IgG4-related disease

Kamisawa, Terumi; Zen, Yoh; Pillai, Shiv; Stone, John H.

doi:10.1016/s0140-6736(14)60720-0

Cited by 1,052 publications

(1,176 citation statements)

References 99 publications

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“…Subsequent pathological studies identified similar IgG4-related fibroinflammatory conditions in various organs outside the pancreas [2][3][4][5][6][7][8][9][10], eventually leading to the recognition of a systemic condition named IgG4-related disease (IgG4-RD) [11,12]. During this process, the bile duct appeared to be one of the target organs commonly affected by this condition [13][14][15].…”

Section: Introductionmentioning

confidence: 99%

IgG4-related sclerosing cholangitis: all we need to know

2016

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Our knowledge and experience of IgG4-related sclerosing cholangitis (ISC) have expanded in the last decade. ISC is one of the common organ manifestations of IgG4-related disease (IgG4-RD); approximately 60 % of patients with this systemic condition have ISC in the proximal and/or distal bile ducts. ISC needs to be discriminated from primary sclerosing cholangitis, cholangiocarcinoma, and other rare forms of lymphoplasmacytic cholangiopathy (e.g., follicular cholangitis and sclerosing cholangitis with granulocytic epithelial lesions). Its diagnosis requires a multidisciplinary approach, in which serology, histology, and imaging play crucial roles. Treatments with high-dose corticosteroids typically lead to the rapid and consistent induction of disease remission. Another promising therapeutic approach is B-cell depletion with rituximab. Although disease relapse is relatively common, provided that appropriate treatments are administered, ISC is considered a ''benign'' disease with a low risk of liver failure and biliary malignancy. Its molecular pathology is characterized by Th2-dominant immune reactions, regulatory T-cell activation, and CCL1-CCR8 interactions. Particular subsets of B cells such as plasmablasts and regulatory B cells also expand. A recent global proteomic study demonstrated that three significantly activated immunological cascades in ISC were all B-cell-or immunoglobulin-related (Fc-gamma receptormediated phagocytosis, B-cell receptor signaling pathway, and Fc-epsilon receptor I signaling pathway), suggesting the crucial roles of B cells in the underlying immune reactions. Despite the expansion of our knowledge of the pathophysiology of ISC, the exact role of IgG4 remains unclear. A better understanding of its immunopathology will offer some potential drug targets for this emerging biliary disease.

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Section: Introductionmentioning

confidence: 99%

IgG4-related sclerosing cholangitis: all we need to know

2016

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“…Both diseases have common clinical features including elevated serum IgG4 levels and histopathologic infiltration of IgG4-positive plasma cells. At present, these diseases are regarded as IgG4-related lesions of the salivary gland [6][7][8][9].…”

Section: Introductionmentioning

confidence: 99%

Sonographic findings of immunoglobulin G4-related sclerosing sialadenitis

et al. 2015

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PurposeWe evaluated the sonographic findings of immunoglobulin G4-related sclerosing sialadenitis (IgG4-SS). MethodsNineteen patients with IgG4-SS and 12 healthy volunteers (controls) were enrolled. The following sonographic features were evaluated: 1) enlargement of the submandibular gland by measurement of the longitudinal diameter and thickness; 2) the contour texture of the submandibular gland (smooth or rough); 3) the internal echo texture, categorized into three sonographic patterns (homogeneous, multiple hypoechoic nodule, and diffuse hypoechoic); and 4) quantitative color Doppler signaling. ResultsThe longitudinal diameter and the thickness (mean ± SD) of the submandibular gland were significantly greater in patients than in controls (p=0.005 and p<0.001, respectively). Contour roughness was seen in 62.9% and 8.3% of patients and controls (p<0.001), respectively.Homogeneous echo textures alone were seen in controls, whereas multiple hypoechoic nodule patterns were seen in 60% of patients, and diffuse hypoechoic patterns were seen in 40%. Color Doppler signaling (mean ± SD) was significantly higher in patients as compared with controls (p<0.001). ConclusionPatients could be distinguished from healthy volunteers using four distinctive sonographic findings, suggesting that ultrasonography would be a useful diagnostic tool for IgG4-SS.

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“…The pathological diagnosis of IgG4-related diseases requires an IgG4-positive plasma cells/IgG positive plasma cells ratio of >40%, fibrosis with a characteristic "storiform" pattern, and obliterative phlebitis [29,30]. Therefore, Hashimoto's thyroiditis seen in our cases did not satisfy the diagnostic criteria of IgG4-related disease.…”

Section: Discussionmentioning

confidence: 78%

Thyroid sclerosing mucoepidermoid carcinoma with eosinophilia distinct from the salivary type

et al. 2018

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Abstract. We report three cases of thyroid sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE), which is an extremely rare variant of mucoepidermoid carcinoma (MEC). The aims of this report were to describe the clinicopathological findings, including results from immunohistochemical and fluorescence in situ hybridization analysis of thyroid SMECE, as well as to discuss the distinction between thyroid SMECE and its salivary counterpart. The cases included a 63-year-old female, a 44-year-old male, and a 66-year-old female, with all patients presenting with Hashimoto's thyroiditis. Nodal metastasis was not found in any of the three cases. Neither regional recurrences nor distant metastases were found in any patient during the follow-up, which was 20 years, 3 years, and 18 months, respectively. Histologically, tumors were composed of epidermoid carcinoma cells, intermediate type carcinoma cells, and goblet cell-type mucus-secreting carcinoma cells, with all tumors displaying a sclerotic stroma with eosinophilic and lymphocytic infiltration. The formation of eosinophilic abscess in the tumor nests that might be a novel characteristic finding of SMECE was observed. Immunohistochemically, the carcinoma cells were positive for cytokeratin 34βE12, TTF-1, and PAX8, but negative for thyroglobulin. In two cases, increased IgG4-positive plasma cells were observed. Mastermind-like transcriptional coactivator 2 (MAML2), according to fluorescence in situ hybridization, was intact in all cases. In conclusion, thyroid SMECE has favorable outcomes and seems to be genetically different from salivary MEC. This is the first report to describe the presence of increased IgG4-positive plasma cells in the stroma of SMECE. [16]. The prognosis of this malignancy is favorable, although nodal metastases, extrathyroidal invasion, vascular invasion, and perineural invasion are common [12]. We encountered three patients with this rare sclerotic variant of MEC. The aims of this report were to describe the clinicopathological findings, including results from immunohistochemical and fluorescence in situ hybridization (FISH) analysis of thyroid SMECE, as well as to discuss the distinction between thyroid SMECE and its salivary counterpart.

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IgG4-related disease

Cited by 1,052 publications

References 99 publications

IgG4-related sclerosing cholangitis: all we need to know

IgG4-related sclerosing cholangitis: all we need to know

Sonographic findings of immunoglobulin G4-related sclerosing sialadenitis

Thyroid sclerosing mucoepidermoid carcinoma with eosinophilia distinct from the salivary type

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