Montefiore Medical Center Yonkers, NY, United States
Carlos J. Figueredo, MD1, Tamoor Shahid, MD2, Jiani Chai, MD, PhD2, Hilary I. Hertan, MD, FACG2 1Montefiore Medical Center, Yonkers, NY; 2Montefiore Medical Center, Bronx, NY
Introduction: Immunoglobulin G4-related disease (IgG4-RD) encompasses a variety of clinical manifestations in the gastroesophageal anatomy. From 2010 to 2018 only 39 cases have been published in the GI system, out of which 9 have related to esophageal involvement. We present a case of IgG4 related disease eosinophilic esophagitis (EoE), and autoimmune (AI) pancreatitis.
Case Description/Methods: 44-year-old with a history of diabetes, presented after the new onset of food bolus impaction with the sensation of choking, confirmed by CT scan. Emergent upper endoscopy provided instant relief. Mucosa findings were consistent with EoE (Figure 1a, b). Laboratory analysis was only significant for increased eosinophil count. Esophageal biopsy confirmed EoE diagnosis (Figure 1c, d). Proton pump inhibitor was started. Moreover, an incidental pancreatic mass was seen on imaging, suspicious for malignancy. Ca19-9 and CEA levels were normal. Endoscopic ultrasound-guided fine-needle aspiration resulted negative for malignancy (Figure 1e, f). Further workup revealed isolated elevated IgG4 levels of 195.6 mg/dL. Therefore, the patient was started on oral steroids for possible autoimmune pancreatitis and discharged with close follow-up.
Discussion: IgG4 related disease (IgG4-RD) consists of an AI systemic inflammatory and fibrotic disorder with potential malignant transformation. It presents with variable eosinophil count elevation. IgG4-RD can present as a solitary mass lesion or as a multifocal disease. Its involvement in the pancreas has been previously well established. However, esophageal involvement of IgG4-RD has rarely been described in the literature. IgG4 -RD with esophageal involvement has been previously described with associated ulceration and stricture. However, recent studies have shown dense IgG4 plasma cell infiltration in the deep lamina proposal fibrosis in EoE, suggesting the possibility of food-specific EoE. Additional studies have shown intrasquamous IgG4 deposits in 16 out 21 eosinophilic esophagitis patients, representing a valuable marker to distinct eosinophilic esophagitis from gastroesophageal reflux disease. According to the latest guidelines in 2015, IgG4 levels greater than 135 mg/dl are consistent with the diagnosis. Corticosteroids are the first line in therapy followed by steroid-sparing agents for refractory disease. Our case represented an unusual IgG4-RD presentation with an unusual organ involvement with an atypical disease form which has rarely been reported previously in the literature.
Figure: Figure 1 a, b. Esophageal trachealization (a) Linear furrows (b) in the middle third of the esophagus suggestive of eosinophilic esophagitis. Figure 1 c, d. Squamous epithelium with intraepithelial eosinophils, reactive epithelial changes, and focal surface erosion. Figure 1 e, f. Pancreatic head mass fine-needle aspiration. Pancreatic head mass fine-needle aspiration biopsy showing pancreatic epithelial cells. Prominent nuclei are seen in scattered cells. A few lymphocytes are seen in the background.
Carlos Figueredo indicated no relevant financial relationships.
Tamoor Shahid indicated no relevant financial relationships.
Jiani Chai indicated no relevant financial relationships.
Hilary Hertan indicated no relevant financial relationships.
Carlos J. Figueredo, MD1, Tamoor Shahid, MD2, Jiani Chai, MD, PhD2, Hilary I. Hertan, MD, FACG2. P1416 - IgG4-Related Disease: An Uncommon Location, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.