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SS-干燥综合症发病机制研究.pdf


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干燥综合征的发病机制及转化研究
香港大学吕力为
干燥综合征(Sjögren's Syndrome)
• Dry eyes, dry mouth
• A systemic disease
Karolinska Institutet
1933
干燥综合征是一种多发病
1993
张乃峥教授
Epidemiology of primary Sjögren’s Syndrome
患病率 Countries Prevalence
(%)
UK
Sweden
Turkey
Canada
Denmark
China
Slovenia
USA
Norway
Italy
Brazil
论文 Greece
Qin B et al, Ann Rheum Dis . 2015 SS :
Sherine E Gabriel et al. Arthritis Res Ther. 2009 France
(pSS: 5千)
原发性干燥综合征发病机理目前不清楚
Dry eyes, dry mouth
CD4 CD20
Pathogenesis of primaryR SEVjIEöWgSren’s syndrome
Lymphocytic Virus? O ther activator infiltrates, but interestingly w ithout any detectable C M V
of innate im m unity? in salivary glands at the onset of auto im m A nother
infiltration potential explanation for the elusiveness of the trigger for
Minor salivary gland biopsy pSS could be that overexpression of som e endogenous
retroviral sequences has been overlooked, and should
- Effector B cell
Epithelial cell be considered instead of searching for an external virus
-AutoAbs responsible for disease-associated activation of the IFN
system . For exam ple, ic abnorm alities related to
-GC-like structure pD C D C T-cell
in SG activation expression of endogenous retroviral elem ents could be
N K responsible for activation of the type I IFN pathw ay.
cell
-B lymphoma IFN -α How about macrophages?
TH 1
N K-cell IL-12 cell N FκB pathw ay dysfunction in pSS pathogenesis
Autoantigen activation
- Effector T cell IL-12R O ne of the consequences of the stim ulation of innate
CD3 / CD20 im m unity is the activation of nuclear factor κB (N FκB),
-Infiltration BAFF w hich can occur in a num ber of different cell types. In
-Cytokine storm IFN -γ salivary gland epithelial cells from patients w ith pSS,
ICs hyperactivation of N FκB has been associated w ith
Th17 decreased expression of one of the regulators of N

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  • 时间2018-02-19