复发性多软骨炎的病理学
- Author
- Clement J Michet, MD
Clement J Michet, MD
- Associate Professor of Medicine
- Mayo Clinic
- Section Editor
- Jane Hoyt Buckner, MD
Jane Hoyt Buckner, MD
- Section Editor — General Rheumatology and Related Topics
- President and Director of Translational Research, Benaroya Research Institute
- Affiliate professor, University of Washington
- Deputy Editor
- Paul L Romain, MD
Paul L Romain, MD
- Deputy Editor — Rheumatology
- Assistant Professor of Medicine, Part-time
- Harvard Medical School
- Translators
- 张银刚, 副教授
张银刚, 副教授
- 西安交通大学第一附属医院骨科
引言
复发性多软骨炎(relapsing polychondritis, RPC)以分布广泛、可能具有破坏性、存在炎症和退行性病变为特征。耳软骨是典型的受累部位，但RPC也有可能累及全身软骨及与其在生化和免疫学上有关的结缔组织结构(表 1)。 (参见“复发性多软骨炎的临床表现”)
本专题将总结RPC的病理学改变。RPC的发病机制将单独讨论。 (参见“复发性多软骨炎的病因与发病机制”)
典型活动性耳部病变的组织学
活动性耳部病变的活检结果也代表着其他部位结缔组织的病理学改变。病理学改变具有一系列取决于活检时间的特征性表现。
●最初可在软骨-真皮交界处观察到软骨膜受到多形态性细胞浸润，其中包括淋巴细胞和比例因人而异的多形核细胞、单核细胞/巨噬细胞和浆细胞(图片 1)。淋巴细胞以CD4辅助性T细胞为主，其多于CD8细胞毒性T细胞[1]。毗邻软骨的区域存在蛋白聚糖大量减少的证据。过在交界处实施直接免疫荧光法可定位主要由IgG和补体C3组成的粗颗粒沉积物。
●随着疾病的进展，肉芽组织侵入软骨破坏使其完整性遭到破坏，这往往会隔离出由退变的软骨细胞和受累基质所形成的孤岛(图片 2)。整个基质中都可见IgG和C3。软骨膜肉芽组织和软骨细胞中均表达基质金属蛋白酶(matrix metalloproteinasis, MMP)[2]。在软骨炎病变区，软骨细胞凋亡增加，且与MMP-3和组织蛋白酶-K的表达相一致。一氧化氮表达增加，反映出软骨细胞生成了促凋亡的MMP。
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