Synovitis in a murine model of human factor VIII deficiency
Synovitis in a murine model of human factor VIII deficiency
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DOI：
10.1111/j.1365-2516.2005.01080.x
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发表时间：
2005-05-01
期刊：
影响因子：
3.9
通讯作者：
Valentino, LA
中科院分区：
文献类型：
--
作者：
Hakobyan, N;Kazarian, T;Valentino, LA
复发性关节出血是血友病最常见的肌肉骨骼表现，并导致靶关节和滑膜炎。血友病性滑膜炎（HS）的病理生物学还不清楚。在此，在人类HS动物模型中检查了关节积血后发生的组织病理学变化。两次关节积血后，出现软组织和关节肿胀，急性滑膜炎的组织学变化包括单核细胞和中性粒细胞浸润滑膜下层、滑膜增厚伴绒毛形成和血管增生。三次关节积血后亚急性变化明显;出现肌肉萎缩，滑膜下间隙充满强烈的单核细胞浸润。关节面破坏，软骨丢失。三次关节积血后17个月，慢性关节变化包括由于致密纤维化组织填充关节间隙导致的严重畸形和一致性丧失。单核炎性细胞浸润和滑膜增厚持续存在。关节面和软骨下囊肿存在凹坑和侵蚀。有纤维软骨和新骨形成。这种人类HS模型应该是有用的，以充分评估生物化学和分子的变化，发生后，关节出血，并测试新的治疗方法，以防止HS。
Recurrent joint bleeding is the most common musculoskeletal manifestation of haemophilia and leads to a target joint and synovitis. The pathobiology of haemophilic synovitis (HS) is not well understood. Here the histopathological changes that occur following haemarthrosis were examined in an animal model for human HS. After two haemarthrosis, there was soft tissue and joint swelling and histological changes of acute synovitis included infiltration of the sub-synovial layer by mononuclear cells and neutrophils, thickening of the synovial membrane with villus formation, and hyperplasia of blood vessels. Subacute changes were evident after three haemarthrosis; muscle atrophy was present and an intense mononuclear cell infiltrate filled the sub-synovial space. There was destruction of articular surfaces and loss of cartilage. Seventeen months after three haemarthrosis, chronic joint changes included gross deformity and loss of congruence due to dense fibrotic tissue filling the joint space. The mononuclear inflammatory cell infiltrate and thickened synovial membrane persisted. Pits and erosions of articular surfaces and sub-chondral cysts were present. There was fibro-cartilage and new bone formation. This model of human HS should be useful to fully evaluate the biochemical and molecular changes that occur following joint bleeding and to test novel therapeutics to prevent HS.