IgG4 sclerosing disease of the esophagus: a case-based review
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Rheumatology International https://doi.org/10.1007/s00296-020-04594-5
INTERNATIONAL
CASES WITH A MESSAGE
IgG4 sclerosing disease of the esophagus: a case‑based review Jessica Joanne Padniewski1 · Gabby Thottam2 · Rawad Nasr1,3 Received: 21 January 2020 / Accepted: 19 April 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract IgG4-related disease (IgG4-RD) is an inflammatory and fibrosing disease which causes tumor-like swelling of organs and commonly mimics symptoms of malignancy. It has been increasing in prevalence in the last decade, but esophageal involvement remains rare. IgG4-RD was first known to involve certain organs, such as the pancreas. It has, since, been described as a systemic disease process. IgG4-RD should be considered in patients presenting with dysphagia. Initiation of appropriate treatment with corticosteroids can avoid unnecessary procedures and improve outcomes. The aim of this review is to discuss 17 cases of IgG4-RD of the esophagus. Literature review was conducted using NCBI database (PMC and PubMed filters) using the keywords “IgG4 disease,” “sclerosing,” “esophagus” and “gastrointestinal.” The search was narrowed to include cases describing IgG4 disease of the esophagus using the same filters. Literature review identified 16 documented cases of IgG4-RD involving the esophagus. Upon literature review, it remains clear that it is extremely rare for IgG4-RD to affect the esophagus. Sixteen cases have been reported. We present a 17th case and discuss the implications of IgG4-RD. It is important to keep a broad differential diagnosis that includes IgG4-RD for patients presenting with dysphagia, especially when symptoms are refractory. Keywords IgG4-related disease · IgG4 infiltration · Eosinophilic infiltrate · Autoimmune disease · Dysphagia · Esophageal mass · Sclerosis
Introduction IgG4-related disease (IgG4-RD) has been increasing in prevalence in the last decade, but esophageal involvement of IgG4-RD remains rare. IgG4-RD was first described in cases of autoimmune pancreatitis [1]. It was not recognized as a systemic disease until 2003, at which time, extrapancreatic manifestations were identified in certain patients with IgG4 autoimmune pancreatitis [1]. Since that time, many * Jessica Joanne Padniewski [email protected] Gabby Thottam [email protected] Rawad Nasr [email protected] 1
Internal Medicine Residency, Hennepin Healthcare, Minneapolis, Minnesota, USA
2
Rheumatology Fellowship Program, University of Minnesota, Minneapolis, Minnesota, USA
3
Rheumatology Division, Department of Medicine, Hennepin Healthcare, Minneapolis, Minnesota, USA
new developments have been made in our understanding of IgG4-RD. IgG4-RD is an inflammatory and fibrosing disease that causes tumor-like swelling of organs and commonly mimics symptoms of malignancy [1]. IgG4-RD processes commonly respond to corticosteroid treatment, which further confirms the suspicion that it is an immune mediated process [1]. The key morphologic features of IgG4-RD are e
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