manifestations of systemic immunologic and infectious diseases to reduce diagnostic delay. Clinicians should 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. 
  4. In individuals with eosinophilic esophagitis (EoE) who continue to experience symptoms of esophageal dysfunction despite histologic and endoscopic disease remission, clinicians should be aware that some patients with EoE may develop motility disorders. Further evaluation of esophageal motility may be warranted. 
  5. In individuals with histologic and endoscopic features of lymphocytic esophagitis, clinicians should consider treatment of lymphocytic-related inflammation with proton-pump inhibitor therapy or swallowed topical corticosteroids and as needed esophageal dilation. 
  6. In patients who present with esophageal symptoms in the setting of hypereosinophilia (absolute eosinophil count [AEC] >1500 cells/uL), consider further work-up of non-EoE eosinophilic gastrointestinal (GI) disease, hypereosinophilic syndrome, and eosinophilic granulomatosis with polyangiitis (EGPA). Consultation with allergy/immunology may help guide further diagnostic work-up and treatment. 
  7. In individuals with rheumatologic diseases of systemic sclerosis (SSc), mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), or Sjogren’s disease, clinicians should 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. The degree of dysfunction is often especially significant in those with SSc or MCTD. 
  8. In individuals with Crohn’s disease, clinicians should be aware that a minority of individuals can develop esophageal involvement from inflammatory, stricturing, or fistulizing changes with granulomas seen histologically. Esophageal manifestations of Crohn’s disease tend to occur in individuals with active intestinal disease. 
  9. In individuals with dermatologic diseases of lichen planus or bullous disorders, clinicians should be aware that 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. 
  10. Clinicians should consider infectious and inflammatory causes of secondary achalasia during initial evaluation. One should query for any history of recent COVID infections, risks for Chagas disease, and symptoms or signs of eosinophilic disease. 

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