Abstract
Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized immune-mediated condition that results in inflammation, stricturing and mass formation. It causes a wide spectrum of disease and clinical presentations depending on the organ system involved. Isolated esophageal IgG4-RD is rare and diagnosis can be difficult. It is highly responsive to corticosteroids, and early identification and instigation of management is key.
We describe the case of a 47-year-old man who presented with a food bolus obstruction on a background of progressive dysphagia and weight loss. Imaging and gastroscopy demonstrated diffuse esophageal thickening with a benign appearing stricture. Following non-specific histologic findings on biopsy and a non-diagnostic endoscopic ultrasound guided fine needle aspiration, he underwent video-assisted thoracoscopic surgery with esophageal core biopsy. This confirmed the diagnosis of IgG4-RD. Initial treatment was with corticosteroids. However, due to recurrence of symptoms upon weaning of corticosteroids, azathioprine maintenance therapy was instituted. Azathioprine has previously been used in systemic cases of IgG4-RD but has not been reported for isolated esophageal disease.
This case highlights the difficulties in the diagnosis and treatment of esophageal IgG4-RD and the need to consider it as a differential diagnosis when histology reveals esophagitis with lymphoplasmacytic infiltration.
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Abbreviations
- IgG4:
-
Immunoglobulin G4
- IgG4-RD:
-
Immunoglobulin G4-related disease
- HPF:
-
High-power field
- CT:
-
Computed tomography
- EUS:
-
Endoscopic ultrasound
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HP, SF and JAH were involved in conceptualization, data curation and manuscript writing and critical appraisal. PM assisted in the diagnosis and provided representative histology slides. PM, AM and ML provided professional comments and critically revised the manuscript. All authors approved the final version of the manuscript.
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Poole, H., Fehily, S., McKelvie, P. et al. Isolated esophageal IgG4-related disease presenting with progressive dysphagia and weight loss in a middle-aged man. Clin J Gastroenterol 15, 526–530 (2022). https://doi.org/10.1007/s12328-022-01623-7
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DOI: https://doi.org/10.1007/s12328-022-01623-7