Chronic Sclerosing Sialadenitis of the Submandibular Gland as the Initial Symptom of IgG4-Related Disease: A Case Report

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition accompanied by tumefactive lesions, dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis in various organs, and, frequently, elevated serum IgG4 levels. Chronic sclerosing sialadenitis (also termed Küttner's tumor) is thought to be a lesion of IgG4-RD; thus, IgG4-related sialadenitis may be the initial symptom of IgG4-RD. We herein report a 64-year-old Japanese female with IgG4-related chronic sclerosing sialadenitis of the right submandibular gland and retroperitoneal fibrosis, who subsequently developed tubulointerstitial nephritis and pancreatitis. She was referred to our Department for treatment of swelling of the right submandibular gland; preoperative imaging studies suggested a malignant tumor. We extirpated the submandibular glands bilaterally and diagnosed IgG4-related chronic sclerosing sialadenitis pathologically. Subsequently, the patient's serum IgG4 concentration increased, and lesions in the retroperitoneum, kidney, and pancreas were confirmed by imaging. Although the radiological characteristics of these lesions mimicked malignancy, steroid treatment was commenced based on the pathology of the submandibular gland and elevated serum IgG4 level. This caused the lesions to disappear, indicating that the patient had experienced IgG4-related retroperitoneal fibrosis, tubulointerstitial nephritis, and pancreatitis. No relapse was detected for 4 years 8 months after surgery. A pathological diagnosis is crucial to exclude the possibility of malignancy and to make treatment decisions when lesions are evident in other organs. In addition, periodic evaluation of the serum IgG4 concentration and imaging of the whole body are warranted in long-term follow-up.

Immunoglobulin G4–related disease mimicking an epidural spinal cord tumor: case report

“…15 IgG4-RD often affects multiple organ systems, most typically causing sialadenitis, pancreatitis, and/or lymphadenopathy. 6,16,26 Common findings in IgG4-RD include an elevated serum IgG4 level (with a ratio of IgG4/IgG greater than 40%), infiltration of organs with IgG4-positive plasma cells, and fibrosis of the affected organs. 6,7,16 In the presented case, the patient’s IgG4 IHC staining was on the lower end of the results reported in other cases affecting the CNS, at 10 cells/hpf.…”

“…13 There are case reports in which masses that have the radiological appearance of cancerous tumors are subsequently biopsied and demonstrate no malignant cells, instead showing the histopathological characteristics consistent with IgG4-RD. 20,26 These lymphoplasmacytic lesions tend to affect solid organs such as the pancreas, thyroid, lung, and kidney. 1,13,26 …”

“…20,26 These lymphoplasmacytic lesions tend to affect solid organs such as the pancreas, thyroid, lung, and kidney. 1,13,26 …”

“…Given that lesions caused by IgG4-RD are almost always responsive to steroid treatment, physicians must keep the condition in mind when evaluating a possibly malignant lesion. Though there are case reports of IgG4-RD in every area of the body, 1,4,6,7,9,13–14,16,18,21–23,26 involvement of the central nervous system (CNS) is quite rare. Here, we present a case of IgG4-RD manifesting as a cervical paraspinal mass that responded well to steroid treatment.…”

Sonographic findings of IgG4‐related disease of the salivary glands: Case report and review of the literature

“…Of the remaining 12 articles, 3 were duplicates. Review of these 9 articles found one additional article fulfilling inclusion criteria, leading to a final sample size of 10 articles …”