IgG4-Related Esophageal Stenosis Treated With Serial Endoscopic Dilation and Concurrent Steroid Injection

Introduction: IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated condition characterized by IgG4-positive plasma cell infiltrate, classically involving the pancreas, bile ducts, and lacrimal or salivary glands. IgG4-RD affecting the esophagus (IgG4-RD-E) is less common and data on treatment is limited. We report a case of IgG4-RD-E treated successfully with serial dilation and steroid injection. Case Description/Methods: An 81-year-old male with history of hypertension and diabetes was referred to endoscopy for progressive dysphagia. Initial exam showed a 6 mm ulcerated stricture at the GE junction (Figure 1a), which was traversed using neonatal scope. Given the concern of malignancy, we obtained multiple biopsies and did not perform dilation. Biopsies showed increased IgG4 plasma cells (>100/HPF) and 40% IgG4-positive/all plasma cell ratio, suggestive of IgG4-RD-E. Endoscopy was repeated with dilation to 8 mm using balloon dilator. Repeat biopsies showed similar findings. Serum IgG4 level was normal and anti-nuclear antibody was negative. Stricture was dilated to 12 mm on a following EGD and patient was started on prednisone 40 mg with slow taper, however this was discontinued due to worsening of blood pressure and glucose level. Repeat endoscopy 3 months later showed worsening of the stricture with a diameter of 8 mm. Due to persistence of symptomatic stricture and intolerance of systemic steroids, we attempted dilation with steroid injection. Over the following 6 months, patient underwent three EGDs with injection of triamcinolone 40 mg. There was no evidence of stricture worsening and we were able to dilate to 15 mm (Figure 1c). Patient’s symptoms completely resolved at 6 and 12 months follow-up and decision was made to repeat EGD only if symptoms reoccur. Discussion: With the small number of published cases, information on treating IgG4-RD-E is limited. Systemic steroids, serial endoscopic dilation, or esophagectomy are the reported options, but may be inapplicable due to patient age or comorbidities. Intralesional steroids have been described in treating refractory strictures, but not particularly in IgG4-RD-E. Given the rarity of this condition, clinical trials are difficult to achieve, thus we feel it is useful to report this case. Our patient had infrequent endoscopies (months apart), but still showed clinical and endoscopic response with steroid injection. This option is appealing as it avoids systemic side effects and may counteract the underlying immune process of the disease.Figure 1.: Figure A represents manometric findings on opioids and was initially diagnosed as type III achalasia. Figure B represents repeat testing off opioids and demonstrates a significant improvement in esophageal motility.