Relapsing Polychondritis With Encephalitis
Relapsing Polychondritis With Encephalitis
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DOI：
10.1097/rhu.0b013e31822e071b
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发表时间：
2011-09-01
影响因子：
3.4
通讯作者：
Lee, Hee Jung
中科院分区：
文献类型：
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作者：
Choi, Hyuk Jai;Lee, Hee Jung
复发性多软骨炎是一种罕见的多系统自身免疫性疾病，对不同的软骨蛋白既有自身抗体又有细胞免疫反应。RP的发病机制尚不清楚。抗II型胶原抗体以及I型和XI型已被描述，但它们缺乏敏感性和特异性。4细胞免疫在RP的发病机制中起重要作用。5患者组巨噬细胞抑制因子水平明显高于对照组。6此外，1例患者具有II型胶原表位特异性T细胞克隆的特征。7患者外周血中人类白细胞抗原-DR4的频率显著升高，且与疾病的发生呈负相关。8
DISCUSSIONRelapsing polychondritis is a rare multisystem autoimmune disease with both autoantibodies and cellular immune reactions to different cartilage proteins. The pathogenesis of RP is still not known. Antibodies to type II collagen, as well as types IX and XI have been described, but they lack sensitivity and specificity. 4 Cell-mediated immunity is believed to be important in the pathogenesis of RP. 5 Levels of macrophage inhibitory factor are significantly higher in patients than in control subjects. 6 Furthermore, 1 patient has been characterized with T-cell clones specific for a type II collagen epitope. 7 There is a significant increase in the frequency of HLA-DR4 in patients, and the disease is associated negatively with HLA-DR6. 8