IgG4-related disease involving the esophagus: a clinicopathological study

Obiorah, I.; Hussain, A.; Palese, C.; Azumi, N.; Benjamin, S.; Ozdemirli, M.
December 2017
Diseases of the Esophagus;Dec2017, Vol. 30 Issue 12, p1
Academic Journal
Immunoglobulin G4 (IgG4)-related disease is a recently coined systemic disease characterized by specific histopathologic findings of an intense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis in the presence of predominant IgG4-positive plasma cells. Although IgG4-related disease has been described in many organs, involvement of the esophagus is very rare. In this study, we describe the clinicopathologic characteristics of eight patients with IgG4-related esophagitis. We evaluated chronic esophagitis specimens with lymphoplasmacytic infiltrate obtained over the past 6 years (from January 2011 to February 2017) using a chart review, pathologic examination, and IgG4 immunohistochemical staining. The diagnoses of the specimens were either confirmed as IgG4-related esophagitis (IgG4-RE) or chronic esophagitis, not otherwise specified (CENOS), and the clinicopathologic data from each group were compared. Eight patients were diagnosed with IgG4-RE and 10 controls were identified and diagnosed with CENOS. In the IgG4-RE group, esophageal strictures were identified in three patients, two patients had postmyotomy treated achalasia, one patient had erosive esophagitis and another presented with an esophageal nodule. Only one patient had an unremarkable mucosa on endoscopy. In the CENOS group, four patients had esophageal strictures, six had erosive esophagitis, one patient had mild esophagitis. The IgG4-RE group had significantly higher numbers of IgG4-positive plasma cells (66.9 ± 21.9 vs. 4.7 ± 2.4 per high power field; P < 0.001) and a greater IgG4: IgG ratio 0.76 ± 0.13 vs. 0.06 ± 0.05; P < 0.001) when compared to CENOS patients. Two of the patients with recurrent esophageal strictures in the IgG4-RE group showed initial response to steroid therapy and are currently on immunosuppressive therapy which has significantly reduced the need for multiple esophageal dilatations. The presentation of IgG4-related esophageal disease can vary and the key to diagnosis is dependent on histopathology. These observations highlight the need for IgG4 immunohistochemical staining of esophageal biopsies especially in patients with mucosal ulceration, chronic inflammation, and plasmacytosis on biopsy. This will prevent unwarranted esophagectomies and failed medical treatment due to lack of recognition of this entity.


Related Articles

  • Eosinophilic Esophagitis and Barrett’s Esophagus: An Occasional Association or an Overlap Disease? Francalanci, Paola; De Angelis, Paola; Minnei, Francesca; Camassei, Francesca Diomedi; Torroni, Filippo; Dall'Oglio, Luigi; Callea, Francesco // Digestion;2008, Vol. 77 Issue 1, p16 

    Background: Esophageal diseases are common in infants and children, and may present with several clinical and pathological aspects. Eosinophilic esophagitis (EE) is characterized by inflammatory, predominantly eosinophilic infiltrate (≥15 eosinophils per high-power field (HPF)) that is not...

  • Resection of Benign Esophageal Stricture through a Minimally Invasive Endoscopic and Transgastric Approach. Lucktong, T.A.; Morton, John M.; Shaheen, Nicholas J.; Farrell, Timothy M. // American Surgeon;Aug2002, Vol. 68 Issue 8, p720 

    Recurrent benign esophageal strictures that are refractory to dilation can be difficult to manage. We report a novel technique for treatment of a recurrent esophageal stricture using a minimally invasive endoscopic and transgastric approach. The patient is a 40-year-old women who developed a...

  • S, M, L, XL …. Wang, T.; Triadafilopoulos, G. // Gut;Jan2003, Vol. 52 Issue 1, p5 

    Reports on methods of surveillance for Barrett's esophagus. Improvement of the small degree of resolution and tissue penetration to medium, large or even extra large levels of refinement, sensitivity and specificity; Use of a standard video endoscope in chromoendoscopy; Detection of...

  • EXPRESSION ANALYSIS OF THE METAPLASIA DYSPLASIA CARCINOMA SEQUENCE IN BARRETT'S OESOPHAGUS AND ADENOCARCINOMA. Penman, I.D.; Smith, V.; Shen, E.F.; Wieand, D.; Landon, T.H.; Wong, N.A.C.S.; Lessells, A.M.; Paterson-Brown, S.; Tang, J.Z.; Wu, T.; Hillan, K.J. // Gut;Apr2003 Supplement 1, Vol. 52, pA7 

    Introduction and Aims: The molecular genetic events involved in the metaplasia dysplasia carcinoma (MDC) sequence in Barrett's oesophagus (CLO) are incompletely understood. We applied microarray expression analysis of endoscopic biopsies to study further these events and to detect novel genes...

  • Black esophagus: should it be considered an unfavorable prognostic factor? Casella, G.; Perego, D.; Corti, G.; Cambareri, A. R.; Buda, C. A.; Zoldan, C.; Baldini, V. // Diseases of the Esophagus;Apr2001, Vol. 14 Issue 2, p166 

    Black esophagus is a rare condition, reported for the first time in 1990. It is always noted in severely compromised patients. The diagnosis is possible using endoscopy. An esophageal ischemic injury should be considered. It is important that a differential diagnosis is made with consideration...

  • TP53 mutations in malignant and premalignant Barrett's esophagus. Dolan, K.; Walker, S. J.; Gosney, J.; Field, J. K.; Sutton, R. // Diseases of the Esophagus;Jun2003, Vol. 16 Issue 2, p83 

    SUMMARY In order to improve the efficacy of endoscopic surveillance of Barrett's esophagus, markers of neoplastic progression in addition to dysplasia are required. The aim of the present study was to assess TP53 mutational analysis as a method of identifying patients with Barrett's esophagus...

  • Temporal Changes in the Endoscopic Frequency of New Cases of Barrett's Esophagus in an Australian Health Region. Kendall, Bradley J.; Whiteman, David C. // American Journal of Gastroenterology;Jun2006, Vol. 101 Issue 6, p1178 

    OBJECTIVES: The number of patients diagnosed with Barrett's esophagus (BE) has increased in recent decades, although data from populations outside Europe and North America are scarce. This increase has significant implications for health resource utilization and costs. We sought to determine...

  • Immunodepressive serum treatment of acute heart transplant rejection. Coulson, Alan S.; Zweitman, Virginia H.; Stinson, Edward B.; Griepp, Randall B.; Bieber, Charles P.; Dong, Eugene; Shumway, Norman E. // British Medical Journal;3/27/1976, Vol. 1 Issue 6012, p749 

    Examines the efficacy of immunodepressive serum treatment of acute heart transplant rejection. Reduction of lymphocyte activity in vitro; Measurement of proteins within the serum sample; Role of immunoglobulin G as an immunodepressive protein.

  • The relationship between anti-HPV-16 IgG seropositivity and cancer of the cervix, anogenital organs, oral cavity and pharynx, oesophagus and prostate in a black South African population. Sitas, Freddy; Urban, Margaret; Stein, Lara; Beral, Valerie; Ruff, Paul; Hale, Martin; Patel, Moosa; O'Connell, Dianne; Xue Qin Yu; Verzijden, Anke; Marais, Dianne; Williamson, Anna-Lise // Infectious Agents & Cancer;2007, Vol. 2, p6 

    Background: Human papillomavirus type 16 (HPV-16) infection is an important cause of cervical cancer, other anogenital cancers and, possibly, some oral and pharyngeal cancers. The association of HPV-16 with oesophageal and with prostate cancers has not been firmly established. Methods: We...


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics