IgG4-Related Disease with Esophageal Involvement

Abstract

We report the case of a 56-year-old man who presented with dysphagia and weight loss. An esophagoduodenoscopy revealed a severe esophageal stricture in the distal esophagus. After surgical resection, the final pathologic analysis revealed that the tumor was comprised of benign-appearing fibroinflammatory cells with an increase and predominance of IgG4-positive plasma cells. He did not, however, have any other symptoms indicative of systemic autoimmune disease or connective tissue disorders. Histologically, abundant infiltration of IgG4-positive plasma cells and lymphocytes was observed. The patient was diagnosed with IgG4-related disease, definitive, with esophageal involvement.

Novel and rare forms of oesophagitis

“…In each case this was significantly greater than seen in control subjects. The case reports provide similar findings to this study, demonstrating 10–200 IgG4 + plasma cells/HPF (median >50) and an IgG4:IgG ratio ranging from 30 to >90% (median = 60%) 44–50,52,53 …”

“…43 IgG4-related oesophagitis is exceedingly rare in the literature, with a handful of case reports and one case-series to date. [44][45][46][47][48][49][50][51][52][53] Awareness and recognition of IgG4-related disease is important due to its propensity to be misdiagnosed as malignancy, based on its clinical and radiological appearance. 44 IgG4-related oesophagitis should be considered in unexplained strictures of the oesophagus, as treatment with steroids may prevent unnecessary oesophagectomies.…”

“…The case reports provide similar findings to this study, demonstrating 10-200 IgG4 + plasma cells/HPF (median >50) and an IgG4:IgG ratio ranging from 30 to >90% (median = 60%). [44][45][46][47][48][49][50]52,53…”

“…Other endoscopic findings include subepithelial mass lesions, 33,48,52 oesophageal rings, 51 oesophageal nodules 51 and sloughing mucosa reminiscent of oesophagitis dissecans superficialis (ODS) 46 . CT imaging demonstrates mass lesions or thickening of the oesophageal wall with oedema in the majority of cases 44–50,52,53 …”

“…Involvement of the biliary tract, lymph nodes, salivary glands, kidneys, thyroid and skin has also been described 43 . IgG4‐related oesophagitis is exceedingly rare in the literature, with a handful of case reports and one case‐series to date 44–53 . Awareness and recognition of IgG4‐related disease is important due to its propensity to be misdiagnosed as malignancy, based on its clinical and radiological appearance 44 .…”