Chronic ulcerative oesophagitis rich in IgG4-positive plasma cells – a distinct clinicopathological entity

Abstract

AIMS : We present a series of patients with chronic, ulcerative oesophagitis refractory to proton-pump inhibitor therapy and rich in IgG4-positive plasma cells that do not strictly meet criteria for IgG4-related disease; aiming to characterize the clinical and histological pattern of this disorder. METHODS AND RESULTS : We retrieved cases of chronic ulcerative oesophagitis rich in IgG4-positive plasma cells, diagnosed from 2019 to 2025. Histological and immunohistochemical slides were re-evaluated and findings correlated with clinical data, including patients' symptoms, endoscopic findings and follow-up information. A total of 12 patients with a mean age of 63.1 years were included. Dysphagia was the most common symptom (9/12 patients), followed by heartburn (8/12 patients). Serum levels of IgG4 were elevated in four patients, in three accompanied by increased IgG levels. Other organ involvement by IgG4-RD was not observed. Endoscopy showed ulceration in 100% and stricture in 50% of cases, respectively; the lower third of the oesophagus was most commonly affected. Histology illustrated dense plasma cell-rich infiltration with a mean number of IgG4-positive plasma cells of 70/HPF (range 25–100) and a mean IgG4/IgG ratio of 73% (range 50%–90%). Corticosteroids led to improvement of symptoms in 8/9 and histological remission in 4/5 patients, respectively. CONCLUSION : Chronic ulcerative oesophagitis rich in IgG4-positive plasma cells may exert a severe clinical impact on affected individuals but appears to respond well to corticosteroid therapy. This case series demonstrates clinicopathological findings of the disorder and highlights the importance of IgG4 immunohistochemistry in therapy-refractory cases of chronic oesophageal ulceration with dense plasma cell-rich inflammation.