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IgG4-related disease and its pathogenesis-cross-talk between innate and acquired immunity.

Umehara H, Nakajima A, Nakamura T, Kawanami T, Tanaka M, Dong L, Kawano M - Int. Immunol. (2014)

Bottom Line: IgG4-related disease (IgG4-RD) is a novel clinical entity proposed in Japan in the 21th century and is attracting strong attention over the world.The characteristic manifestations of IgG4-RD are increased serum IgG4 concentration and tumefaction by IgG4(+) plasma cells.Recently, many reports of aberrant acquired immunity such as Th2-diminated immune responses have been published.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Division of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Japan Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501, Japan Present address: Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501, Japan umehara606@gmail.com.

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IgG4-RD. (a) IgG4-related dacryoadenitis, sialadenitis and parotitis (so-called IgG4-related MD). MRI (upper) and PET (lower). (b) IgG4-related AIP (type I AIP). CT shows swelling of the pancreas (arrows) with hepatic phase enhancement and low-density capsule like rim (left). Pancreatogram shows diffusely irregular narrowing of the main pancreatic duct (right). CT and X-P films were provided by Dr K. Okazaki (Kansai Medical University). (c) IgG4-related kidney disease (IgG4-related KD). Multiple low-density lesions on enhanced CT (arrows in left) and diffuse thickening of the renal pelvis wall with smooth intraluminal surface (arrows in right).
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Figure 1: IgG4-RD. (a) IgG4-related dacryoadenitis, sialadenitis and parotitis (so-called IgG4-related MD). MRI (upper) and PET (lower). (b) IgG4-related AIP (type I AIP). CT shows swelling of the pancreas (arrows) with hepatic phase enhancement and low-density capsule like rim (left). Pancreatogram shows diffusely irregular narrowing of the main pancreatic duct (right). CT and X-P films were provided by Dr K. Okazaki (Kansai Medical University). (c) IgG4-related kidney disease (IgG4-related KD). Multiple low-density lesions on enhanced CT (arrows in left) and diffuse thickening of the renal pelvis wall with smooth intraluminal surface (arrows in right).

Mentions: The All Japan IgG4-RD Research Group, organized by the Ministry of Health, Labor and Welfare (MHLW) of Japan reached a consensus on the general concept of IgG4-RD (18): (i) IgG4-RD is characterized by organ enlargement or nodular/hyperplastic lesions in various organs concurrently or metachronously, caused by marked infiltration of lymphocytes and IgG4+ plasma cells and fibrosis with unknown etiology; (ii) IgG4-RD can affect various organs, including the pancreas, bile duct, lacrimal gland, salivary gland, thyroid, lung, liver and kidney (Fig. 1); (iii) many patients with IgG4-RD may have lesions in several organs, either synchronously or metachronously; (iv) clinical symptoms vary depending on the affected organ(s), with some patients experiencing serious complications, such as obstruction or compression symptoms as a result of organomegaly or hypertrophy and organ dysfunction caused by cellular infiltration or fibrosis; (v) IgG4-RD mainly affects middle-aged to elderly men; (vi) many patients with IgG4-RD can be treated effectively by steroid therapy; (vii) although the infiltration of IgG4+ cells and increased serum concentrations of IgG4 are common features of IgG4-RD, the severity of fibrosis is dependent on the individual organs involved.


IgG4-related disease and its pathogenesis-cross-talk between innate and acquired immunity.

Umehara H, Nakajima A, Nakamura T, Kawanami T, Tanaka M, Dong L, Kawano M - Int. Immunol. (2014)

IgG4-RD. (a) IgG4-related dacryoadenitis, sialadenitis and parotitis (so-called IgG4-related MD). MRI (upper) and PET (lower). (b) IgG4-related AIP (type I AIP). CT shows swelling of the pancreas (arrows) with hepatic phase enhancement and low-density capsule like rim (left). Pancreatogram shows diffusely irregular narrowing of the main pancreatic duct (right). CT and X-P films were provided by Dr K. Okazaki (Kansai Medical University). (c) IgG4-related kidney disease (IgG4-related KD). Multiple low-density lesions on enhanced CT (arrows in left) and diffuse thickening of the renal pelvis wall with smooth intraluminal surface (arrows in right).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4201844&req=5

Figure 1: IgG4-RD. (a) IgG4-related dacryoadenitis, sialadenitis and parotitis (so-called IgG4-related MD). MRI (upper) and PET (lower). (b) IgG4-related AIP (type I AIP). CT shows swelling of the pancreas (arrows) with hepatic phase enhancement and low-density capsule like rim (left). Pancreatogram shows diffusely irregular narrowing of the main pancreatic duct (right). CT and X-P films were provided by Dr K. Okazaki (Kansai Medical University). (c) IgG4-related kidney disease (IgG4-related KD). Multiple low-density lesions on enhanced CT (arrows in left) and diffuse thickening of the renal pelvis wall with smooth intraluminal surface (arrows in right).
Mentions: The All Japan IgG4-RD Research Group, organized by the Ministry of Health, Labor and Welfare (MHLW) of Japan reached a consensus on the general concept of IgG4-RD (18): (i) IgG4-RD is characterized by organ enlargement or nodular/hyperplastic lesions in various organs concurrently or metachronously, caused by marked infiltration of lymphocytes and IgG4+ plasma cells and fibrosis with unknown etiology; (ii) IgG4-RD can affect various organs, including the pancreas, bile duct, lacrimal gland, salivary gland, thyroid, lung, liver and kidney (Fig. 1); (iii) many patients with IgG4-RD may have lesions in several organs, either synchronously or metachronously; (iv) clinical symptoms vary depending on the affected organ(s), with some patients experiencing serious complications, such as obstruction or compression symptoms as a result of organomegaly or hypertrophy and organ dysfunction caused by cellular infiltration or fibrosis; (v) IgG4-RD mainly affects middle-aged to elderly men; (vi) many patients with IgG4-RD can be treated effectively by steroid therapy; (vii) although the infiltration of IgG4+ cells and increased serum concentrations of IgG4 are common features of IgG4-RD, the severity of fibrosis is dependent on the individual organs involved.

Bottom Line: IgG4-related disease (IgG4-RD) is a novel clinical entity proposed in Japan in the 21th century and is attracting strong attention over the world.The characteristic manifestations of IgG4-RD are increased serum IgG4 concentration and tumefaction by IgG4(+) plasma cells.Recently, many reports of aberrant acquired immunity such as Th2-diminated immune responses have been published.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Division of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Japan Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501, Japan Present address: Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501, Japan umehara606@gmail.com.

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Related in: MedlinePlus