Abstract
We present a case of a 23-year-old gentleman who presented with dysphagia, weight loss, and recurrent esophageal strictures requiring multiple dilatations. An endoscopic ultrasound with esophagogastroduodenoscopy revealed a mass present in the distal esophagus. Fine needle aspiration suggested that the mass in the lower esophagus resembled a gastrointestinal stromal tumor. After surgical resection, final pathologic analysis revealed that the tumor was comprised of benign-appearing fibroinflammatory cells with an increase and predominance of IgG4-positive plasma cells. The microscopic appearance was consistent with a benign condition as a result of an IgG4-related process. He did not, however, have any other symptoms indicative of systemic autoimmune disease or connective tissue disorders. We present the pre-operative imaging, operative management, pathologic diagnosis, and literature review of this rare condition and the first known report of autoimmune esophagitis as part of the IgG4 spectrum of diseases.
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References
Schneider A, Merikhi A, Frank B. Autoimmune disorders: gastrointestinal manifestations and endoscopic findings. Gastrointest Endoscopy Clin N Am 2006;16:133–151.
Kim T, Grobmyer SR, Dixon LR, Hochwald SN. Isolated lymphoplasmacytic sclerosing pancreatitis involving the pancreatic tail. Am Surg 2008;74:654–658.
Kim T, Grobmyer SR, Dixon LR, Allan RW, Hochwald SN. Autoimmune pancreatitis and concurrent small lymphocytic lymphoma: not just a coincidence? J Gastrointest Surg 2008;12:1566–1570.
Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, Fukushima M, Nikaido T, Nakayama K, Usuda N, Kiyosawa K. High serum IgG4 concentrations in patients with sclerosing pancreatitis. NEJM 2001;344:732–738.
Hirano K, Komatsu Y, Yamamoto N, Nakai Y, Sasahira N, Toda N, Isayama H, Tada M, Kawabe T, Omata M. Pancreatic mass lesions associated with raised concentration of IgG4. Am J Gastroenterol 2004;99:2038–2040.
Sarles H, Sarles JC, Muratore R, Guien C. Chronic inflammatory sclerosis of the pancreas—an autonomous pancreatic disease? Am J Dig Dis 1961;6:688–698.
Raina A, Yadav D, Krasinskas AM, McGrath KM, Khalid A, Sanders M, Whitcomb DC, Slivka A. Evaluation and management of autoimmune pancreatitis: experience at a large US center. Am J Gastroenterol 2009;104:2295–2306.
Kamisawa T, Satake K. Clinical management of autoimmune pancreatitis. Adv Med Sci 2007;52:61–65.
Okazaki K, Uchida K, Matsushita M, Takaoka M. How to diagnose autoimmune pancreatitis by the revised Japanese clinical criteria. J Gastroenterol 2007;42:32–38.
Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001;344:732–738.
Hochwald SN, Hemming AW, Draganov P, Vogel SB, Dixon LR, Grobmyer SR. Elevation of serum IgG4 in western patients with autoimmune sclerosing pancreatocholangitis: a word of caution. Ann Surg Oncol 2008;15:1147–1154.
Fitzgerald RC, Triadafiliopoulos G. Esophageal manifestations of rheumatic disorders. Semin Arthritis Rheum 1997;26:641–666.
Gutierrez F, Valenzuela JE, Ehresmann GR, Quismorio FP, Kitridou RC. Esophageal dysfunction in patients with mixed connective tissue diseases and systemic lupus erythematosus. Dig Dis Sci 1982;27:592–597.
Volter F, Fain O, Mathieu E, Thomas M. Esophageal function and Sjogrens syndrome. Dig Dis Sci 2004;49:248–253.
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Lopes, J., Hochwald, S.N., Lancia, N. et al. Autoimmune Esophagitis: IgG4-related Tumors of the Esophagus. J Gastrointest Surg 14, 1031–1034 (2010). https://doi.org/10.1007/s11605-010-1172-4
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DOI: https://doi.org/10.1007/s11605-010-1172-4