Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman's disease

Sato, Yasuharu; Kojima, Masaru; Takata, Katsuyoshi; Morito, Toshiaki; Asaoku, Hideki; Takeuchi, Tamotsu; Mizobuchi, Kohichi; Fujihara, Megumu; Kuraoka, Kazuya; Nakai, Tokiko; Ichimura, Kouichi; Tanaka, Takehiro; Tamura, Maiko; Nishikawa, Yuriko; Yoshino, Tadashi

doi:10.1038/modpathol.2009.17

Cited by 255 publications

(250 citation statements)

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“…Features of Rosai-Dorfman disease were not seen. Plasma cells were shown to be polyclonal in ten cases using in situ stains for kappa and lambda (Cases 1,5,8,9,11,13,14,18,25,27). Polyclonal kappa and lambda staining was demonstrated by immunohistochemistry in three cases (Case 24, 25, 27).…”

Section: Additional Histological and Immunohistochemical Findingsmentioning

confidence: 99%

“…Ten cases had concurrent flow cytometry results on the lymph node specimens (Cases 2, 5,6,8,11,14,15,16,23,25). In all ten cases, CD4-positive T cells were increased compared with CD8 cells with CD4:CD8 ratios ranging from 3.36 to 11.24.…”

Section: Flow Cytometrymentioning

confidence: 99%

“…These patterns are: multicentric Castleman disease-like, follicular hyperplasia, interfollicular expansion, progressive transformation of germinal centers and nodal inflammatory pseudotumor-like. 5,[7][8][9] We selected cases with these histological findings and other features of benign adenopathy in adult patients, and evaluated for IgG and IgG4 by immunostaining.…”

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Histopathological findings in 29 lymph node biopsies with increased IgG4 plasma cells

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Section: Additional Histological and Immunohistochemical Findingsmentioning

confidence: 99%

Section: Flow Cytometrymentioning

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Histopathological findings in 29 lymph node biopsies with increased IgG4 plasma cells

et al. 2012

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“…[2][3][4][5][6] IgG4-related disease is a recently recognized syndrome characterized by mass-forming lesions with lymphoplasmacytic infiltration, accumulation of IgG4 þ plasma cells in affected tissues and increased serum IgG4 levels. [7][8][9][10][11][12][13][14] IgG4-related disease generally involves either localized or systemic lymph nodes, 8,13,14 and five histological subtypes of IgG4-related lymphadenopathy have been recognized. 8 In 2009, we were the first to report patients with IgG4-related disease in progressively transformed germinal centers of the lymph nodes (progressively transformed germinal centers-type IgG4-related lymphadenopathy).…”

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Association between IgG4-related disease and progressively transformed germinal centers of lymph nodes

et al. 2012

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Progressively transformed germinal centers is a benign condition of unknown pathogenesis characterized by a distinctive variant form of reactive follicular hyperplasia in lymph nodes. We recently reported Ig G4-related disease in progressively transformed germinal centers. However, no large case series has been reported and clinicopathologic findings remain unclear. Here, we report 40 Japanese patients (28 men, 12 women; median age, 56 years) with progressively transformed germinal centers of the lymph nodes who fulfilled the histological diagnostic criteria for IgG4-related disease (IgG4 þ progressively transformed germinal centers), with asymptomatic localized lymphadenopathy involving the submandibular nodes in 24, submandibular and cervical nodes in 14, cervical nodes only in 1, and cervical and supraclavicular nodes in 1. In all, 16 (52%) of 31 examined patients had allergic disease. Histologically, the lymph nodes demonstrated uniform histological findings, namely marked follicular hyperplasia with progressively transformed germinal centers, and localization of the majority of IgG4 þ plasma cells in the germinal centers. Serum IgG4, serum IgE and peripheral blood eosinophils were elevated in 87%, 92% and 53% of examined patients, respectively. Eighteen patients subsequently developed extranodal lesions (including five who developed systemic disease), which on histological examination were consistent with IgG4-related disease. IgG4 þ progressively transformed germinal centers presents with uniform clinicopathological features of asymptomatic localized submandibular lymphadenopathy, which persists and/or relapses, and sometimes progresses to extranodal lesions or systemic disease. Nine patients were administered steroid therapy when the lesions progressed, to which all

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“…The lymph nodes are non-tender and 1-3 cm in diameter (71,72). Fibrosis is uncommon feature and is only seen in pseudotumor-like lymph nodes (34).…”

Section: Lymph Nodesmentioning

confidence: 99%

IgG4-related disease: A relatively new concept for clinicians

Vasaitis

2016

European Journal of Internal Medicine

100

129

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IgG4-related disease (IgG4-RD) is a recently recognized chronic fibrotic inflammation, which can affect almost every organ, and may come to clinical attention first due to visible organ swelling or organ dysfunction, or is identified incidentally by imaging and specific biopsy.The disorder has an allergic background and is immune-mediated. Up-regulated responses of T helper 2 and T regulatory cells and their cytokines play a major role in disease progression.About 30-50% of patients are atopic or have mild eosinophilia. IgG4-RD predominantly affects middle-aged male patients. The cornerstones of diagnosis of the disease are compatible clinical features and typical histopathology. Swelling of salivary and lacrimal glands, lymphadenopathy, and type 1 autoimmune pancreatitis (AIP) are the most common manifestations of the disease. However, other tissues and organs, such as retroperitoneum, lung, kidney, aorta, upper airways, thyroid gland, meninges, heart, mesenterium and skin may be involved. Typical histopathology is lymphoplasmacytic infiltration abundant in IgG4-positive plasma cells, storiform-type fibrosis, and obliterative phlebitis. Elevated serum IgG4 concentration supports the diagnosis. Characteristic imaging features such as a "capsule-like rim" surrounding the pancreatic lesions is highly specific to type 1 AIP. 18F-fluorodeoxyglucose positron emission tomography/computed tomography enables mapping the sites of inflammation, permits evaluation of the extent of the disease, helps in guiding biopsy decision, and may be used in monitoring response to treatment. Glucocorticoids alone or in combination with B-cell depletion with rituximab induces prompt clinical response to IgG4-RD.This article reviews the current understanding, different clinical manifestations, and approaches to diagnosis and treatment of IgG4-RD. 3Highlights Ø IgG4-related disease is a chronic fibrotic inflammation.Ø The disease affects multiple organs, is progressive, and impairs function.Ø The disorder has an allergic background and is immune-mediated.Ø Compatible clinical signs and typical pathology are the basis for the diagnosis.Ø Glucocorticoids and/or B-cell depletion induces prompt clinical response.Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibrotic inflammation, characterized by tissue infiltration by lymphocytes, IgG4-positive plasma cells, simultaneous development of fibrosis, and often, but not always, elevated serum levels of IgG4 (1, 2). The disease can affect multiple organs. Early diagnosis of IgG4-RD is not easy because of the indolent course of the disease with vague, nonspecific symptoms. It may first come to clinical attention as visible organ swelling or detected organ dysfunction due to fibrosis, or is identified incidentally by imaging and specific biopsy when the patient undergoes investigation of suspected tumor disease. Most forms of organ involvement in IgG4-RD, even in the advanced fibrotic stages, respond well to glucocorticoids (3, 4). 4A comprehensive search for all articles in E...

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Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman's disease

Cited by 255 publications

References 30 publications

Histopathological findings in 29 lymph node biopsies with increased IgG4 plasma cells

Histopathological findings in 29 lymph node biopsies with increased IgG4 plasma cells

Association between IgG4-related disease and progressively transformed germinal centers of lymph nodes

IgG4-related disease: A relatively new concept for clinicians

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