P3351 - Recurrent IgG4 Plasma Cell Esophagitis in a Healthy Young Man

Brian W. McSteen, BS, MD¹, Donevan Westerveld, MD², David Wan, BS, MD^3
1New York-Presbyterian/Weill Cornell Campus, New York, NY; ²New York-Presbyterian/Weill Cornell Medicine, New York, NY; ³Weill Cornell Medicine, New York, NY

Introduction: Immunoglobulin G4 (IgG4) plasma cell esophagitis is a rare manifestation of systemic IgG4-related disease, with pathologic findings of dense lymphocytic inflammation with IgG4 plasma cells, storiform fibrosis, and obliterative phlebitis. Further characterization of this rare condition is valuable for clinical recognition.

Case Description/Methods: An 18 year-old man with a history of mononucleosis presented with acute-onset epigastric pain for three days, preceded by two weeks of fatigue, night sweats, 13 pound unintentional weight loss, and fever. The patient described an aching epigastric pain, worse with oral intake. Labs showed white blood cells of 9.28 (10^3 cells/uL, 3.98 - 10.04), hemoglobin of 13.5 (g/dL, 13.7 - 17.5), Lipase 44 (U/L, 12 - 53), SARS-CoV-2 antibody positive and SARS-CoV-2 PCR test negative. Abdominal ultrasound showed no acute findings. Esophagogastroduodenoscopy revealed three 8mm cratered clean-based esophageal ulcers with no stigmata of recent bleeding. Pathology was notable for increased IgG4-positive plasma cells, attributed to reactive inflammation in the setting of COVID-19. The patient was discharged with gastroenterology and pediatrics follow-up.

The patient presented again 3 years later with 2 days of fever and substernal and epigastric pain associated with dyspnea and burning, exacerbated by oral intake. Lab results were largely within normal limits. Repeat esophagogastroduodenoscopy showed congested, eroded, hemorrhagic-appearing mucosa in the esophagus and stomach. Pathology showed increased IgG4/ IgG ratio, with >50 IgG4+ plasma cells/ HPF. The patient was prescribed viscous lidocaine, sucralfate, PPI and oral inhaled fluticasone and counseled to followup with PCP for serum IgG4 testing.

Discussion: IgG4 plasma cell esophagitis presents with non-specific symptoms, including odynophagia, dysphagia, and weight loss. Typical endoscopic findings include ulceration, stricture, and submucosal tumor. Given the non-specific symptoms of IgG4 plasma cell esophagitis, diagnosis and treatment can be delayed. Diagnosis should be correlated with serum IgG4. As IgG4 plasma cell esophagitis is thought to cause chronic recurrent inflammatory injury, delayed diagnosis and treatment increases the risk for fibrotic changes, complex strictures and recurrent procedures. Corticosteroids are the mainstay of treatment. Further characterization of this patient’s course will be an important contribution to learning about this rare condition.


Disclosures:


Brian W. McSteen, BS, MD¹, Donevan Westerveld, MD², David Wan, BS, MD³. P3351 - Recurrent IgG4 Plasma Cell Esophagitis in a Healthy Young Man, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.