The IgG4 subclass accounts only for a small amount of total IgG (3-5%) and exhibits poor effector properties with regard to complement and FcRgamma activation. Yet since 10 years an increasing number of reports has defined a new set of IgG4-related diseases (IgG4-RD) characterised by elevated serum IgG4 levels and diffuse or nodular lympho-plasmocytic infiltrates (>40% IgG4+ plasma cells) in various, mainly exocrine organs. The infiltrates are typically associated with a chronic-fibrosing inflammation. While the serum IgG4+ fraction is not always increased it contains an increased proportion of various autoantibodies. Here we review the structural properties of the IgG4 molecule and the factors regulating class-switch to IgG4 and we discuss how the unique property of IgG4 to undergo Fab-arm exchange may influence the peripheral B cell tolerance and impact on the immunopathology of IgG4-RD.
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Queen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R ChinaQueen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R China
Cheuk, Wah
Yuen, Hunter K. L.
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Chinese Univ Hong Kong, Dept Ophthalmol & Visual Sci, Hong Kong Eye Hosp, Hong Kong, Hong Kong, Peoples R ChinaQueen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R China
Yuen, Hunter K. L.
Chu, Stephenie Y. Y.
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Queen Elizabeth Hosp, Dept Med, Kowloon, Hong Kong, Peoples R ChinaQueen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R China
Chu, Stephenie Y. Y.
Chiu, Edinond K. W.
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Bank Amer Tower, Hong Kong, Hong Kong, Peoples R ChinaQueen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R China
Chiu, Edinond K. W.
Lam, L. K.
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机构:Queen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R China
Lam, L. K.
Chan, John K. C.
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Queen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R ChinaQueen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R China
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Queen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R ChinaQueen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R China
Cheuk, Wah
Yuen, Hunter K. L.
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Chinese Univ Hong Kong, Dept Ophthalmol & Visual Sci, Hong Kong Eye Hosp, Hong Kong, Hong Kong, Peoples R ChinaQueen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R China
Yuen, Hunter K. L.
Chu, Stephenie Y. Y.
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机构:
Queen Elizabeth Hosp, Dept Med, Kowloon, Hong Kong, Peoples R ChinaQueen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R China
Chu, Stephenie Y. Y.
Chiu, Edinond K. W.
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h-index: 0
机构:
Bank Amer Tower, Hong Kong, Hong Kong, Peoples R ChinaQueen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R China
Chiu, Edinond K. W.
Lam, L. K.
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机构:Queen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R China
Lam, L. K.
Chan, John K. C.
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h-index: 0
机构:
Queen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R ChinaQueen Elizabeth Hosp, Dept Pathol, Kowloon, Hong Kong, Peoples R China