Hyper IgG4 disease or IgG4-related sclerosing/autoimmune disease is a multisystem condition characterized histologically by fibrosis, lymphoplasmacytic infiltration, and abundant IgG4 plasma cells associated with raised serum IgG4 levels. We present a case of salivary duct carcinoma of the parotid gland in a background of chronic sclerosing sialadenitis that also involved the submandibular gland with associated regional lymphadenopathy. The serology showed raised total IgG levels of 16.3 g/L (reference range, 6.0-15.0) and raised IgG4 levels of 3.41 g/L (reference range, 0.07-1.70). The salivary duct carcinoma contained areas of dense fibrosis and abundant IgG4-positive plasma cells (>100 per high-power field [hpf]). The adjacent noncarcinomatous areas, submandibular gland, and regional lymph nodes also contained plasma cells immunoreactive to IgG4 with densities higher than 100/hpf. To the best of our knowledge, this case is the first documentation of malignancy occurring in a background of IgG4-related autoimmune disease of the salivary gland.
"Therefore, it is unclear whether the pathogenetic mechanism is same among individual organs or not. In addition to IgG4-RD, IgG4-associate conditions such as high serum levels of IgG4 or abundant infiltration of IgG4-positive cells were reported in some patients with malignancy; pancreatic  , biliary  and salivary cancer , gastrointestinal sarcoma , and ocular adnexal lymphoma   . Therefore, the term " systemic " may lead us to misdiagnosis of other organ lesions showing IgG4- related conditions in cases of malignancy . "
[Show abstract][Hide abstract] ABSTRACT: Recent studies suggest simultaneous or metachronous lesions in multiorgans characterized by elevated serum levels of IgG4 and abundant infiltration of IgG4-positive plasma cells with various degrees of fibrosis. Two Japanese research committees for IgG4-RD, one from fibrosclerosis (Okazaki team) and the other from lymph proliferation (Umehara team) supported by the "Research Program for Intractable Disease" of the Ministry of Health, Labor, and Welfare of Japan, have agreed with the unified nomenclature as "IgG4-RD" and proposed the comprehensive diagnostic criteria (CDC) for IgG4-RD. Validation of the CDC demonstrated satisfactory sensitivity for the practical use of general physicians and nonspecialists but low sensitivity in the organs to be difficult in taking biopsy specimens such as type1 autoimmune pancreatitis (IgG4-related AIP), compared with IgG4-related sialadenitis/dacryoadenitis (Mikulicz's disease) and IgG4-related kidney disease. Although the diagnostic criteria covering all IgG4-RD are hard to be established, combination with the CDC and organ-specific diagnostic criteria should improve sensitivity.
International Journal of Rheumatology 05/2012; 2012(11):357071. DOI:10.1155/2012/357071
"Several cases of pancreatic ductal adenocarcinoma have been published in association with IgG4-RSD.183-186 Case reports of pulmonary adenocarcinoma, salivary duct carcinoma, urothelial carcinoma in situ and gastrointestinal clear cell sarcoma in association with IgG4-RSD have been reported in the lung, parotid gland, ureter and small intestine, respectively.140,150,187,188 Further studies are mandated in this field to determine if there is any causal relationship between the malignancies and IgG4-RSD. "
[Show abstract][Hide abstract] ABSTRACT: Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recently defined emerging entity characterized by a diffuse or mass forming inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosclerosis and obliterative phlebitis. IgG4-RSD usually affects middle aged and elderly patients, with a male predominance. It is associated with an elevated serum titer of IgG4, which acts as a marker for this recently characterized entity. The prototype is IgG4-related sclerosing pancreatitis or autoimmune pancreatitis (AIP). Other common sites of involvement are the hepatobiliary tract, salivary gland, orbit, and lymph node, however practically any organ can be involved, including upper aerodigestive tract, lung, aorta, mediastinum, retroperitoneum, soft tissue, skin, central nervous system, breast, kidney, and prostate. Fever or constitutional symptoms usually do not comprise part of the clinical picture. Laboratory findings detected include raised serum globulin, IgG and IgG4. An association with autoantibody detection (such as antinuclear antibodies and rheumatoid factor) is seen in some cases. Steroid therapy comprises the mainstay of treatment. Disease progression with involvement of multiple organ-sites may be encountered in a subset of cases and may follow a relapsing-remitting course. The principal histopathologic findings in several extranodal sites include lymphoplasmacytic infiltration, lymphoid follicle formation, sclerosis and obliterative phlebitis, along with atrophy and destruction of tissues. Immunohistochemical staining shows increased IgG4+ cells in the involved tissues (>50 per high-power field, with IgG4/IgG ratio >40%). IgG4-RSD may potentially be rarely associated with the development of lymphoma and carcinoma. However, the nature and pathogenesis of IgG4-RSD are yet to be fully elucidated and provide immense scope for further studies.
Yonsei medical journal 01/2012; 53(1):15-34. DOI:10.3349/ymj.2012.53.1.15 · 1.29 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: In June 2008, a 74-year-old male was diagnosed with IgG4-related disease including histologically proven IgG4-related prostatitis, and then followed as an outpatient. In July 2011, cervical, chest, and abdominal computed tomography (CT) revealed right parotid gland swelling and lymph node enlargement of the supraclavicular, mediastinal, left hilar, porta hepatis, and para-aorta. A biopsy of the right parotid gland was performed, and we diagnosed diffuse large B-cell lymphoma (DLBCL). As malignancies are possible complications for patients with IgG4-related disease, we must be careful in the follow-up of IgG4-related disease patients.
Clinical Journal of Gastroenterology 02/2012; 6(1). DOI:10.1007/s12328-012-0345-y
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